Phototherapy and Photochemotherapy (PUVA) for Skin Conditions

Number: 0205

Policy

PUVA

Aetna considers psoralens and ultraviolet A light (PUVA) treatments medically necessary for the following conditions after conventional therapies have failed:
- Alopecia areata;
- Chronic palmoplantar pustulosis;
- Cutaneous T-cell lymphoma (mycosis fungoides);
- Cutaneous manifestations of graft versus host disease;
- Eosinophilic folliculitis and other pruritic eruptions of HIV infection;
- Granuloma annulare;
- Grover's disease (transient and persistent acantholytic dermatosis);
- Lymphomatoid papulosis;
- Morphea (circumscribed scleroderma) and localized skin lesions associated with scleroderma;
- Necrobiosis lipoidica;
- Photodermatoses;
- Pityriasis lichenoides;
- Polymorphous light eruption;
- Severe lichen planus;
- Severe parapsoriasis;
- Severe refractory atopic dermatitis/eczema;
- Severe refractory pruritus of polycythemia vera;
- Severe urticaria pigmentosa (cutaneous mastocytosis);
- Severely disabling psoriasis (i.e., psoriasis involving 10 % or more of the body, or severe psoriasis involving the hands, feet, or scalp);
- Vitiligo.
Generally 2 to 3 PUVA treatments per week for up to 23 weeks are considered medically necessary for psoriasis. After 23 weeks, the medically necessary frequency of PUVA therapy is 1 treatment every 1 to 3 weeks with the majority of persons treated once every 3 weeks for an indefinite period. If the psoriasis fails to improve after 2 months of PUVA therapy, continued treatment is generally not considered medically necessary due to lack of efficacy.

Note: Home PUVA treatment is considered experimental and investigational because of insufficient evidence of its safety and effectiveness.

Aetna considers PUVA treatment experimental and investigational for all other indications. There is inadequate evidence in the peer-reviewed medical literature of the effectiveness of PUVA in any of these conditions (not an all-inclusive list):
- Acne;
- Eosinophilic cellulitis (Wells syndrome);
- Keratosis follicularis (Darier disease or Darier-White disease);
- Lichen amyloidosis;
- Lichen myxedematosus;
- Melasma;
- Necrobiotic xanthogranuloma;
- Necrobiosis lipoidica;
- To increase skin tolerance to sunlight.
UVA/UVB

Aetna considers phototherapy with UVA medically necessary for the following indications:
- Acne;
- Eczema (atopic dermatitis);
- Eosinophilic folliculitis and other pruritic eruptions of HIV infection;
- Lichen planus;
- Morphea (circumscribed scleroderma);
- Parapsoriasis;
- Photodermatoses;
- Pityriasis lichenoides;
- Pityriasis rosea;
- Prurigo nodularis;
- Psoriasis
- Scleredema that is functionally limiting or symptomatic.
Aetna considers the use of UVA experimental and investigational for infectious keratitis, lymphomatoid papulosis, uremic pruritus, and all other indications because there is inadequate evidence in the peer-reviewed medical literature of the effectiveness of UVA in any of these conditions .

Aetna considers narrow-band UVB phototherapy medically necessary for the following indications:
- Atopic dermatitis (atopic eczema);
- Chronic urticaria;
- Cutaneous mastocytosis (after conventional therapies have failed)
- Early-stage mycosis fungoides;
- Kyrle disease (perforating dermatosis) that is refractory to topical or intralesional therapy;
- Morphea (circumscribed scleroderma);
- Photodermatoses (e.g., actinic dermatitis and solar urticaria;
- Polymorphous light eruption;
- Psoriasis;
- Prurigo nodularis that is refractory to topical or intralesional corticosteroids
- Uremic pruritus that is refractory to emollients, topical analgesics and oral antihistamines or gabapentin.
For narrow-band UVB phototherapy for vitiligo, see CPB 0422 - Vitiligo.

Aetna considers narrow-band UVB phototherapy experimental and investigational for all other indications (e.g., alopecia mucinosa, chemical dermatitis/contact dermatitis, cholestasis of pregnancy, dermatographic urticaria (also known as dermographism and dermatographism), drug-related hypersensitivity reaction, erythema annulare centrifugum, graft-versus-host disease, granuloma annulare, Grover's disease (transient and persistent acantholytic dermatosis), Hailey-Hailey disease, hidradenitis suppurativa, lichen amyloidosis, lichen sclerosus, lichen simplex chronicus, lichenoid dermatitis, lymphomatoid papulosis, papular mucinosis, papular urticaria, progressive macular hypomelanosis, pruritus, scleroderma, skin hypo-pigmentation from scarring, and superficial mixed-cell dermatitis; not an all-inclusive list) because there is inadequate evidence in the peer-reviewed medical literature of the effectiveness of narrow-band UVB in any of these conditions.

Aetna considers UVB with the addition of topical coal tar (also known as the Goeckerman regimen) medically necessary for persons with severe psoriasis (defined as psoriasis that affects more than 10 % of body surface area). Aetna considers UVB with the addition of topical coal tar experimental and investigational for all other indications (e.g., pemphigoid, pruritis).

Aetna considers home phototherapy (UVB) treatment medically necessary DME for persons with severe psoriasis with a history of frequent flares who are unable to attend on-site therapy or those needing to initiate therapy immediately to suppress psoriasis flares. Aetna considers home phototherapy (UVB) medically necessary for persons with atopic dermatitis (eczema) who are unable to attend on-site therapy. Home ultraviolet light booths or ultraviolet lamps, as well as replacement bulbs sold by prescription only, are considered medically necessary for persons eligible for home UVB phototherapy.

Aetna considers home phototherapy (UVB) experimental and investigational for the treatment of cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome) because the effectiveness of this approach has not been established.
For excimer laser treatment of psoriasis, see CPB 0577 - Laser Treatment for Psoriasis and Other Selected Skin Conditions.

Aetna considers tanning beds for home UVB phototherapy not medically necessary. Unlike tanning beds, home UVB devices are designed solely for the medical treatment of skin diseases and emit a different wavelength of ultraviolet light than tanning beds. Note: In addition, tanning beds do not meet Aetna's definition of covered durable medical equipment in that they are of use in the absence of illness or injury.

See also CPB 0656 - Photodynamic Therapy for Acne.

Background

Psoralens and ultraviolet A light (PUVA) therapy is contraindicated in any of the following conditions:

history of arsenic exposure; or
history of ionizing radiation exposure; or
history or presence of melanoma or other skin cancer; or
pregnancy.

Khan and colleagues (2011) presented the first 3 cases of Acanthamoeba keratitis (AK), unresponsive to medical treatment, that were successfully treated with a novel adjunctive therapy using UVA and riboflavin (B2). Two patients with confirmed AK and 1 patient with presumptive AK, which were all refractive to multi-drug conventional therapy were included in this study. Two treatment sessions involving topical application of 0.1% B2 solution to the ocular surface combined with 30 mins of UVA irradiation focused on the corneal ulcer were carried out. Main outcome measures were clinical examination by slit lamp, confocal microscopy, and histopathology, when available. All patients in these series showed a rapid reduction in their symptoms and decreased ulcer size after the first treatment session. The progress of the clinical improvement began to slow after 1 to 3 weeks of the first application and was then renewed after the second application. All ancillary signs of inflammation mostly resolved after the second treatment session. The ulcers in all patients continued to decrease and were closed within 3 to 7 weeks of the first application. Two patients developed dense central corneal scars, and penetrating keratoplasty was performed for visual rehabilitation. Histopathological examination of the excised tissue revealed no Acanthamoeba organisms. The remaining patient had no symptoms or signs of infection, both clinically and by confocal microscopy, and was left with a semi-transparent eccentric scar that did not affect visual acuity. The authors concluded that adjunctive use of UVA and B2 therapy seems to be a possible alternative for selected cases of medication-resistant AK. Moreover, they noted that further research is needed and currently underway to examine how best to use photochemical therapy for the treatment of infectious keratitis.

Lowe (1992) stated that home UV phototherapy is extremely popular with many psoriasis patients. However, it is essential that they understand the need for regular skin examination by the dermatologist. Patients with psoriasis are not trained nor are many non-dermatologist physicians to recognize the early features of many skin cancers, and continued home UV therapy in the presence of such skin cancers is clearly unwise for the safety of that patient. The use of UVA tanning salon treatments in the therapy of psoriasis is usually unsuccessful and is extremely unwise with concomitant psoralen and drug therapy. This is to be discouraged, and the patient should always be treated with PUVA in the dermatologist's office with carefully monitored UVA machines and staff trained in the administration of PUVA phototherapy.

In an open-label, randomized controlled trial, van Coevorden et al (2004) examined if oral PUVA with a portable tanning unit at home is as effective as hospital-administered bath PUVA in patients with chronic hand eczema. A total of 158 patients with moderate-to-severe chronic hand eczema (more than 1 year in duration) were included in this study. The primary outcome was clinical assessment by a hand eczema score (evaluation of desquamation, erythema, vesiculation, infiltration, fissures, itch, and pain, each on a 4-point scale) after 10 weeks of treatment. The secondary outcome was hand eczema score at 8 weeks of follow-up, after completion of treatment. The tertiary outcome was travel cost and time off work. Both groups showed a comparable and substantial decrease in hand eczema score (meaningful clinical improvement). This decrease was maintained during the follow-up period. Patients treated with oral PUVA at home had lower travel costs and less time off work. The authors concluded that oral PUVA at home has a clinically relevant efficacy, similar to that of hospital-administered bath PUVA. This effect was maintained during an 8-week follow-up period. It resulted in lower travel costs and less time off work. These promising results need to be validated by more research.

Dermatographic urticaria (also known as dermographism and dermatographism) is a skin disorder observed in 4 to 5% of the population and is one of the most common types of urticaria, in which the skin becomes raised and inflamed when stroked, scratched, rubbed, and sometimes even slapped. Dermatographic urticaria can be treated by anti-histamines, which may need to be given as a combination of H1 antagonists, or possibly with an H2-receptor antagonist such as cimetidine. Therapies may also include immunosuppressive agents and steroids. There is a lack of evidence regarding the clinical value of narrow-band UVB phototherapy in the treatment of this condition.

Kreutz et al (2012) noted that depletion of host Langerhans cells (LCs) prevents cutaneous graft-versus-host disease (GVHD) in mice. These researchers analyzed whether UVB irradiation is tolerated during the course of human allogeneic hematopoietic cell transplantation and whether depletion of LCs by broadband UVB could improve GvHD outcome. A total of 17 patients received 6 whole-body UVB irradiations with 75% of the individually determined minimal erythemal dose after conditioning with a reduced intensity protocol. Langerhans cells, dermal dendritic cells (DCs), and macrophages were analyzed before and after UVB irradiation by immunohistochemical analysis. Circulating blood cells and serum factors were analyzed in parallel. In striking contrast to previous data, this irradiation protocol was well-tolerated in all patients. Treatment with UVB decreased the number of LCs and also affected dermal DCs. Patients treated with UVB also had significantly higher 25-hydroxyvitamin D3 serum levels and higher numbers of circulating CD4+ FoxP3+ regulatory T cells. Strikingly, 9 out of 9 patients with complete LC depletion (less than 1 LC per field) developed only grade I GVHD or no GVHD up to day 100. The authors concluded that these results strongly suggest that prophylactic UVB irradiation post-transplant is safe and should be further explored as a clinical strategy to prevent acute (skin) GVHD.

