Polymorphous Light Eruption

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Background

Polymorphous light eruption (PMLE) is an acquired disease and is the most common of the idiopathic photodermatoses. PMLE is characterized by recurrent, abnormal, delayed reactions to sunlight, ranging from erythematous papules, papulovesicles, and plaques to erythema multiforme –like lesions on sunlight-exposed surfaces. Within any 1 patient, only 1 clinical form is consistently manifested.

The possibility that a subset of PMLE called benign summer light eruption or BSLE which might be milder and might be more UVA driven has been suggested by an Italian group.[1]

Richards et al found that PMLE engenders a substantial psychosocial impact on patients who have the condition.[2] Based on results from the Illness Perception Questionnaire sent to 302 patients and returned by 150 patients, 40% experienced emotional distress linked to PMLE. The psychological impact was related to the predicted consequences of PMLE, whereas health-related variables played a lesser role. Women associated more severe consequences linked to PMLE and were more emotionally distressed than men.

Note the images below.



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Polymorphous light eruption on the thighs and hand. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/reactions/pmle2.jpg).



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Polymorphous light eruption on the arm. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_re....

Pathophysiology

The etiology of polymorphous light eruption (PMLE) is not fully known, and it is likely to be multifactorial. The immunologic pathogenesis of PMLE is supported by the study of timed biopsy samples of PMLE lesions. The CD4 subtype of T cells seen very early after exposure is replaced by CD8 lymphocytes 72 hours after irradiation. In general, findings conform to type IV delayed-type hypersensitivity mechanism. The action spectrum is variable and can include UVA and UVB light. Some patients react in the visible light spectrum.[3, 4, 5, 6]

Some have suggested that glutathione S-transferases (GSTs) act to protect against PMLE, but a study of the isoenzymes of the GST genes GSTM1, GSTT1, and GSTP1 found no protective relationship of these isoenzymes to PMLE.[7]

It is possible that the use of tobacco makes PMLE worse.[8]

In some PMLE lesions induced by UV-A, keratinocytes were found to express intercellular adhesion molecule 1 (ICAM-1).[9, 10] ICAM-1 is absent from normal keratinocytes, but it is known to be strongly induced by interferon gamma. The induction of ICAM-1 on keratinocytes results either from direct effects of UV on the promoter region of the ICAM-1 gene or from indirect effects of interferon gamma produced by activated lymphocytes aggregating in an underlying PMLE.

Intravascular and focal perivascular deposits of fibrin were detected in biopsy samples of PMLE papules. Vascular deposits of C3 and immunoglobulin M (IgM) were noted in a few patients. These findings may suggest that vascular injury with activation of a clotting cascade may play a role in the pathogenesis of PMLE. Repair of ultraviolet-damaged DNA is normal.

The demonstration that the female hormone 17beta-estradiol prevents UV radiation–induced suppression of the contact hypersensitivity response caused by the release of immunosuppressive cytokines (interleukin [IL]–10) from keratinocytes might thus explain why the risk of PMLE is higher in females than in males and why the risk decreases in women after menopause.[11]

Neutrophils may play a role in the development of PMLE. Immunohistochemical analysis by Schornagel et al in 2004 showed a significant decreased neutrophil infiltration in PMLE skin after UV-B irradiation compared with healthy case control subjects (P< .05).[12] ICAM-1 and E-selectin expression on endothelial cells increased in both healthy controls and in the PMLE patients after UV-B irradiation. Chemotactic response towards IL-8 and C5a was not different between PMLE patients and healthy controls. The authors concluded that PMLE is marked by an altered immune response resulting in decreased skin infiltration of neutrophils after UV-B irradiation.