Duarte et al (2010) noted that progressive macular hypomelanosis (PMH) is a common dermatosis; its cause is unknown and proposed treatments have had little effect. These researchers examined epidemiological aspects of PMH in patients referred to a phototherapy clinic between 1997 and 2008 and evaluated therapeutic response to PUVA (psoralen + UVA) photochemotherapy or narrow-band ultraviolet B (NB-UVB) phototherapy. A total of 84 patients with PMH were evaluated. After 16 phototherapy sessions, therapeutic response was classified as: unchanged, slightly improved (less than 50% of re-pigmentation), moderately improved (50 to 79% of re-pigmentation), much improved (80 to 99%) or cured (100%). After a minimum of 3 months, patients whose response was classified as cured or much improved were contacted by telephone to evaluate the persistence of the therapeutic response. Most of the patients were women (79%) and white (85%). Age at onset of PMH ranged from 13 to 36 years. PUVA was prescribed for 27 patients and NB-UVB phototherapy for 57. No significant difference was found between the outcomes obtained with PUVA and those obtained with NB-UVB phototherapy (Fisher's exact test; p < 0.05). The majority of patients (81%) had 50% or more re-pigmentation, with 65% being classified as cured or much improved. Nevertheless, there was a recurrence of the lesions in 72% of patients. The authors concluded that the fact that no patients were over 40 years of age suggested that PMH is a self-limiting disease. Both PUVA and NB-UVB are effective therapeutic options; however, they do not prevent recurrence of the disease. The main drawback of this study was its uncontrolled nature and small sample size.

Montero et al (2011) stated that PMH is an acquired disorder of skin pigmentation, which is mostly under-diagnosed. It is characterized by nummular hypo-pigmented lesions appearing on the trunk in young persons. Several treatment options are available, although topical clindamycin and benzoyl peroxide have been used traditionally. However, good results have recently been achieved using NB-UVB phototherapy. These researchers presented the case of a 13-year old girl with hypo-pigmented lesions on the trunk and limbs that had progressed over 1 year and that were diagnosed as PMH. The patient was initially treated with topical clindamycin and benzoyl peroxide. However, little improvement was seen and treatment was then started with NB-UVB phototherapy. After 25 sessions, with a total cumulative dose of 18 J/cm(2), the patient showed almost total re-pigmentation of the lesions. The treatment of PMH is often difficult, and very little is currently known about the treatment response in this disorder, as most reports have very small series of patients with a short disease progression time. The authors concluded that NB-UVB phototherapy has been shown to be effective, as seen in this patient, although in many cases, there is recurrence after the cessation of treatment.

Kim et al (2012) examined the clinical features of PMH in Koreans and determined the therapeutic efficacy of NB-UVB therapy in the management of PMH. These investigators performed an uncontrolled prospective study designed to evaluate the usefulness of NB-UVB therapy in PMH. A total of 23 patients with PMH were enrolled in the study. Of these, 17 patients underwent treatment with NB-UVB therapy once- or twice-weekly and were eligible for analysis. The remaining 6 patients were lost to follow-up before completion of the treatment. Re-pigmentation was evaluated by 2 dermatologists using photographic documentation. Narrow band-UVB therapy was used successfully in 9 of 16 patients (56.2%), who showed more than 90% re-pigmentation. They found that 13 of 16 patients (81.3%) experienced at least 50% re-pigmentation. The re-pigmented sites showed an excellent color match. No signs of recurrence have been detected in 11 of these 16 patients (68.7%) up to the present time (13.2 +/- 8.2 months of follow-up). The authors concluded that the findings of this study suggested that NB-UVB therapy is an effective and safe method for use in the treatment of PMH. Drawbacks of this study included a small number of subjects examined, and it was an uncontrolled and non-double-blind study.

Berg et al (1994) carried out a study in 22 patients with polymorphous light eruption (PMLE) who were prophylactically treated with UVA with and without trimethylpsoralen; 12 of the patients were treated during 2 consecutive springs with placebo during one spring and psoralens during the other. Eighteen of the patients improved after the therapy, but there was no clear-cut difference between the 2 regimens. As many as 12 patients got light eruptions during the treatment, but all but 1 continued with the therapy. The authors concluded that the findings of this study indicated that UVA alone is as good prophylactic therapy for PMLE as PUVA with trimethylpsoralen. However, because of the high incidence of provoked eruptions during therapy, the treatment may be difficult to handle for the patients themselves, at least during the initial treatment.

Fesq and colleagues (2003) stated that management of PLE should focus on basic preventative measures and additional therapeutic approaches, depending on the clinical condition. Polymorphous light eruption can be classified into 4 severity groups:

mild,
moderate-to-severe,
severe, and
therapy-resistant.

These classifications are useful for determining appropriate prophylactic measurements. No specific laboratory tests were available for the diagnosis of PLE, therefore, a clinician must rely on the clinical appearance of the disorder (e.g., clinical symptoms, the location of the lesions, the relationship of the occurrence of the lesions with sun exposure and the time course of the lesions) as well as a patient's medical history in order to make a diagnosis. Basic preventative management of PLE consists of adequate sun protection comprising avoidance of sun exposure, the use of textile sun protection and the application of broadband sunscreens with high UVA protection potential. Other supportive measurements have to be managed individually and are dependent on the patient's medical history and the severity of the disease. Topical anti-oxidants, systemic immunomodulation, photo(chemo)therapy and systemic immunosuppression may be required in some cases of PLE. Topical anti-oxidants represent a new treatment approach for moderate-to-severe PLE and are an effective and well-tolerated option for this patient population. Severe PLE also requires photo(chemo)therapy. Phototherapy can be in the form of 311-nm UVB or UVA1 irradiation. In cases where 311-nm UVB or UVA1 are ineffective, psoralen plus UVA (PUVA) bath therapy may be used. However, PUVA bath therapy must be used with caution because it is associated with acute and long-term adverse effects. In rare exceptions we would consider using oral PUVA therapy. However, in our outpatient department, quality of life of most patients is improved with the treatment regimens that are recommended for patients with moderate-to-severe PLE, without the need for photo(chemo)therapy.

An UpToDate review on “Polymorphous light eruption” (Elmets, 2013) states that that PUVA plus psoralens is considered second-line therapy for PMLE.

First-line therapies Includes the following:

Sun protection - Sun protection is first-line therapy for patients with PMLE and includes sun avoidance, sun protective clothing, and sunscreens. Sunscreens should be broad spectrum, with both UVA and UVB protection. A sunscreen with an SPF (sun protection factor) of at least 30 should be regularly applied. Products containing photostabilized avobenzone or ecamsule (Mexoryl SX) offer improved protection against UVA, and have been effective in preventing PMLE eruptions. Sunscreens that contain the non-micronized form of zinc oxide or titanium dioxide also offer photoprotection that extends throughout the UV and into the visible spectrum. Examples of broad spectrum sunscreens containing photostabilized avobenzone or ecamsule, or zinc oxide and titanium oxide are provided.
Topical corticosteroids - No randomized trials have evaluated the efficacy of topical corticosteroids for PMLE. Clinical experience suggests that potent topical corticosteroids (groups one to three) may be used for symptomatic relief, and may be sufficient pharmacologic therapy for mild cases. Facial lesions should be treated with lower potency topical corticosteroids (groups six to seven).

Second-line therapies include the following:

Phototherapy - Prophylactic phototherapy with low dose PUVA (psoralens plus UVA) or UVB in early spring to induce tolerance to sun exposure may be an option for patients who are expected to develop significant symptoms during the spring or summer. Treatments are usually given 2 to 3 times per week over 5 to 6 weeks.
PUVA therapy is superior to broadband UVB. In one randomized trial, treatment was successful in 92% of patients treated with PUVA, compared with 62% of patients treated with broadband UVB. In contrast, a small randomized trial showed narrowband UVB to be as effective as PUVA. Because narrowband UVB is easier to administer, it is often preferred to PUVA therapy for patients with PMLE. Narrowband UVB phototherapy can be administered 3 times per week, starting with a dose equivalent to 50 to 70% of the MED. The dose is increased during subsequent treatments as tolerated by the patient.

Treatment-induced exacerbations may occur early in the course of phototherapy. If these occur, potent topical corticosteroids (groups one to three) or a short course of oral glucocorticoid therapy (e.g., prednisone 1 mg per kg for 7 to 10 days) may be required.

Samson et al (2003) reviewed the evidence regarding NB-UVB for the treatment for vitiligo, pruritus, and inflammatory dermatoses. This was a retrospective review of the treatment outcomes of 117 consecutive patients with vitiligo, pruritus, and other inflammatory dermatoses, excluding those with psoriasis and cutaneous T-cell lymphoma (CTCL), who were treated with NB-UVB between 1998 and 2001 at the authors’ institution. Approximately 80% of all patients showed improvement in their condition. Narrow-band UVB phototherapy was well-tolerated, with no serious adverse effects. In patients with vitiligo, 6.4% had an abnormal thyroid-stimulating hormone level and 6.5% had anemia. The authors concluded that NB-UVB may be considered as a viable therapeutic option in the treatment of vitiligo, pruritus, and other inflammatory dermatoses. Moreover, they stated that long-term adverse effects and cost-benefit analysis of NB-UVB therapy compared to other treatment modalities remain to be determined.

Gambichler et al (2005) provided an update on clinical experiences in NB-UVB of non-psoriatic skin conditions, and established its current position within the spectrum of competing photo(chemo)therapeutic options. The computerized bibliographic database PubMed, without time limits, and other sources were screened for clinical trials on NB-UVB. Included were research articles of randomized controlled trials (RCTs), open prospective studies, and retrospective observations on NB-UVB in skin disorders other than psoriasis. A total of 28 articles met eligibility criteria including 6 RCTs, 16 open prospective studies, and 6 retrospective observations. Narrow-band UVB is effective in patients with chronic atopic dermatitis (AD) (n = 719) and generalized vitiligo (n = 305) and appears to have some advantages over competing photo(chemo)therapeutic regimens. Narrow-band UVB also seems to be effective in patients with PMLE (n = 25), early stages of CTCL (n = 108), chronic urticaria (n = 88), lichen planus (n = 15), pruritus associated with polycythemia vera (n = 10), seborrheic dermatitis (n = 18), actinic prurigo (n = 6), and acquired perforating dermatosis (n = 5). The quality of evidence determined for the afore-mentioned diagnoses ranged from high to moderate to very low. The authors concluded that the best currently available data on NB-UVB in non-psoriatic conditions exist for AD and generalized vitiligo. In view of its efficacy, benefit/risk profile, and costs, NB-UVB may be considered the first-line photo(chemo)therapeutic option for moderately severe AD and widespread vitiligo. In the treatment of most other non-psoriatic conditions, NB-UVB appears to be effective, but current data allow no definitive conclusions as to whether NB-UVB should be preferred to competing photo(chemo)therapeutic options such as PUVA regimens. Because NB-UVB may have a wider indication spectrum, including AD, vitiligo, and early-stage CTCL, and appears to be equally effective or even more effective than broad-band UVB (BB-UVB), a switch from BB-UVB to NB-UVB seems to be justified. This study had a small number of patients with PMLE (n = 25).