Kölgen et al noted that the reduced expression of tumor necrosis factor-alpha, IL-4, and IL-10 in the UV-B–irradiated skin of patients with PMLE.[13] The reduction of these cytokines seems linked to a relative neutropenia and is a manifestation of decreased Langerhans cell migration and reduced TH2 skewing. An impairment of these mechanisms underlying UV-B–induced immunosuppression may be important in the pathogenesis of PMLE.[14]

A study by Koulu et al assessed a total of 48 subjects (24 patients with PMLE and 24 healthy sex-matched and age-matched controls).[15] The study found similar immunosuppression of contact sensitization to diphenylcyclopropenone due to earlier exposure to solar-simulating UV radiation between both groups. However, among patients with PMLE who were immunosuppressed by UV radiation, only one exhibited immunotolerance to the same allergen 10–24 months later (P=0.023). The study concluded that impaired propensity to UV-induced, allergen-specific immunotolerance may promote recurrent PMLE.

It has also been shown that UVC can cause PMLE.[16]

Etiology

Although most authorities now consider UV-A light as the causative factor in polymorphous light eruption (PMLE) eruption, UV-B, or even visible light, may be responsible in some individuals. PMLE-like lesions have been reported in welders, resulting from exposure to UV-C light.[17]

Epidemiology

Frequency

United States

Polymorphous light eruption (PMLE) affects about 10% of the US population. This figure is likely to be an underestimate because many patients do not seek medical attention. Many of the photodermatoses were lumped together before their individual pathogeneses were identified. PMLE is now a distinct clinical entity, as are many of the other photodermatoses (eg, solar urticaria, photoallergic dermatitis, hydroa vacciniforme, chronic actinic dermatitis, erythropoietic porphyria, lupus erythematosus).

Reported in 2007, Kerr and Lim identified 280 patients with photodermatoses.[18] One hundred thirty-five (48%) were African Americans, 110 (40%) were white, and 35 (12%) were patients of other races. They noted a statistically significantly higher proportion of African Americans with PMLE compared with whites.

Benanni et al noted a low incidence of PMLE in renal transplant recipients,[19] and Hönigsmann reports a prevalence of 10-20% in the United States and Western Europe.[20]

International

Deng et al used a questionnaire to survey 4899 residents (49% men and 51% women) of random Chinese villages in Yuan Jiang county (Dai and Hani minorities), Kunming city (Han people and Yi minority), Lijiang county (Naxi minority), and Shangri-La county (Zang minority).[21] The altitudes of these regions were 380 meters, 1870 meters, 2410 meters, and 3280 meters, respectively. The prevalence of PMLE was 32 (0.65%) in 4899 residents and was 3.8 times higher in women compared with men. At higher elevations, the prevalence of PMLE increased. The mean time of sun exposure for PMLE was 6 h/d. The mean duration of PMLE was 5.8 years.

PMLE affects 21% of the population in Sweden.

A report from India in 2013[22] notes that PMLE is the most common photodermatitis after chronic actinic dermatitis, while hydroa vacciniforme and solar urticaria are uncommon. Furthermore, this report stated lichenoid PMLE and pin-point papular PMLE are the most common types found in the subcontinent.

Race

Polymorphous light eruption (PMLE) affects all racial skin types. Overall, family history is positive for PMLE in about 15% of the patients. However, Native Americans have a hereditary form of PMLE with apparent autosomal dominant inheritance; 75% reveal disease in a family member.[23] The popular variant can involve the face and seems most common in patients with Fitzpatrick type skin III-VI.[24, 25]

One study of a cohort of 229 patients with photodermatoses reported that 63 (42.2%) were white and 138 (46.6%) were African American and PMLE was present in 54% and 86.2%, respectively, (P  < 0.0001); thus, the researchers suggested that PMLE occurs more frequently in African Americans.[26]

Sex

Polymorphous light eruption (PMLE) affects females 2-3 times more often than males. However, these data may be skewed because women are more likely to seek medical attention for cosmetic problems than males.

Age

Polymorphous light eruption (PMLE) usually has an onset in the first 3 decades of life. Men seem to have later onset of the disease than women.

Naleway et al reviewed records of 124 patients diagnosed with PMLE and found most were women and that the mean age of PMLE onset was 37.8 years.[27] They noted only 4 required phototherapy treatment.