Pugashetti and colleagues (2010) noted that BB-UVB phototherapy has demonstrated effectiveness in the treatment of cutaneous disorders including psoriasis, AD, uremic pruritus and idiopathic pruritus. In the last decade, there has been a rapidly escalating process of replacing BB-UVB phototherapy units with NB-UVB equipment, as studies have demonstrated that NB-UVB (ranging from 311 mm to 312 nm) is more effective in the treatment of psoriasis. Nevertheless, it is important to recognize the efficacy of BB-UVB phototherapy in the treatment of uremic pruritus, idiopathic pruritus, eosinophilic folliculitis and other inflammatory pruritic conditions. Furthermore, as high-lighted in this report, there was a small but significant proportion of psoriasis and AD patients who do not tolerate NB-UVB but demonstrated an excellent clinical response to BB-UVB. It is critical for dermatologists to recognize the role of BB-UVB as a complement to NB-UVB phototherapy for patients who cannot tolerate or experience an inadequate therapeutic response from NB-UVB. This study did not mention PMLE as an indication of BB-UVB or NB-UVB.

Walker and Jacobe (2011) stated that dermatologists are presented with a diversity of therapeutic modalities for the treatment of inflammatory, sclerosing, and neoplastic conditions, but with the development of various new irradiation devices that utilize specific parts of the electromagnetic spectrum, phototherapy has become a more viable, accessible, and effective option in the treatment of these conditions. The UV range (10 to 400 nm) is further sub-divided into UVA and UVB, each of which has been particularly useful in a number of skin conditions. The most commonly used forms of UV irradiation are UVA1, PUVA, and NB-UVB. Each of these modalities differ in their mechanism of action, indications, and side effect profiles, and it is important that clinicians be familiar with these differences. Today, phototherapy is a valuable option in the treatment of many non-psoriatic conditions including AD, sclerosing skin conditions such as morphea, vitiligo, and mycosis fungoides. Due to its relative safety, phototherapy may be used in most populations, including children and pregnant women. However, contraindications and side effects are known and should be considered before patients begin a phototherapeutic regimen. Again, this study did not mention PMLE as an indication of BB-UVB or NB-UVB.

Veith et al (2011) noted that phototherapy is used for the medical care of cutaneous conditions that do not respond to topical or systemic medical agents, and for conditions that require broad exposure to UV as a stabilizing agent for disease. Numerous wavelengths and delivery devices of UV light are used in childhood. This article was a brief overview of the medical usage of phototherapy in childhood. In the neonatal nursery blue light (459 to 460 nm) is used to reduce bilirubin levels and prevent kernicterus. While PUVA has been demonstrated to be effective in a variety of pediatric skin conditions, NB-UVB therapy (311 nm) has largely replaced PUVA as initial choice in full-body phototherapy for children. The latter is easier to deliver, with less resultant erythema than systemic PUVA, which requires strict use of 24-hour protective eyewear. Narrowband-UVB is therefore preferred for stabilization and clearance of a variety of inflammatory and autoimmune conditions especially AD, psoriasis and vitiligo. Conditions with lymphocytic infiltration, including mycosis fungoides, alopecia areata and pityriasis lichenoides can improve with NB-UVB as well. Alternatively, localized delivery of NB-UVB can be performed using the Excimer laser (308 nm), which has been described for the therapy of vitiligo and alopecia areata in childhood. Some diseases with dermal infiltration including morphea and mastocytosis may do better with PUVA or UVA1. Delivery of psoralens can also be performed topically for said conditions and in the setting of alopecia areata, thereby limiting UVA exposure, while retaining efficacy. Phototherapy can be a helpful adjunct in pediatric skin disease, but is limited by compliance issues. Parents can act as partners in the safe and effective delivery of phototherapy by standing outside the booth or inside with the child to ensure lack of movement and to aid in maintenance of eyewear. Choice of type of phototherapy and close monitoring, with parental partnership, is the key to successful treatment. Again, this study did not mention PMLE as an indication of BB-UVB or NB-UVB.

Also, an UpToDate review on “Polymorphous light eruption” (Elmets, 2013) does not mention the use of photochemotherapy/Goeckerman treatment as therapeutic options. The review provides several “Summary and Recommendations”:

UVA is the most common inciting spectrum of light, but UVB and visible light may also provoke PMLE in some patients
Primary treatment for PMLE includes sun avoidance, sun-protective clothing, and sunscreen. Broad spectrum sunscreens with an SPF of at least 30 should be regularly used
For patients with active lesions, we suggest treatment with potent topical corticosteroids (groups one to three). An alternative in patients with infrequent exacerbations, particularly those who require rapid improvement, is a short course of systemic glucocorticoids
For patients who develop frequent exacerbations during the spring and summer, we suggest prophylactic phototherapy in early spring
Juvenile spring eruption is a variant of PMLE that is manifested by erythematous papules or bullae typically on ears of children or adolescents after sun exposure. Symptoms are self-limited and resolve within several weeks

Note: Regarding UVB in the treatment of prurigo nodularis, Ferrandiz et al (1997) reported that an excellent response was obtained after an average of 32 UVB courses.

Note: Delrosso et al (2008) reported that an aggressive bath PUVA treatment is not substantially more effective in clearing chronic plaque-type psoriasis than a milder therapeutic approach.

Tan et al (2010) reviewed the efficacy and tolerability of NB-UVB phototherapy in children at a tertiary center in New Zealand, and determined if there were any factors that differentiated responders from non-responders. These researchers performed a prospective analysis of children (less than 16 years old) who had undergone phototherapy over a 15-year period. A total of 116 children received phototherapy with a total of 144 courses. Mean age was 11.0 years with the majority being European and having skin phototype II. Atopic dermatitis was the most common indication for treatment followed by psoriasis, pityriasis lichenoides, nodular prurigo, morphea, vitiligo, urticaria pigmentosa and erythropoietic porphyria. Treatment was effective in the majority of children (72%). Most received only 1 course. For responders, the mean number of treatments was 32.4. The mean dose per treatment to achieve clearance was 886 mJ/cm(2) and the mean maximum treatment dose per treatment was 1,328 mJ/cm(2). All children tolerated treatment well with 36% developing brief, minimally symptomatic, erythema. Only 2 children experienced exacerbations of their underlying dermatoses. The authors concluded that the findings of this study showed that phototherapy is an effective and well-tolerated treatment modality in children.

Weibel (2012) stated that localized scleroderma or morphea is a sclerosing connective tissue disease of the skin, which may affect underlying tissues such as subcutis, muscle and bone. Many patients show extra-cutaneous symptoms and anti-nuclear antibodies, however, secondary transformation into systemic sclerosis does not occur. Localized scleroderma usually begins in childhood with a wide variation in its clinical spectrum. The linear variant is the most common subtype in children, associated with a progressive course and increased risk of complications. The disease may progress over years and result in severe functional and cosmetic disability. The etiology of localized scleroderma remains unknown. A genetic background is suspected, while triggers such as trauma, vaccinations and infections may lead to secondary immunologic phenomena. Localized scleroderma often remains unrecognized for a long time, resulting in substantial delay in treatment. The combination of systemic corticosteroids and methotrexate has been established as first-line therapy for progressive (usually linear) disease, whereas phototherapy (UVA-1 or NB-UVB) is suitable for adolescents with superficial circumscribed subtypes.

Newland and Marshman (2012) stated that post-irradiation morphea is a rare but under-recognized complication of radiotherapy treatment for breast cancer. Management of this condition is difficult, and many cases are recalcitrant to therapy. A 43-year old woman with breast cancer received radiotherapy following a mastectomy and partial axillary lymph node dissection, shortly after which she developed a hot, tender, erythematous and indurated plaque at the mastectomy site. Subsequently the skin became retracted, depressed and hyper-pigmented. The clinical features, along with histological findings, were consistent with post-irradiation morphea. Treatment with NB-UVB and acitretin 10 mg daily was commenced 5 years following radiotherapy. After 2 months of therapy the patient reported significant improvement in tenderness and range of left arm movement. Objectively the plaque was less indurated and softer to palpation. The authors proposed that this treatment regimen is an option in the management of post-irradiation morphea.

Spalek et al (2015) noted that radiation-induced morphea (RIM) is a rare and under-recognized skin complication of radiotherapy. It is commonly wrongly diagnosed as other dermatological conditions or malignancy because of similar clinical characteristics. This literature review analyzed 66 cases that have been reported in the literature since 1989. The clinical appearance often includes pain and disfiguration of affected area, which may influence the patient's quality of life. There is no clear connection between the radiotherapy dose, the fractionation scheme, the use of a boost, age, the presence of other dermatological conditions or other connective tissue diseases and the occurrence of RIM. Its pathogenesis is still unclear, but several theories are proposed to explain this phenomenon. The available data suggested that the abnormally high secretion of some cytokines (interleukin 4, interleukin 5, transforming growth factor) induced by radiation causes an extensive fibrosis after an activation of fibroblasts. Histological confirmation is crucial in distinguishing RIM from similar-looking diseases, such as chronic radiation dermatitis, cancer recurrence, radiation, recall dermatitis, new carcinoma or cellulitis. There is no clear treatment regimen for this condition. Clinical outcome after therapy is often unsatisfactory. The commonly used methods and agents include: topical and systemic steroids, calcineurin inhibitors, systemic immunosuppressants including methotrexate, tacrolimus, heparin, hyaluronidase, phototherapy (UVA, UVA1, UVB, PUVA), systemic antibiotics, imiquimod, mycophenolate mofetil, photopheresis. The differential diagnosis is challenging and requires a multi-disciplinary approach to avoid misdiagnosis and to plan appropriate treatment.

Mizuno et al (2014) noted that Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by development of recurrent blisters, erosions, and crustations in the intertriginous areas. The treatment of HHD is often challenging, and various methods have been tried. These investigators reported the case of a 45-year old woman with a generalized form of HHD that was dramatically improved and well controlled by NB-UVB phototherapy. This preliminary finding needs to be validated by well-designed studies.

An UpToDate review on “Hailey-Hailey disease (benign familial pemphigus)” (Morrell, 2014) does not mention phototherapy/NB-UVB as a therapeutic option.

Treister et al (2015) evaluated the safety and effectiveness of intra-oral NB-UVB phototherapy in the management of oral chronic GVHD (cGVHD). Patients with oral cGVHD were treated using a custom NB-UVB unit for a course of 24 phototherapy sessions. Treatments were initiated at 50 mJ/cm2 and increased by 10% at each visit unless toxicity was noted. Toxicity and response were assessed weekly. A total of 11 patients received a median of 22 (range of 4 to 39) NB-UVB treatments; 5 patients completed 24 treatments and elected to receive a median of 7 additional treatments. Median symptom scores (0 to 10) for sensitivity, pain, and dryness at baseline/end of therapy were 7.5, 3, 1, and 3, 1, 2, respectively. Taking into account all patient-reported outcomes, 7/11 patients had improvement and 2/11 worsened. At least partial improvement was reported in 8/11 patients with none reporting worsening. Over-treatment occurred in 10/11 patients with all graded mild or moderate and resolving in 1 to 2 days. The authors concluded that intra-oral NB-UVB may be effective for management of refractory oral cGVHD. They stated that further optimization of treatment parameters, as well as minimal erythema dose testing, and inclusion of a control arm are needed in the consideration of future studies.