Prognosis

Expression of polymorphous light eruption (PMLE) may range from an insignificant, mild rash to severe disease affecting the patient's quality of life. Some PMLE patients experience a less severe reaction with each consecutive year, but many patients have reactions that may worsen with time without appropriate treatment. Each case should be evaluated individually.

Richards et al found that emotional distress attributable to PMLE occurred in greater than 40% of individuals.[2] Women more than men associated more severe consequences with their PMLE and experienced more emotional distress.

History

Polymorphous light eruption (PMLE) tends to manifest in the spring.[28] In addition, PMLE is a recurrent condition and patients state they have had the eruption before and that it went away as time passed.

Sunlight is clearly the primary etiologic factor for PMLE. The eruption of PMLE typically occurs in spring or, rarely, in winter following ultraviolet radiation exposure reflected from snow. Typically, the lesions of PMLE first erupt at the onset of a vacation in a sunny place or at a high altitude and disappear by the time the patient returns home. The eruption decreases in severity as the summer progresses.

The onset of the disease is sudden. The accompanying rash is pruritic and, in some instances, painful. Thirty minutes to several hours of exposure are required to trigger the eruption. Sun-exposed skin, especially that normally covered in winter (eg, upper chest, arms), is primarily affected, but autosensitization may lead to a generalized involvement. The rash appears within hours to days of exposure, and it subsides over the next 1-7 days without scarring. Most patients have associated pruritus, but some patients describe stinging and pain.

Occasionally, patients experience systemic flulike symptoms after sun exposure.

Unless severe and particularly bothersome, many patients do not visit a physician for PMLE rash. It is often discovered incidentally.

Jansen traced the natural history of chronic PMLE in 138 people, 85 of whom were female.[29] Their mean age was 26.4 years. The length of time in the study was 10.5 years. In 57% of cases, the PMLE happened in a rapid fashion. It started in a small photoexposed area in 88% of cases, and extended to a greater area each year. Light sensitivity tended to increase with each subsequent year. The patients’ threshold tolerance to solar radiation occurred 30 minutes after exposure in ≤60% of patients. In 50% of patients, yearly hardening phenomena occurred. Ocular and oral involvement occurred in 46% and 49% of the patients, respectively. About 66% patients experienced some general symptoms after solar radiation exposure.

Majoie et al in 2010 noted PMLE-like skin lesions in welders caused by ultraviolet C light.[17]

A 2013 report noted a 37-year-old women who received UVA-1 phototherapy and developed PMLE that persisted for 5 weeks.[30]

Physical Examination

As the name implies, clinical manifestations of polymorphous light eruption (PMLE) vary. Many different morphologies may appear on sun-exposed areas, but, usually only one morphology dominates in a given individual.

Papules (greatest incidence), plaques, papulovesicles, and erythema multiforme–like lesions are the most common morphologies. Photosensitive erythema multiforme and erythema multiforme–like PMLE can be difficult to distinguish clinically. Combined morphological types of lesions, while uncommon, do occur. For example, the small papular variety may coalesce to form an eczematous type and large papular lesions may produce plaques or assume an annular configuration. Note the images below.



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Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/Watermar....



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Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/Watermar....



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Polymorphous light eruption on the arm. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_re....



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Polymorphous light eruption on the thighs and hand. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/reactions/pmle2.jpg).

Sun-exposed skin, especially that normally covered in winter (eg, upper chest, arms), is affected primarily, but autosensitization may lead to a generalized involvement.

Cheilitis is uncommon in patients in the United States. In such patients, the rare diagnosis of actinic prurigo is a more likely cause of the inflammatory photosensitivity disorder. Cheilitis often occurs in the tropics and, when this is the case, can be the only manifestation of the PMLE. That is, it can manifest without involvement of the extremities, face, or torso. In the case of photosensitive cheilitis, PMLE must be distinguished from chronic actinic cheilitis and the eczematous cheilitis produced by photosensitizing agents.