The American Academy of Dermatology (AAD) state that "topical agents form the mainstay of treatment in psoriasis and all other treatment modalities are often used with concomitant topical therapy. Emollients increase the transmission of UV radiation by altering the optical properties of psoriatic skin lesions and improving therapeutic efficacy. Application of a thin layer of emollient such as petrolatum before UV exposure is traditionally used. However, there are no randomized controlled studies to prove the benefit of concomitant use of emollients with UVB. It is important to pay attention to the application of sunscreens or salicylic acid containing preparations that may interfere with the penetration of UV radiation. UV blocking properties may be used to cover uninvolved skin with preparations such as zinc oxide to prevent unnecessary exposure and adverse effect" (AAD, 2018).

UVB for the Treatment of Photodermatoses

Collins and Ferguson (1995) reported that 20 patients with photodermatoses [actinic prurigo (n = 6), hydroa vacciniforme (n = 4), idiopathic solar urticaria (n = 1), amiodarone-induced photosensitivity (n = 1) and a range of cutaneous porphyrias (n = 8)] were treated with a “hardening” course of narrow-band ultraviolet (UV) B (TL-01) phototherapy in springtime. The response to phototherapy was monitored subjectively, by interviewing patients after the summer, and objectively by monochromator photo-testing, before and after phototherapy. Fifteen patients reported that treatment was worthwhile. Monochromator photo-testing after phototherapy revealed a 4-fold increase in the minimal erythema dose (MED) in those with abnormal photosensitivity to ultraviolet A wavebands. Adverse effects included erythema (n = 7), pruritus (n = 5) and provocation of the eruption (n = 4). The authors concluded that they now routinely consider narrow-band UVB phototherapy for problem photodermatoses.

Gupta et al (2000) stated that hydroa vacciniforme (HV) is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. The lesions typically heal with vacciniform scarring. These researchers identified and reviewed the clinical features and follow-up data of Scottish patients with HV and reported on the prevalence of this condition. They noted that this was the largest recent study of HV patients from a single center. In this retrospective study, patients with HV were identified by means of the diagnostic database from the Photobiology Unit, Dundee. Patients were contacted and details of clinical features, duration of disease, results of investigations, and treatment were recorded. At review, disease progress was assessed. Between 1973 and 1997, a total of 17 patients (9 males and 8 females) with a diagnosis of HV were investigated. Data from 15 patients showed a mean age at onset of 7.9 years (range of 1 to 16 years), with females (mean of 6.7 years; range of 2 to 12 years) having an earlier onset than males (mean of 8.7 years; range of 1 to 16 years). A bi-modal age distribution was also identified with onsets between the ages of 1 and 7 years and 12 and 16 years. At review, spontaneous clearing had occurred in 9 patients (60%) with mean duration of disease being 9 years (range of 4 to 17 years). Males had longer disease duration (mean of 11 years; range of 5 to 17 years) than females (mean of 5 years; range of 4 to 7 years). Eight patients (53%) were sensitive in the UVA wave-band on monochromator photo-testing, and 6 (40%) experienced papulo-vesicular lesions on repetitive broad-spectrum UVA irradiation. All patients received broad-spectrum sunscreens with variable results. Of the 5 patients treated with narrow-band UVB (TL-01) phototherapy, 3 reported beneficial results with an increase in tolerance to sunlight exposure and associated reduction in disease severity. The authors concluded that the estimated prevalence of HV was at least 0.34 cases per 100,000 with an approximately equal sex ratio. Males had a later onset and longer duration of disease than females. Photo-testing showed abnormal responses in the UVA wavebands in 53% of cases, whereas 60% of patients treated with prophylactic TL-01 phototherapy found it beneficial.

Dummer et al (2003) stated that polymorphous light eruption (PLE), a type of idiopathic photodermatoses, is an eruption induced by ultraviolet (UV) radiation (UVR). These researchers evaluated the clinical aspects, diagnostic criteria of PLE in a major Swiss referral center. A total of 25 patients (22 women and 3 men) with PLE were tested with a standardized protocol for the assessment of photodermatoses. Papular and papular-vesicular eruptions were the most common clinical presentations; 6 of 25 patients had a reduced MED for UVA and 8 of 25 patients had a reduced MED for UVB. Photo-provocation was positive in 11 of 20 patients for UVA and 7 of 20 patients for UVB. Photo-hardening with narrow-band UVB was successful in 8 of 10 patients. Combined UVA/UVB therapy had a satisfactory effect in 10 of 15 patients. Narrow-band UVB therapy was still successful after ineffective UVA/UVB therapy. The authors concluded that MED was of no value for the diagnosis of PLE. The typical lesions were reproduced by UVA and UVB photo-provocation. These investigators recommended photo-hardening with narrow-band UVB (311 nm).

Fesq et al (2003) stated that optimal management of patients with PLE, the most frequent photodermatosis, requires knowledge of the individual clinical course of the disease and pathogenic factors. As PLE often causes problems during leisure-time activities and holidays, resulting in a substantial loss of quality of life, prophylaxis is the most important therapeutic approach. Management of PLE must, therefore, focus on basic preventative measures and additional therapeutic approaches, depending on the clinical condition. PLE can be classified into 4 severity groups (mild, moderate-to-severe, severe and therapy-resistant), which are useful for determining appropriate prophylactic measurements. No specific laboratory tests are available for the diagnosis of PLE, therefore, a clinician must rely on the clinical appearance of the disorder (e.g., clinical symptoms, the location of the lesions, the relationship of the occurrence of the lesions with sun exposure and the time course of the lesions) as well as a patient's medical history in order to make a diagnosis. Basic preventative management of PLE consists of adequate sun protection comprising avoidance of sun exposure, the use of textile sun protection and the application of broad-band sunscreens with high UVA protection potential. Other supportive measurements have to be managed individually and are dependent on the patient's medical history and the severity of the disease. Topical antioxidants, systemic immunomodulation, photo(chemo)therapy and systemic immunosuppression may be required in some cases of PLE. Topical antioxidants represent a new treatment approach for moderate-to-severe PLE and are an effective and well-tolerated option for this patient population. Severe PLE also requires photo(chemo)therapy. Phototherapy can be in the form of 311 nm UVB (narrow-band UVB) or UVA1 irradiation. In cases where 311 nm UVB or UVA1 are ineffective, psoralen plus UVA (PUVA) bath therapy may be used. However, PUVA bath therapy must be used with caution because it is associated with acute and long-term adverse effects. In rare exceptions the authors would consider using oral PUVA therapy. However, in their outpatient department, quality of life of most patients is improved with the treatment regimens that are recommended for patients with moderate-to-severe PLE, without the need for photo(chemo)therapy.

Khaled et al (2011) stated that chronic actinic dermatitis (CAD) is a debilitating photodermatosis with characteristic clinical, histological and photo-biological features (reduced MED). Its management involves various therapeutic approaches, among them there is phototherapy. Efficacy of psoralen ultraviolet therapy (PUVA therapy) was previously demonstrated but there are no current data on the use of narrowband UVB therapy (NB-UVB) in CAD. NB-UVB has already been proven to be effective and safe in several other photodermatoses. These researchers reported the findings of 2 dark-skinned patients (skin type IV and V) with CAD, successfully treated with an incremental regimen of NB-UVB phototherapy coupled to a 3 month-course of systemic steroids (1 mg/Kg/day). The authors concluded that their protocol of NB-UVB with steroids appeared to be effective for the management of CAD with a good short-term safety profile.

An UpToDate review on “Photosensitivity disorders (photodermatoses): Clinical manifestations, diagnosis, and treatment” (Elmets, 2015) states that “Actinic prurigo -- Phototherapy with narrowband UVB or psoralen plus ultraviolet A (PUVA) is a therapeutic option in patients with persistent symptoms …. Chronic actinic dermatitis -- Low-dose PUVA or narrowband UVB, initially given with oral glucocorticoids to decrease treatment-induced flares, has been effective in small case series …. Solar urticaria -- Pharmacologic therapy or photodesensitization with low-dose PUVA or narrowband UVB initially given with oral glucocorticoids is usually required for management …. ”.

PUVA for Eosinophilic Cellulitis (Wells Syndrome)

Raßler et al (2016) noted that eosinophilic cellulitis (Wells syndrome) is a rare inflammatory skin disease defined by erythematous, tender, sometimes urticarial plaques, possibly with vesicles and bullae, and granulomatous eosinophilic infiltrates in the dermis. Usually the disease has a benign course with spontaneous remission within a few weeks. Nevertheless, recurrences are quite frequent and may occur for several years. These investigators reviewed the therapeutic options for Wells syndrome in a systematic manner, which was based on a search on Medline, Embase and Cochrane Central Register for English and German articles from 1970 to 2015. Advices on the treatment of Wells syndrome were limited predominately to case reports or to small case-series studies. There were no RCTs, and control groups were missing. A variety of therapeutic options for Wells syndrome were reported including anti-histamines, anti-malarial medications, azathioprine, colchicine, cyclosporine, dapsone, doxycycline, griseofulvin, interferon alpha and gamma, minocycline, oral tacrolimus/topical tacrolimus, PUVA therapy, sulfasalazine, tumor necrosis factor (TNF)-alpha inhibitors, as well as topical and systemic corticosteroids. As well-designed RCTs are missing, no guidelines for the treatment of this disease can be given. The authors noted that due to the small number of patients and the frequent misdiagnosis of this clinical entity, the aim of this systematic overview was to call attention to this rare condition and to help clinicians to diagnose and treat Wells syndrome effectively. The authors concluded that due to the good prognosis and tendency to resolve, systemic treatment should be limited to cases resistant to local therapy or with widespread lesions.

PUVA for Necrobiotic Xanthogranuloma

Miguel and colleagues (2017) stated that necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the peri-orbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. These researchers summarized all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query “necrobiotic xanthogranuloma treatment”, both in English and German, about “human” subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Therapeutic options include azathioprine, chlorambucil, cladribine, cyclophosphamide, extracorporeal photopheresis, fludarabine, high-dose intravenous immunoglobulin (IVIG), hydroxychloroquine, infliximab, interferon alpha, laser therapy, melphalan, methotrexate, plasmapheresis, PUVA, radiotherapy, rituximab, surgery, thalidomide, as well as topical and systemic corticosteroids. The authors stated that RCTs and studies on long-term outcomes after treatment were not found and are needed to focus on in the future.

Phototherapy for Scleredema

An UpToDate chapter on scleredema (Kreuter, 2017) states that, for patients with functionally limiting or symptomatic scleredema that is not expected to resolve spontaneously, initial treatment with phototherapy is suggested. This is a grade 2C recommendation based upon a retrospective study and case reports. The authors stated that, when available, they prefer to use UVA1 phototherapy. Improvement in scleredema has also been reported with PUVA and narrowband UVB phototherapy.

Kalfa and associates (2015) noted that scleredema is a rare connective tissue disorder of unknown pathogenesis; and 3 types of scleredema have been described, based on its association with post-infection, monoclonal gammopathy and diabetes mellitus. These investigators reported a case of scleredema in which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and PUVA therapy. The authors concluded that treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.

Narrow-Band UVB for the Treatment of Chronic Urticaria

Khafagy and associates (2013) compared the effectiveness of PUVA versus NB-UVB in the treatment of chronic urticarial (CU). A total of 24 patients with CU were included and divided into 2 groups:

12 patients subjected to PUVA and
12 subjected to NB-UVB.

They were compared according to the urticaria Total Severity Score (TSS) before and after treatment, cumulative dose, and adverse events (AEs). There was a statistically significant decrease in urticaria TSS in both the NB-UVB- and PUVA-treated groups after than before treatment (p < 0.05), with no significant difference between both groups regarding the percentage of improved patients and the mean decrease of urticaria TSS (p > 0.05). Gastro-intestinal upset was reported at a significantly higher percentage in the PUVA-treated group than in the NB-UVB-treated group. The authors concluded that both NB-UVB and PUVA showed comparable effectiveness in the treatment of CU with minimal reversible AEs.

In a randomized, prospective, observer-blinded comparative study, Bishnoi and colleagues (2017) compared the effectiveness of PUVA and NB-UVB in steroid-dependent anti-histamine-refractory CU. A total of 50 patients with steroid-dependent CRU (6 months of spontaneous urticaria with no response after 3 consecutive months of anti-histamines and steroid dependence) were administered either PUVA (group A) or NB-UVB (group B) for 90 days, with a post-treatment follow-up of 90 days. Treatment effectiveness was assessed using the average urticaria activity score 7 (aUAS7) and outcome scoring scale (OSS) every 2 weeks. The mean values of aUAS7 progressively decreased from 4.9 ± 0.8 and 5.0 ± 0.7 at baseline to 1.9 ± 0.7 and 1.4 ± 0.7 in groups A and B, respectively, by day 90. This further decreased to 1.5 ± 0.8 and 1.4 ± 1.0 at day 180 in both groups. The values of OSS progressively increased from baseline (1.6 ± 0.5 in group A and 1.3 ± 0.5 in group B) to 3.9 ± 0.3 and 4.0 ± 0.3 in groups A and B, respectively, by day 90, and 3.9 ± 0.5 and 4.0 ± 0.6 by day 180. NB-UVB fared statistically better than PUVA at different time-points; AEs encountered were minimal and did not warrant treatment discontinuation. The authors concluded that phototherapy, especially NB-UVB, is a safe and effective therapeutic modality for steroid-dependent CU and should be tried prior to 3rd-line treatment options (e.g., cyclosporine and omalizumab).

Narrow-Band UVB for the Treatment of Alopecia Mucinosa

An Medscape review on “Alopecia Mucinosa” (Gerstner, 2017) stated that “No uniformly effective therapy exists for alopecia mucinosa, although several treatments are routinely used. Treatments include topical, intralesional, and systemic corticosteroids. In addition, topical and systemic psoralen plus ultraviolet A light (PUVA) therapy, topical nitrogen mustard, and radiation therapy have demonstrated some success. Isolated cases document the beneficial responses of dapsone, indomethacin, and interferons. Because of the variable course of the disease and the likelihood of spontaneous resolution, therapeutic efficacy is difficult to prove”.

Furthermore, an UpToDate review on “Approach to the patient with a scalp disorder” (Goldstein and Goldstein, 2017) does not mention narrow-band UVB as a therapeutic option.

Narrow-Band UVB Phototherapy for the Treatment of Cutaneous Mastocytosis

Brazzelli et al (2012) stated that mastocytoses represent a heterogeneous group of stem cell disorders marked by an abnormal hyperplasia and accumulation of mast cells in one or more tissues, including bone marrow, gastro-intestinal (GI) tract, liver, spleen, lymph nodes and skin. Indolent systemic mastocytosis (ISM) is characterized by red-brownish and pruriginous maculopapular lesions, a bone marrow infiltration without functional impairment and an indolent clinical course with a good prognosis. In particular, the most common cutaneous symptoms are urticarial rash and mild-to-high pruritus. These researchers analyzed the clinical outcome of patients affected by ISM with prevalent pruriginous cutaneous symptoms and a scarce response to antihistamines treated using narrowband ultraviolet B (NB-UVB) phototherapy, which was administered in a UV-irradiation cabin equipped with fluorescent UVB lamps with a peak emission at 311 to 313 nm. The perception of pruritus severity was examined using the visual analogue scale (VAS) before starting the treatment and at each control. A complete remission of the cutaneous lesions and pruritus was documented in all patients after a median of 40.3 UV treatments and a median cumulative dose of 51.4 J/cm(2), with a lasting remission over a 6-month follow-up. The median VAS score at the beginning of the treatment was 86.6 (SD = 6.64), whereas it decreased to 6.66 (SD = 3.75) after 3 months of therapy. The authors concluded that the findings of this study provided evidence that NB-UVB phototherapy was useful for the treatment of the cutaneous symptoms and pruritus in ISM.

Brazzelli et al (2016) noted that in mastocytosis, the skin is almost invariably involved, and cutaneous symptoms deeply affect patients' quality of life (QOL). In a retrospective, observational study, these researchers analyzed the outcomes of patients affected by cutaneous mastocytosis (CM) and ISM treated with phototherapy/photochemotherapy (PUVA or NB-UVB). For each patient, total numbers of PUVA or NB-UVB exposures, the cumulative UV dose (J/cm2 ), serum tryptase profile, and pruritus, before and after treatment, according to the VAS were considered. Skin lesions of each patient were examined, before and after treatment, according to a cutaneous scale score. A total of 20 patients affected by CM and ISM were studied; in particular, 10 patients received NB-UVB therapy, and other 10 patients received PUVA. A statistically significant mean reduction of pruritus in both groups (p < 0.01) was observed. The number of treatments needed to attain symptom relief was significantly lower in the PUVA group, but the mean exposure dose was significantly higher, if compared to the NB-UVB group. Serum tryptase levels showed a downward trend. The cutaneous score improved in both groups. The authors concluded that this study provided evidence that both NB-UVB and PUVA represent a safe and useful 2nd-line therapy of the cutaneous symptoms in mastocytosis. Moreover, these investigators stated that the drawbacks of this trial were that it was a retrospective study with a small sample size (n = 10 in each of the 2 treatment groups) and without a control group.

Furthermore, an UpToDate review on “Cutaneous mastocytosis: Treatment, monitoring, and prognosis” (Castells and Akin, 2020) states that “Psoralen-ultraviolet A therapy (PUVA) or narrow band UVB decreases the number of mast cells and controls pruritus that cannot be managed with antihistamines alone. However, long-term use is associated with an increased risk of skin cancer, and the skin lesions usually recur after therapy is stopped. Phototherapy may be considered for temporary symptomatic relief in patients with diffuse cutaneous mastocytosis with extensive skin involvement refractory to medical management”.

Phototherapy for the Treatment of Grover's Disease (Transient and Persistent Acantholytic Dermatosis)

An UpToDate review on “Grover's disease (transient and persistent acantholytic dermatosis)” (Riemann and High, 2020) recommends PUVA among third-line options for treatment. The review states that there are no published randomized clinical trials of therapies for Grover's disease, and that nearly all evidence for therapies comes from case series, case reports, and clinical experience. High-potency topical corticosteroids, moisturizers, and emollients are typically recommended as first-line therapy. The review does not mention narrowband UVB as a therapeutic option.

Phototherapy for the Treatment of Lymphomatoid Papulosis

Guidelines from the National Comprehensive Cancer Network (NCCN, 2020) state that, when managing patients with lymphomatoid papulosis, it is important to be reminded that this is not a malignant disorder, but a recurrent, benign, self-regressing lymphoid proliferation. Observation is preferred for patients with asymptomatic disease. Topical steroids and phototherapy (citing data on PUVA for lymphomatoid papulosis) are the most commonly used skin-directed therapies for initial treatment of limited lesions as well as extensive lesions. Systemtic therapy is indicated only for persons with extensive lesions.

Tan and Giam (2004) stated that lymphomatoid papulosis is a chronic benign disease that may be associated with malignant lymphomas. This case illustrated the relapsing and remitting nature of both lymphomatoid papulosis and its potential of developing cutaneous T-cell lymphoma; and NB-UVB phototherapy as a new modality of treatment of early-stage mycosis fungoides in these patients. A 44-year old woman has had recurrent crops of papules and nodules of lymphomatoid papulosis on her limbs for 15 years. Histological features ere consistent with the type B lesions of lymphomatoid papulosis. Eight years after the initial onset of these lesions she developed cutaneous T-cell lymphoma (mycosis fungoides). Since then, she has had recurrence of mycosis fungoides following the cessation of phototherapy; but had no evidence of systemic involvement. The lesions of lymphomatoid papulosis responded to intermittent courses of oral methotrexate. Mycosis fungoides was treated with oral psoralen and UVA phototherapy with good response. Unfortunately, the lesions relapsed, whenever phototherapy was discontinued. The most recent recurrence of mycosis fungoides was treated with NB-UVB therapy. The papules of lymphomatoid papulosis continued to appear but she remained free of lesions of mycosis fungoides 10 months following cessation of NB-UVB therapy. The authors concluded that long-term surveillance was essential in all cases of lymphomatoid papulosis as accurate predictors for the development of malignant lymphoma in these individuals are still lacking. Moreover, these researchers stated that it would be interesting to examine the long-term safety and effectiveness of NB-UVB as compared with PUVA in the treatment of mycosis fungoides in individuals with lymphomatoid papulosis.

An UpToDate review on “Lymphomatoid papulosis” (Kadin, 2020) states that “Narrowband UVB phototherapy or, if available, UVA1 (long-wave [340 to 400 nm] UVA) phototherapy, which do not require the oral or topical administration of psoralens, may be alternatives to PUVA. In one study, UVA1 phototherapy induced a complete response in 5 of 7 patients. Three patients relapsed between 1 and 20 months after discontinuation but responded to a second treatment cycle … For children with symptomatic lesions, scarring, or cosmetic concerns, we suggest topical corticosteroids or narrowband UVB therapy (Grade 2C)”.

Phototherapy (UVA and Narrow-Band UVB) for the Treatment of Uremic Pruritus

Simonsen and colleagues (2017) noted that uremic pruritus is a common and burdensome symptom afflicting patients with advanced chronic kidney disease (CKD) and has been declared a priority for CKD research by patients. The optimal treatments for uremic pruritus are not well defined. In a systematic review, these investigators reviewed the evidence on the various treatments for this condition; eligible subjects were adult patients with advanced CKD (stage greater than or equal to 3) or receiving any form of dialysis . PubMed, CINAHL, Embase, International Pharmaceutical Abstracts, Scopus, Cochrane Library, and ClinicalTrials.gov from their inception to March 6, 2017, were systematically searched for RCTs of uremic pruritus treatments in patients with advanced CKD (stage greater than or equal to 3) or receiving any form of dialysis; 2 reviewers extracted data independently. Risk of bias was assessed using the Cochrane Collaboration risk-of-bias tool. Any intervention for the treatment of uremic pruritus was included. A quantitative change in pruritus intensity on a visual analog, verbal rating, or numerical rating scale. A total of 44 RCTs examining 39 different treatments were included in the review. These treatments included gabapentin, pregabalin, mast cell stabilizers, phototherapy, hemodialysis modifications, and multiple other systemic and topical treatments. The largest body of evidence was found for the effectiveness of gabapentin. Due to the limited number of trials for the other treatments included, these researchers were unable to comment on their efficacy. Risk of bias in most studies was high. The authors concluded that despite the acknowledged importance of uremic pruritus to patients, with the exception of gabapentin, the current evidence for treatments is weak. They stated that large, simple, rigorous, multi-arm RCTs of promising therapies are urgently needed. The main drawbacks of this study were its heterogeneity in design, treatments, and outcome measures’ thus making comparisons difficult and precluded meta-analysis.