In African Americans, a variant of PMLE with pinpoint papules (1-2 mm) can be observed on sun-exposed areas, sparing the face and flexural surfaces.[31]

Pinpoint papular PMLE has been described in a series from Singapore.[32] This variant seems to manifest in patients in Fitzpatrick type IV-VI skin,[25] and was also noted in a series of 34 Taiwanese patients whose rash resolved with sun protection. Ten of the patients had mild spongiosis on biopsy.[24]

A 2010 study by Gronhagen et al reported on the importance of differentiating systemic lupus and cutaneous lupus erythematosus from PMLE. In this study, 23% of 260 systemic lupus erythematosus patients had cutaneous lupus erythematosus, and a history of PMLE was found in 42%.[33]

In a retrospective study from Spain in 2013,[34] 5 men and 4 women were suggested to have a localized variant of PMLE, termed ”spring and summer eruption of the elbows" by the authors. The mechanism that confined the lesions to these specific areas was not determined.

Scar PMLE that can occur on hypopigmented scars has been noted in India.[35]

Laboratory Studies

In polymorphous light eruption (PMLE), laboratory tests are generally performed to rule out other dermatoses, such as erythropoietic protoporphyria or lupus erythematosus. Antinuclear antibody (ANA), anti-Ro (SS-A), and anti-La (SS-B) tests, as well as urine, stool, and blood porphyrin levels, should be obtained.[37]

The diagnosis of PMLE is usually based on the clinical picture. Normal titers of ANA, as well as normal urine, stool, and blood porphyrin levels, support the diagnosis.

Other Tests

Results of phototesting in polymorphous light eruption (PMLE) patients are controversial, ranging from an ability to reproduce the eruption by repeat phototesting in 60-100% of patients to an inability to do so except in patients who are very photosensitive.[38] These differences may be explained by a lack of a standardized test procedures, variation in radiation sources used, and imprecision in diagnostic criteria for the disease.

Perform phototests with UV-A, UV-B, and visible light sources. Determine the MED from these sources. MEDs are normal in PMLE and lowered or abnormal in chronic actinic dermatitis. In solar urticaria, irradiation results in reproduction of the lesion. Perform repetitive light testing; irradiating 3 times the MED to UV-A on the right forearm and 3 times the MED to UV-B on the left forearm for 3 consecutive days. Results are read immediately, at 24 and 72 hours. A delayed reading at 1 week may also be helpful. The test results are often positive in PMLE. A negative result does not exclude the diagnosis. If a lesion (eg, papule, vesicle) develops, biopsy confirmation is suggested. Histologically, a superficial and deep perivascular lymphocytic infiltrate will be apparent with dermal edema.

Photopatch tests to rule out a photoallergic or airborne contact dermatitis should be performed. Two identical strips of standard photoallergens are placed on the back. One of the two strips is exposed to UV-A radiation 24 hours later. Both the irradiated site and the unirradiated site are read at 24, 48, and 96 hours. A positive reaction at the irradiated site but not at the unirradiated patch test site is diagnostic of a photocontact allergy. Positive reactions at both the irradiated site and the unirradiated site are indicative of a contact allergy. According to Leroy et al in 2002, polychromatic phototesting seems to be more sensitive than UV-A phototesting to assess PMLE, and results suggest UV-B is a key trigger of PMLE.[39]

Histologic Findings

The most striking feature of the biopsy specimen from a patient with polymorphous light eruption (PMLE) is edema in the upper part of the dermis. Tight, perivascular lymphocytic infiltrate is observed in the upper and mid dermis. When eczematous epidermal changes are present clinically, spongiosis, edema, dyskeratosis, and basal cell vacuolization may be observed. Occasionally, neutrophils and eosinophils may be present in the infiltrate. The dominant cell, however, is the lymphocyte. Mucin, which is thought to distinguish PMLE from lupus, can be present in a specimen from a PMLE patient. A study of dermal mucin from lupus samples found that it is not so much different from that found in PMLE in comparison with other dermatitides, which also had some, but less, mucin (eg, erythema multiforme, fixed drug eruption, graft versus host disease, lichen planus, photodamaged skin).[40]

Note the images below.