PUVA for the Treatment for Necrobiosis Lipoidica

Peckruhn and colleagues (2017) stated that necrobiosis lipoidica (NL) is a rare granulomatous disease of unclear etiology and is often seen in patients with diabetes. Characterized by its potential for ulcerations, it presents a serious burden for the afflicted patients. There are currently neither German nor European guidelines for the treatment of NL. At the same time, standard treatment with topical or intra-lesional corticosteroids does not always show satisfactory results. These researchers examined if the various therapeutic regimens published since 2000 have actually expanded the armamentarium in a relevant manner. Included were all publications that described more than 1 patient being treated with any given therapeutic modality. Overall, these investigators analyzed data for 16 different therapeutic regimens reported in 49 publications. The largest amount of data exists for topical PUVA therapy, photodynamic therapy (PDT), and systemic treatment with fumaric acid esters. However, this analysis showed that with an increase in the number of documented patients treated with a given therapeutic modality, the proportion of those achieving a complete response (CR) or partial response (PR) actually decreased. This was interpreted as publication bias. The authors concluded that no clear recommendation can be rendered for 2nd-line therapy in case topical or intra-lesional corticosteroids fail.

Furthermore, an UpToDate review on “Necrobiosis lipoidica” (Wanat and Rosenbach, 2017) stated that “For patients with nonulcerated necrobiosis lipoidica who cannot be managed adequately with local corticosteroids but still desire treatment, other interventions, such as topical tacrolimus, topical psoralen plus ultraviolet A (PUVA) photochemotherapy, systemic medications, and procedural therapies may be beneficial. Data to support the efficacy of these interventions are limited and insufficient for conclusions on the comparative efficacy of treatments”.

UVB for the Treatment of Kyrle Disease (Perforating Dermatosis)

An UpToDate review on “UVB therapy (broadband and narrowband)” (Honigsmann, 2019) states that “Acquired perforating dermatosis (APD, which encompasses Kyrle disease, acquired RPC, acquired PF, and acquired EPS and is often associated with underlying chronic renal failure or diabetes mellitus … Narrowband UVB is a low-cost treatment and may delay or avoid costly third-line treatments for many patients with inflammatory dermatoses”.

UVB for the Treatment of Papular Mucinosis

An UpToDate review on “Localized lichen myxedematosus” (Rongioletti, 2019) states that “Lichen myxedematosus (LM, also called papular mucinosis) is a chronic idiopathic cutaneous mucinosis characterized by lichenoid papules, nodules, and/or plaques; mucin deposition and a variable degree of fibrosis in the dermis; and an absence of associated thyroid disease … Localized LM is a benign condition for which treatment is not required. We typically reassure patients regarding the benign nature of localized LM and do not initiate treatment”.

UVB for the Treatment of Prurigo Nodularis

An UpToDate review on “Prurigo nodularis” (Watsky, 2019) states that “Patients with widespread or recalcitrant disease. Phototherapy -- For patients with widespread disease and for those with recalcitrant disease that does not respond to topical or intralesional corticosteroids, we suggest narrowband ultraviolet B (NBUVB) phototherapy as first-line therapy. NBUVB is administered 2 to 3 times weekly for up to 10 weeks in combination with topical corticosteroids. Oral antihistamines, the tricyclic antidepressants doxepin or amitriptyline, or gabapentin/pregabalin may be given as adjunctive treatment to control pruritus. Emollients and topical corticosteroids may also be used as needed. Psoralen plus ultraviolet A (PUVA) can be an alternative form of phototherapy if NBUVB is not available. Limited evidence for efficacy of phototherapy, including NBUVB, systemic and bath/topical PUVA, ultraviolet A1 (UVA1), and monochromatic 308 nm excimer light, for the treatment of PN is derived from small observational studies and a single randomized trial”.

UVB for the Treatment of Lichen Amyloidosis

An UpToDate review on “Cutaneous manifestations of amyloidosis” (Bohjanen and Miller, 2019) states that “Few studies have directly compared interventions, leaving uncertainty about relative efficacy. An open left-right comparison study that compared the efficacy of moderate to potent topical corticosteroids with the efficacy of ultraviolet B (UVB) or psoralen plus ultraviolet A (PUVA) phototherapy in 20 patients with a clinical diagnosis of lichen amyloidosis found a trend towards greater reductions in patient-reported itch and skin roughness with phototherapy, but the difference was not statistically significant … Treatment of primary localized cutaneous amyloidosis is not mandatory. Treatment is performed to improve symptoms and/or cosmesis. No treatment is consistently effective for macular amyloidosis and lichen amyloidosis. We suggest interventions to minimize pruritus and scratching as initial treatment (Grade 2C). Our initial treatment approach consists of local corticosteroid therapy to reduce pruritus. Other interventions that may be useful for patients who fail to improve with these conservative measures include phototherapy, laser, dermabrasion, and systemic medications”.

UVB for the Treatment of Refractory Uremic Pruritus

An UpToDate review on “Uremic pruritus” (Kobrin, 2020) states that ” Refractory pruritus -- Most patients with uremic pruritus will at least partially respond to emollients, topical analgesics and oral antihistamines or gabapentin. For patients who are refractory to these agents, ultraviolet B (UVB) phototherapy is a therapeutic option.”

Gilchrest et al (1977) examined the effect of ultraviolet (UV)-light phototherapy on severe persistent pruritus in 18 adult patients on hemodialysis. Patients were randomly assigned to 1 of 2 light sources. The experimental group received conventional sunburn-spectrum light (conventionally designated as UVB) in gradually increasing doses. The control group received time-matched exposures to long-wave ultraviolet (UV) light. All patients received 8 exposures to the entire skin surface over a 4-week treatment period; 9 of 10 patients in the sunburn-spectrum group reported marked decrease in pruritus as opposed to 2 of 8 in the placebo group (p < 0.01). Of those responding to sunburn-spectrum light, improvement usually occurred 2 to 3 weeks into treatment. Mild sunburn, noted by some patients in this group, was the only side effect . The response to phototherapy was unaffected by the presence of secondary hyperparathyroidism. The authors concluded that UV phototherapy was a safe, convenient, inexpensive and effective treatment for uremic pruritus. These researchers stated that these findings mandated further study of use of UVB in patients with uremic pruritis.

Ada et al (2005) reported the results of a pilot study of narrowband UVB (NB-UVB) phototherapy for the treatment of 20 patients with uremic pruritus; 10 patients completed the 6-week study period. A total of 8 patients were found to be responders. Of the remaining 10 patients who left the study before 6 weeks, 6 were satisfied with the response. In the follow-up period, 7 responders could be examined, and 3 were in remission 6 months after completing treatment. However, pruritus recurred in the remaining 4 responders. The authors concluded that NB-UVB phototherapy may be an effective treatment for patients with uremic pruritus; however, recurrence of pruritus was a frequent problem. Moreover, these researchers stated that randomized controlled trials (RCTs) are needed to better define the place of NB-UVB in the treatment protocol of this condition. In the meantime, these investigators were in the process of designing such a trial that would include 3 patient groups: one receiving NB UVB, and the others UVA (as a placebo) and broadband UVB (BB-UVB).

In a single-blind, randomized, controlled trial, Ko et al (2011) examined if NB-UVB phototherapy is an effective treatment for uremic pruritus. The treatment group received NB-UVB phototherapy 3 times/week for 6 weeks. The dose of NB-UVB started from 210 mJ cm(-2) and was increased by 10% each time. The control group received time-matched exposures to long-wave UVA radiation. A visual analog scale (VAS) score was evaluated weekly for pruritus intensity for 12 weeks. The characteristics of pruritus were also assessed by a questionnaire at baseline and after 6 weeks of phototherapy. Both the NB-UVB and control groups had significant and comparable improvement in the pruritus intensity VAS scores during the period of phototherapy and follow-up. Compared with the control group, the NB-UVB group showed a significant improvement in the involved body surface area (BSA) affected by pruritus (p = 0·006), but not in sleep quality. More detailed regression and estimating analysis revealed that the patients in the NB-UVB group had lower pruritus intensity scores at week 6, week 10 and week 12. This may indicate a beneficial difference at certain time-points, but the effect appeared marginal. The authors concluded that NB-UVB phototherapy did not show a significant effect in reducing pruritus intensity compared with a control group for refractory uremic pruritus. These researchers stated that further studies are needed.

NB-UVB Phototherapy for the Treatment of Cutaneous Mastocytosis

Furthermore, an UpToDate review on “Cutaneous mastocytosis: Treatment, monitoring, and prognosis” (Castells and Akin, 2021) states that “Psoralen-ultraviolet A therapy (PUVA) or narrow band UVB decreases the number of mast cells and controls pruritus that cannot be managed with antihistamines alone. However, long-term use is associated with an increased risk of skin cancer, and the skin lesions usually recur after therapy is stopped. Phototherapy may be considered for temporary symptomatic relief in patients with diffuse cutaneous mastocytosis with extensive skin involvement refractory to medical management”.

NB-UVB Phototherapy for the Treatment of Drug-Related Hypersensitivity Reaction

An UpToDate review on “UVB therapy (broadband and narrowband)” (Honigsmann, 2021) does not mention drug-related hypersensitivity reaction as an indication for UVB therapy.

NB-UVB Phototherapy for the Treatment of Erythema Annulare Centrifugum

Reuter et al (2007) noted that erythema annulare centrifugum is an acute dermatosis of unclear etiology, which presents with annular erythematous lesions with marginal scale. Therapeutically, systemic and topical glucocorticoids are used primarily. These investigators treated a patient with large lesions in the area of the thighs resistant to a therapy with topical glucocorticoids, with topical calcitriol in combination with 311-nm narrow band ultraviolet B (NB-UVB) phototherapy. After 4 weeks of treatment the skin lesions had cleared nearly completely without any side effects. The combination topical vitamin D3-analog calcitriol and 311-nm NB-UVB phototherapy was effective and can be regarded as a useful alternative to glucocorticoids for the treatment of erythema annulare centrifugum. This was a single-case study; and its findings were confounded by the combined use of topical glucocorticoids, topical calcitriol, and NB-UVB.

Furthermore, an UpToDate review on “Erythema annulare centrifugum” (Haeberle, 2021) does not mention NB-UVB as a management / therapeutic option.

NB-UVB Phototherapy for the Treatment of Lichen Sclerosus

Petersen et al (2018) stated that radiation induced morphea (RIM) is an increasingly common complication of radiation treatment for malignancy as early detection has made more patients eligible for non-surgical therapeutic options. In many cases, the radiation oncologist is the first person to learn of the initial skin changes, often months before a dermatologist sees them. These researchers presented the case of a breast cancer patient who developed a rare bullous variant of RIM, which delayed her diagnosis and subsequent treatment. It is imperative to diagnose RIM early as it carries significant morbidity and permanent deformity if left untreated. The lesions typically present within 1 year of radiation therapy and extend beyond the radiated field. RIM is often mistaken for radiation dermatitis or cellulitis. Bullae, when present, are often hemorrhagic in appearance, which can serve as another clinical clue. It is important to refer these patients for a full gynecologic examination as there can be concurrent anogenital lichen sclerosus et atrophicus, which is both debilitating and carries a long-term risk for squamous cell carcinoma. Treatment with systemic agents is often necessary, and can be managed by a dermatologist. The most proven regimen in the literature appeared to be methotrexate, with or without concurrent narrow-band UVB phototherapy.

The British Association of Dermatologists’ guidelines on “Management of lichen sclerosus” (Lewis et a, 2018) did not have a recommendation for ultraviolet light therapy.

Furthermore, an UpToDate review on “Vulvar lichen sclerosus” (Cooper and Arnold, 2021) does not mention narrow-band ultraviolet B (NB-UVB) as a management / therapeutic option.

NB-UVB Phototherapy for the Treatment of Lichenoid Dermatitis

An UpToDate review on “Lichenoid drug eruption (drug-induced lichen planus)” (Ziemer, 2021) states that “Lichenoid granulomatous dermatitis is a histopathologic reaction pattern with vacuolar alteration of the basal layer with necrotic keratinocytes and a chronic, inflammatory infiltrate consisting of lymphocytes, eosinophils, plasma cells, and macrophages forming variable types of granulomas. In more than one-third of the cases, the most common clinical correlates are drug eruptions … A special and rare subtype is giant cell lichenoid dermatitis, a rare condition considered an unusual variant of lichenoid drug eruption or a manifestation of sarcoidosis. Histopathologic findings include multinucleated giant cells … For patients with symptomatic disease involving a limited skin area (e.g., the extremities), we suggest topical corticosteroids rather than oral corticosteroids (Grade 2C). We generally use a super-potent topical corticosteroid (e.g., clobetasol propionate 0.05 %) ointment or cream twice daily for 2 to 4 weeks. Less potent topical corticosteroids, such as mometasone furoate 0.1 % ointment or cream, can be used for facial lesions … For patient with oral erosive lichenoid drug eruption, we suggest topical corticosteroids as first line treatment (Grade 2B). We typically use clobetasol propionate 0.05 % ointment 2 or 3 times per day for 4 to 8 weeks”. However, narrow-band UVB is not mentioned as a therapeutic option.

NB-UVB Phototherapy for the Treatment of Lymphomatoid Papulosis

In a case report, Tan and Giam (2004) reported on the findings of a 44-year-old woman with recurrent crops of papules and nodules of lymphomatoid papulosis and who had early-stage mycosis fungoides. The lesions of lymphomatoid papulosis responded to intermittent courses of oral methotrexate. Mycosis fungoides was treated with oral psoralen and ultraviolet A phototherapy with good response. Unfortunately, the lesions relapsed, whenever phototherapy was discontinued. The most recent recurrence of mycosis fungoides was treated with NB-UVB therapy. The papules of lymphomatoid papulosis continued to appear but she remained free of lesions of mycosis fungoides 10 months after cessation of NB-UVB therapy.

Furthermore, an UpToDate review on “Lymphomatoid papulosis” (Kadin, 2021) states that “For children with symptomatic lesions, scarring, or cosmetic concerns, we suggest topical corticosteroids or narrowband UVB therapy (Grade 2C)”.

Contraindications to Ultraviolet Phototherapy

An evidence-based analysis on “Ultraviolet phototherapy management of moderate-to-severe plaque psoriasis” (Medical Advisory Secretariat, 2009) noted that there are a range of contraindications for UVB phototherapy and for PUVA. Both treatments have contraindications including any history of light sensitivity disorders (i.e., lupus erythematosus, porphyria, cutanea tarda, xeroderma pigmentosum etc.), melanoma, squamous cell carcinoma, aphakia, and/or basal cell carcinoma. The safety for PUVA has also not been established in pregnancy, nursing mothers, or children. There are also contraindications for patients with significant hepatic impairment and for those taking warfarin or phenytoin.

An UpToDate review on “UVA1 phototherapy” (Krutmann and Morita, 2019) states that “Ultraviolet A1 (UVA1) phototherapy is contraindicated in patients with xeroderma pigmentosum, porphyria, melanoma and nonmelanoma skin cancer, and in patients on long-term immunosuppressive therapy (e.g., after organ transplantation). UVA1 phototherapy should not be used for patients with UVA-sensitive photodermatoses or photosensitive atopic dermatitis or patients taking photosensitizing drugs. The efficacy and long-term safety of UVA1 therapy has not been evaluated and therefore should be used with caution in patients younger than 18 years”.

An UpToDate review on “UVB therapy (broadband and narrowband)” (Honigsmann, 2019) lists lupus erythematosus and xeroderma pigmentosum as absolute contraindications as well as history of photosensitivity diseases (e.g., chronic actinic dermatitis, solar urticaria), history of melanoma, history of nonmelanoma skin cancer, history of treatment with arsenic or ionizing radiation because of the increased risk for skin cancer, and immunosuppression for organ transplant patients as relative contraindications.

Home Phototherapy (UVB) for the Treatment of Atopic Dermatitis (Eczema)

An UpToDate review on “Treatment of atopic dermatitis (eczema)” (Weston and Howe, 2020) does not mention home phototherapy as a management option.

Guidelines from the American Academy of Dermatology guidelines of care for the management of atopic dermatitis (Sidbury, et al., 2014) states that home phototherapy under the direction of a physician may be considered for patients who are unable to receive phototherapy in an office setting. The guidelines state that, although there are no studies that document the efficacy or safety of home light therapy for patients with atopic dermatitis, or that contrast its use to in-office phototherapy, results similar to home phototherapy for psoriasis might be expected.

Home Phototherapy for the Treatment of Cutaneous T-Cell Lymphoma (Mycosis Fungoides and Sezary Syndrome)

Milstein et al (1982) described the findings of 31 patients with early mycosis fungoides (MF) and 3 patients with parapsoriasis en plaques who were treated with ultraviolet (UV) phototherapy (280 to 350 nm) at home using a commercially available light source containing 4 Westinghouse FS40 lamps. A complete clinical and histologic remission of disease, lasting for a median duration in excess of 18 months, was achieved in 19 patients (61 %) with MF. Although higher complete response rates generally were achieved with other therapeutic modalities, UV phototherapy with its minimal adverse effects may be indicated for selected patients. Moreover, the authors concluded that controlled studies are needed to examine the full potential of conventional phototherapy in the management of MF.

Resnik et al (1993) noted that in 1982, they reported their preliminary observations on the use of home UV phototherapy for patch and early plaque phase MF. These researchers presented follow-up data of the original 31 patients, covering an interval of up to 15 years. All patients used a commercially available UV phototherapy unit that contained 4 Westinghouse FS40 fluorescent lamps for daily exposures of their non-sun-exposed skin regions. A complete clinical and histologic response to home phototherapy occurred in 23 patients (74 %) with a maximum duration of the response from 5 months to more than 15 years (median of 51 months). After maintenance phototherapy was discontinued, 7 patients (23% ) had a sustained disease-free interval lasting more than 58 months (median of greater than 90 months). This indicated that cure may have been achieved in a minority of patients. Phototherapy was well-tolerated without evidence of significant photo-damage or photo-carcinogenicity. The authors concluded that these findings indicated that home phototherapy may be a therapeutic option for treatment of selected patients with early MF.

In a review on “Phototherapy of mycosis fungoides” (Hodak and Pavlovsky, 2015), home phototherapy is not mentioned as therapeutic option.

A consensus statement of the United States Cutaneous Lymphoma Consortium on “Guidelines for phototherapy of mycosis fungoides and Sezary syndrome” (Olsen et al, 2016) noted that broadband-UVB, both home- and office-based, has been demonstrated to be safe but has fallen out of favor as demonstrated by a recent survey of cutaneous lymphoma experts, being largely supplanted by NB-UVB.

An UpToDate review on “Treatment of early stage (IA to IIA) mycosis fungoides” (Hoppe et al, 2021) states that “Both narrow-band ultraviolet B (NBUVB, 311 nm wavelength) and broad-band (BBUVB; 290 to 320 nm wavelength) have been used as skin-directed treatments for early-stage MF, although BBUVB emitting sources have mostly been replaced by NBUVB lamps worldwide. In retrospective cohort studies, NBUVB has demonstrated superior efficacy and decreased toxicity compared with BBUVB, but not PUVA, with complete response rates ranging from 54 to 90 %. However, there are no randomized trials evaluating the relative efficacy of these phototherapy modalities in patients with early-stage MF … NBUVB phototherapy is administered in a dermatology office 3 to 5 times per week with gradual incremental dose delivery. Improvement is generally seen after 20 to 40 treatments. After a complete response is achieved, the frequency of therapy is tapered very slowly during the maintenance period and then discontinued. The tapering schedule is non-standardized and differs by institution. As an example of a slow taper, after a complete response or plateau in response, treatments may be decreased from 3 times a week to twice-weekly for 1 to 2 months, then decreased to once-weekly for 1 to 2 months, followed by discontinuation of therapy”. This UTD review does not mention home phototherapy as a therapeutic option

Furthermore, National Comprehensive Cancer Network’s clinical practice guideline on “T-cell lymphomas” (Version 1.2022) does not mention phototherapy / UVB as a management option.