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Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathol....



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Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathol....



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Polymorphous light eruption pathology showing papillary dermal edema with lymphocytes in the epidermis (exocytosis). Courtesy of DermNet New Zealand (....



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Polymorphous light eruption pathology. The infiltrate is mainly lymphocytic but there may be intermixed eosinophils, neutrophils, and histiocytes. Cou....

Medical Care

UVA1 therapy can harden the skin against the development of polymorphous light eruption (PMLE). A case of PMLE that failed UVA1 therapy has been reported; a woman aged 37 years developed an recalcitrant PML that lasted 5 weeks after completion of UVA1 phototherapy.[41]

Prophylactic therapy (eg, avoiding sunlight, wearing protective clothing, using sunscreen) remains a key factor in the care of patients with PMLE. Sunscreens with high sun protection factor (SPF) values are not protective against UV-A–induced PMLE. Systemic vitamin C and vitamin E do not prevent photoprovocation test reactions in persons with PMLE.

In a randomized, double-blinded, placebo-controlled clinical study by Hadshiew et al, the efficacy of a new topical formulation was compared with a broad-spectrum sunscreen.[42] The new product contained 0.25% alpha-glucosylrutin (a natural, modified flavonoid) and 1% tocopheryl acetate (vitamin E). Thirty patients with a history of PMLE were pretreated with the formulations 30 minutes prior to daily photoprovocation with UV-A irradiation of 60-100 J/cm2 to the upper arms.

The authors found a statistically significant difference (P< .001) between the antioxidant-containing formulations and placebo and between the sunscreen-only formulation. Only a single patient treated with the new antioxidant UV-protective gel formulation developed clinical signs of PMLE in the area treated. In comparison, 62.1% of the placebo-treated areas and 41.3% of the sunscreen-only treated areas showed mild-to-moderate signs of PMLE. The authors suggested that combining a potent antioxidant with a broad-spectrum sunscreen is far more effective in preventing PMLE than sunscreen alone. Also see Sunscreens and Photoprotection.

Gruber-Wackernagle et al evaluated the preventive effect of a cream containing calcipotriol (an analog of calcitriol, 1,25-dihydroxyvitamin D3) in a randomized double-blinded placebo-controlled intraindividual half-body trial.[43] Thirteen patients with PMLE applied cream (calcipotriol or placebo) topically to symmetrically located pairs of test areas twice daily for 7 days before photoprovocation with solar-simulated UV radiation was begun.

The authors used a specific PMLE test score based on affected area, skin infiltration, and pruritus to rate symptom severity at 48, 72, and 144 h after the first photoprovocation exposure. They found pretreatment with calcipotriol, compared with placebo, significantly reduced PMLE symptoms on average by 32% (P=0.0022) over the time series, suggesting a possible benefit from prophylactic use of topical 1,25-dihydroxyvitamin D3 analogs in patients with PMLE.[43] However, this does not change the standard of care for PMLE.

Jeanmougin et al studied the effectiveness 0.25% alpha-glucosyl-rutin, 1% vitamin E, and a broad-spectrum highly UVA–protective sunscreen (SPF 15; persistent pigmentation darkening 6) under real solar exposure conditions in the spring and summer.[44] The cream was applied every 2 hours after the first summer exposure. No topical or systemic treatments to prevent PMLE were used; dermatologists checked patients after the summer was over and interviewed them.

In this study, 52 of 54 patients finished study, and 67% of patients had no eruptions, 19% had minor eruptions, and 13% had severe eruptions of PMLE.[44] Pruritus, which had been present in all patients the year preceding the study, was not observed in 69% of patients and was unbearable for only 3 patients (compared with 27 patients before the study preparation was used).The dermatologic assessment was that global efficacy was approximately 80%, with inadequate results in 10% of cases; specifically, it was deemed excellent for 35 patients and good for 7 patients.

DeLeo et al reported that sunscreen with 4 UVA filters (ie, ecamsule 3%, octocrylene 10%, avobenzone 2%, and titanium dioxide 5%) was more effective for preventing PMLE flares than a sunscreen with a triad of UVA blockers.[45] Other studies support the use of UVA blockers to help prevent PMLE,[46] including a report that describes a lower percentage of PMLE (0% at 2 mg/m2; 33% at 1 mg/m2) in subjects who used high UVA sunscreen protection versus those who used lower UVA sunscreen protection (73% at 2 mg/m2; 80% at 1 mg/m2).[47]

Prophylactic phototherapy or photochemotherapy at the beginning of spring for several weeks may prevent flare-ups throughout the summer. PUVA was found to be superior to UV-B in several studies, controlling the outbreaks in 90% of patients.[48] Oral prednisone may be useful in conjunction with phototherapy to avoid eruption during therapy. Narrow-band UV-B (311 nm) may be an acceptable alternative to PUVA. In a study of 25 patients with severe PMLE, both modalities were equally effective.[49] Barolet and Boucher report on the use of light-emitting diode (LED) nonthermal therapy as a prophylactic measure for PMLE.[50]

When preventive measures fail and light therapy is ineffective or contraindicated, pharmacologic treatment assumes its role. Topical corticosteroids are useful, as would be expected in many dermatoses associated with lymphocytic skin infiltrate. Tachyphylaxis and skin atrophy limit their use. Antihistamines may help with pruritus. Systemic steroids may be needed to suppress acute flares or extensive generalized eruption. Adverse effects of prolonged systemic steroid use include decreased glucose tolerance, osteoporosis, impaired immunity, and weight gain. Obviously, this treatment can only be offered intermittently and for a short period of time. It may also be considered for patients going on vacation or for those patients experiencing other unavoidable sun exposure.

Antimalarials at low doses are sometimes helpful, especially in patients with a large papular variety of PMLE. Beta-carotene, which is effective in erythropoietic protoporphyria, may be an alternative to chloroquine.[51] Oral carotenoid preparation (beta-carotene and canthaxanthin in a daily total dose of 100 mg) was compared to hydroxychloroquine (200 mg qd). Both offered full sun tolerance in an equal but small, percentage of patients, when compared to a placebo.

Some authorities believe that vitamin therapy is helpful in the treatment of PMLE. Nicotinamide was successful in 60% of 42 patients treated with 3 g/d orally for 2 weeks.[52] The rationale for its use was the knowledge that it blocks the formation of kynurenic acid, a photosensitizer that may play a role in PMLE. Ahmed et al found that oral vitamin E supplementation (400 IU) and use of sunblock decreased the markers of oxidative stress and lipid peroxidation in patients with PMLE.[53]

Azathioprine was reported to be effective in 2 cases of recalcitrant severe disease at 0.8-2.5 mg/kg/d for 3 months.[54] In one patient, the effect lasted up to 4 months after the discontinuation of therapy. However, the limited available data and azathioprine toxicity should necessitate extreme caution in choosing this form of treatment.

Interest in the use of thalidomide for a number of dermatoses (eg, Behçet syndrome, cutaneous lupus, porphyria cutanea tarda [PCT], PMLE) is reemerging. The immunomodulatory action on subsets of T cells was proposed. Thalidomide (50-200 mg PO qhs) has reportedly been very effective for Native American patients with PMLE. The most commonly described adverse effects with thalidomide are sedation, constipation, and weight gain.

The most serious complications of thalidomide are peripheral neuropathy and teratogenicity. Unfortunately, no recent published controlled trials of this drug being used in the treatment of PMLE are available. Thalidomide is available only to pharmacies and physicians participating in the System for Thalidomide Education and Prescribing Safety (STEPS) program. A new variant of thalidomide is available.