Table: CPT Codes / HCPCS Codes / ICD-10 Codes
Code	Code Description
*Information in the [brackets] below has been added for clarification purposes. Codes requiring a 7th character are represented by "+"*:
*PUVA*:
CPT codes covered if selection criteria are met:
96912	Photochemotherapy; psoralens and ultraviolet A (PUVA)
96913	Photochemotherapy (Goeckerman and/or PUVA) for severe photoresponsive dermatoses requiring at least 4-8 hours of care under direct supervision of the physician (includes applications of medication and dressings)
ICD-10 codes covered if selection criteria are met:
B20	Human immunodeficiency virus [HIV] disease
C84.00 - C84.09 C84.A0 - C84.A9	Mycosis fungoides and cutaneous T-cell lymphoma
C86.6	Primary cutaneous CD30-positive T-cell proliferations
D45	Polycythemia vera
D89.810 - D89.813	Graft-versus-host disease
L11.1	Transient acantholytic dermatosis [Grover]
L20.0 - L20.82 L20.84 - L20.9	Atopic dermatitis [severe refractory]
L29.0 - L29.9	Puritis [refractory uremic pruritus]
L40.0 - L40.9	Psoriasis [severe disabling, involving 10% or more of body or severe psoriasis involving the hands, feet or scalp]
L41.0 - L41.9	Parapsoriasis [severe]
L43.0 - L43.9, L66.1	Lichen planus [severe]
L56.4	Polymorphous light eruption
L56.8	Other specified acute skin changes due to ultraviolet radiation
L63.0 - L63.9	Alopecia areata
L80	Vitiligo
L92.0	Granuloma annulare
L94.0	Localized scleroderma [morphea]
Q82.2	Mastocytosis [Urticaria pigmentosa] [severe]
T86.09	Other complications of bone marrow transplant [skin conditions]
ICD-10 codes not covered for indications listed in CPB (not all-inclusive):
D76.3	Other histiocytosis syndromes. Reticulohistiocytoma (giant-cell); Sinus histiocytosis with massive lymphadenopathy; Xanthogranuloma
E85.4 [L99 also required]	Organ-limited amyloidosis [lichen amyloidosis]
L70.0 - L70.9	Acne
L81.1	Chloasma [melasma]
L98.3	Eosinophilic cellulitis [Wells]
L98.5	Mucinosis of the skin [lichen myxedematosus]
Q82.8	Other specified congenital malformations of skin [Darier-White]
ICD-10 codes contraindicated for this CPB:
C43.0 - C43.9	Malignant melanoma of skin
C44.00 - C44.99	Other and unspecified malignant neoplasm of skin
O00.00 - O9a.53	Pregnancy, childbirth and the puerperium
L58.0 - L58.9 [W88.0xx+ - W88.8xx+ also required]	Radiodermatitis [history of ionizing radiation exposure]
L59.0 - L59.9 [T66.xxxS also required]	Other disorders of skin and subcutaneous tissue related to radiation [late effect of ionizing radiation exposure]
T37.8x1+ - T37.8x4+	Poisoning by arsenical anti-infectives [history of arsenic exposure]
T57.0x1+ - T57.0x4+	Toxic effect of arsenic and its compounds [history of arsenic exposure]
T66.xxx+	Radiation sickness, unspecified [history of ionizing radiation exposure]
Z34.00 - Z34.93	Encounter for supervision of normal pregnancy
Z85.820	Personal history of malignant melanoma of skin
Z85.828	Personal history of other malignant neoplasm of skin
*UVA/UVB and UVB with the addition of topical coal tar (also known as the Goeckerman regimen)*:
CPT codes covered if selection criteria are met:
96900	Actinotherapy (ultraviolet light)
96910	Photochemotherapy; tar and ultraviolet B (Goeckerman treatment) or petrolatum and ultraviolet B
96913	Photochemotherapy (Goeckerman and/or PUVA) for severe photoresponsive dermatoses requiring at least four to eight hrs of care under direct supervision of the physician (includes applications of medication and dressings)
ICD-10 codes covered if selection criteria are met:
B20	Human immunodeficiency virus [HIV] disease
C84.00 - C84.09 C84.A0 - C84.A9	Mycosis fungoides and cutaneous T-cell lymphoma
L20.0 - L20.82 L20.84 - L20.9	Atopic dermatitis [severe refractory]
L24.9	Irritant contact dermatitis, unspecified cause
L25.9	Unspecified contact dermatitis, unspecified cause
L30.0	Nummular dermatitis
L30.1	Dyshidrosis [pompholyx]
L30.2	Cutaneous autosensitization
L30.8 - L30.9	Other specified and unspecified dermatitis
L40.0 - L40.9	Psoriasis [severe disabling, involving 10% or more of body or severe psoriasis involving the hands, feet or scalp]
L41.0 - L41.9	Parapsoriasis [severe]
L42	Pityriasis rosea
L43.0 - L43.9, L66.1	Lichen planus
L56.4	Polymorphous light eruption
L63.0 - L63.9	Alopecia areata
L70.0 - L70.9	Acne
L80	Vitiligo
L92.0	Granuloma annulare
L94.0	Localized scleroderma [morphea] [only UVA is covered for morphea - not UVB]
M34.81 - M34.89	Other forms of systemic sclerosis [scleroderma]
Q82.1 - Q82.2, Q82.8	Other congenital pigmentary malformations of skin
T86.09	Other complications of bone marrow transplant [skin conditions]
ICD-10 codes not covered for indications listed in the CPB (not all-inclusive):
H16.001 - H16.9	Keratitis [infectious]
L12.0, L12.8 - L12.9	Pemphigoid
L12.1	Cicatricial pemphigoid [benign mucous membrane pemphigoid]
L12.2	Chronic bullous disease of childhood [Juvenile dermatitis herpetiformis]
L29.0 - L29.9	Puritis
L73.2	Hidradenitis suppurativa
M34.0 - M34.2	Systemic sclerosis [scleroderma]
P83.0	Sclerema neonatorum
*Home phototherapy (UVB treatment)(not covered for home PUVA)*:
HCPCS codes covered if selection criteria are met:
A4633	Replacement bulb/lamp for ultraviolet light therapy system, each
E0691	Ultraviolet light therapy system panel, includes bulbs/lamps, timer and eye protection; treatment area 2 sq feet or less
E0692	Ultraviolet light therapy system panel, includes bulbs/lamps, timer and eye protection; 4 ft panel
E0693	Ultraviolet light therapy system panel, includes bulbs/lamps, timer and eye protection; 6 ft panel
E0694	Ultraviolet multidirectional light therapy system in 6 ft cabinet, includes bulbs/lamps, timer and eye protection
ICD-10 codes covered if selection criteria are met:
L20.0-L20.82 L20.84-L20.9	Atopic dermatitis [atopic eczema]
L40.0 - L40.9	Psoriasis [severe/ with frequent flares/ needing to initiate therapy immediately/ unable to attend on-site therapy]
ICD-10 codes not covered for indications listed in the CPB:
C84.00 – C84.09	Mycosis fungoides
C84.10 – C84.19	Sezary disease
C84.A0 – C84.A9	Cutaneous T-cell lymphoma
*Narrow-band UVB*:
CPT codes covered if selection criteria are met:
Narrow-band UVB - no specific code
96900	Actinotherapy (ultraviolet light)
96910	Photochemotherapy; tar and ultraviolet B (Goeckerman treatment) or petrolatum and ultraviolet B
ICD-10 codes covered if selection criteria are met:
C84.00 - C84.09 C84.A0 - C84.A9	Mycosis fungoides and cutaneous T-cell lymphoma [early state]
D47.01	Cutaneous mastocytosis
L20.0 - L20.82 L20.84 - L20.9	Atopic dermatitis [atopic eczema]
L28.1	Prurigo nodularis
L29.8	Other pruritus [uremic pruritus]
L40.8	Other psoriasis
L50.6 - L50.8	Contact and other urticaria [papular] [chronic urticaria if first-line therapies (e.g. systemic corticosteroids and methotrexate) have failed]
L56.3	Solar urticaria
L56.4	Polymorphous light eruption
L56.8 - L56.9	Other specified and unspecified acute skin changes due to ultraviolet radiation
L57.8	Other skin changes due to chronic exposure to nonionizing radiation [actinic dermatitis]
L80	Vitiligo
L87.0	Keratosis follicularis et parafollicularis in cutem penetrans [Kyrle disease]
L94.0	Localized scleroderma [morphea]
L94.1	Linear scleroderma
L94.3	Sclerodactyly
ICD-10 codes not covered for indications listed in the CPB (not all-inclusive):
C86.6	Primary cutaneous CD30-positive T-cell proliferations
D89.810 - D89.813	Graft-versus-host disease
E85.4	Organ-limited amyloidosis [lichen amyloidosis]
L11.1	Transient acantholytic dermatosis [Grover]
L23.0 - L24.7	Allergic and Irritant contact dermatitis [superficial mixed-cell dermatitis]
L28.0	Lichen simplex chronicus [lichenoid dermatitis]
L29.0 - L29.9	Pruritis
L43.2	Lichenoid drug reaction [lichenoid dermatitis]
L50.3	Dermatographic urticaria
L53.1	Erythema annulare centrifugum
L65.2	Alopecia mucinosa
L73.2	Hidradenitis suppurative
L81.8	Other specified disorders of pigmentation [melasma][ progressive macular hypomelanosis]
L81.9	Disorder of pigmentation, unspecified [skin hypo-pigmentation from scarring]
L90.0	Lichen sclerosus et atrophicus
L92.0	Granuloma annulare
L94.0	Localized scleroderma [morphea]
L98.5	Mucinosis of the skin
M34.0 - M34.9	Systemic sclerosis [scleroderma]
O26.611 - O26.619	Liver and biliary tract disorders in pregnancy [cholestasis of pregnancy]
Q81.0 - Q81.9	Epidermolysis bullosa
Q82.8	Other specified congenital malformations of skin
T88.7XXA - T88.7XXS	Unspecified adverse effect of drug or medicament [drug-related hypersensitivity reaction]

The above policy is based on the following references:

Ada S, Seçkin D, Budakoğlu I, Ozdemir FN. Treatment of uremic pruritus with narrowband ultraviolet B phototherapy: An open pilot study. J Am Acad Dermatol. 2005;53(1):149-151.
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American Academy of Dermatology Committee on Guidelines of Care. Guidelines of care for phototherapy and photochemotherapy. J Am Acad Dermatol. 1994;31(4):643-648.
American Academy of Dermatology (AAD). Psoriasis: Recommendations for UVB combination therapies. Practice Management Center. 2018. Available at: https://www.aad.org/practicecenter/quality/clinical-guidelines/psoriasis/phototherapy-and-photochemotherapy/uvb-combination-therapies. Accessed July 19, 2018.
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Clark C, Dawe RS, Evans AT, et al. Narrowband TL-01 phototherapy for patch-stage mycosis fungoides. Arch Dermatol. 2000;136:748-752.
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Last Review 04/15/2022

Effective: 03/12/1998

Next Review: 02/09/2023
Review History
Definitions

Phototherapy and Photochemotherapy (PUVA) for Skin Conditions

Policy

PUVA

UVA/UVB

Background

UVB for the Treatment of Photodermatoses

PUVA for Eosinophilic Cellulitis (Wells Syndrome)

PUVA for Necrobiotic Xanthogranuloma

Phototherapy for Scleredema

Narrow-Band UVB for the Treatment of Chronic Urticaria

Narrow-Band UVB for the Treatment of Alopecia Mucinosa

Narrow-Band UVB Phototherapy for the Treatment of Cutaneous Mastocytosis

Phototherapy for the Treatment of Grover's Disease (Transient and Persistent Acantholytic Dermatosis)

Phototherapy for the Treatment of Lymphomatoid Papulosis

Phototherapy (UVA and Narrow-Band UVB) for the Treatment of Uremic Pruritus

PUVA for the Treatment for Necrobiosis Lipoidica

UVB for the Treatment of Kyrle Disease (Perforating Dermatosis)

UVB for the Treatment of Papular Mucinosis

UVB for the Treatment of Prurigo Nodularis

UVB for the Treatment of Lichen Amyloidosis

UVB for the Treatment of Refractory Uremic Pruritus

NB-UVB Phototherapy for the Treatment of Cutaneous Mastocytosis

NB-UVB Phototherapy for the Treatment of Drug-Related Hypersensitivity Reaction

NB-UVB Phototherapy for the Treatment of Erythema Annulare Centrifugum

NB-UVB Phototherapy for the Treatment of Lichen Sclerosus

NB-UVB Phototherapy for the Treatment of Lichenoid Dermatitis

NB-UVB Phototherapy for the Treatment of Lymphomatoid Papulosis

Contraindications to Ultraviolet Phototherapy

Home Phototherapy (UVB) for the Treatment of Atopic Dermatitis (Eczema)

Home Phototherapy for the Treatment of Cutaneous T-Cell Lymphoma (Mycosis Fungoides and Sezary Syndrome)

Information in the [brackets] below has been added for clarification purposes. Codes requiring a 7th character are represented by "+":

PUVA:

CPT codes covered if selection criteria are met:

ICD-10 codes covered if selection criteria are met:

ICD-10 codes not covered for indications listed in CPB (not all-inclusive):

ICD-10 codes contraindicated for this CPB:

UVA/UVB and UVB with the addition of topical coal tar (also known as the Goeckerman regimen):

CPT codes covered if selection criteria are met:

ICD-10 codes covered if selection criteria are met:

ICD-10 codes not covered for indications listed in the CPB (not all-inclusive):

Home phototherapy (UVB treatment)(not covered for home PUVA):

HCPCS codes covered if selection criteria are met:

ICD-10 codes covered if selection criteria are met:

ICD-10 codes not covered for indications listed in the CPB:

Narrow-band UVB:

CPT codes covered if selection criteria are met:

ICD-10 codes covered if selection criteria are met:

ICD-10 codes not covered for indications listed in the CPB (not all-inclusive):

The above policy is based on the following references:

Policy History

Additional Information