Lenalidomide (Revlimid, formerly known as Revimid) is the first of a new class of oral cancer drugs called IMiDs. This immunomodulatory drug is chemically similar to thalidomide. However, it is more powerful in the laboratory. It appears to lack some of the more common adverse effects of thalidomide. Its role in the treatment of PMLE is uncertain.

Prevention

Avoiding sunlight during the hours of most intense UV irradiation (from 10 am to 2 pm) and wearing protective clothing (eg, hats, gloves, long sleeves) should be emphasized to polymorphous light eruption (PMLE) patients. Blue denim clothing is particularly beneficial in terms of sun protection. Wide range sunblocks with a high SPF should be applied and reapplied during the day. Polypodium leucotomos extract may be of some benefit.[55]  

Medication Summary

The goals of pharmacotherapy for polymorphous light eruption (PMLE) are to reduce morbidity and to prevent complications. The inclusion of both ecamsule and avobenzone in one preparation of sunscreen provides clinical benefit to patients with PMLE compared with sunscreens containing either ecamsule or avobenzone alone, each UVA filter individually.[45]

Hydroxychloroquine (Plaquenil)

Clinical Context:  Hydroxychloroquine inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions.

Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

Class Summary

These agents may have immunomodulatory effects.

Prednisone (Deltasone, Meticorten, Orasone)

Clinical Context:  Prednisone is an immunosuppressant for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Thalidomide (Thalomid)

Clinical Context:  Thalidomide is only supplied to pharmacies participating in the STEPS program. It is an immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell surface adhesion molecules involved in leukocyte migration.

Class Summary

These agents modify the activity of key factors in the immune system.

Vitamin A

Clinical Context:  Vitamin A may provide a limited level of photoprotection. It causes yellowing of skin (carotenoderma). Any photoprotection afforded will increase slowly after the drug is commenced over 4- to 6-week period. When discontinued, skin color and benefit fade over several weeks.

Niacin (Vitamin B-3)

Clinical Context:  The source of niacin is used in tissue respiration, lipid metabolism, and glycogenolysis.

Class Summary

These agents are essential for normal DNA synthesis and cell function.

What is polymorphous light eruption (PMLE)?What is the pathophysiology of polymorphous light eruption (PMLE)?What causes polymorphous light eruption (PMLE)?What is the prevalence of polymorphous light eruption (PMLE) in the US?What is the global prevalence of polymorphous light eruption (PMLE)?What are the racial predilections of polymorphous light eruption (PMLE)?What are the sexual predilections of polymorphous light eruption (PMLE)?Which age groups have the highest prevalence of polymorphous light eruption (PMLE)?What is the prognosis of polymorphous light eruption (PMLE)?Which clinical history findings are characteristic of polymorphous light eruption (PMLE)?Which physical findings are characteristic of polymorphous light eruption (PMLE)?Which physical findings are characteristic of polymorphous light eruption (PMLE) in patients outside the US?Which conditions should be included in the differential diagnoses of polymorphous light eruption (PMLE)?What are the differential diagnoses for Polymorphous Light Eruption?What is the role of lab testing in the diagnosis of polymorphous light eruption (PMLE)?What is the role of phototesting in the diagnosis of polymorphous light eruption (PMLE)?Which histologic findings are characteristic of polymorphous light eruption (PMLE)?How is polymorphous light eruption (PMLE) treated?What is the efficacy of topical agents in the treatment of polymorphous light eruption (PMLE)?What is the role of phototherapy in the prevention of polymorphous light eruption (PMLE)?When is drug treatment used for polymorphous light eruption (PMLE)?What is the role of vitamin therapy in the treatment of polymorphous light eruption (PMLE)?What is the role of azathioprine in the treatment of polymorphous light eruption (PMLE)?What is the role of thalidomide in the treatment of polymorphous light eruption (PMLE)?What is the role of lenalidomide in the treatment of polymorphous light eruption (PMLE)?How is polymorphous light eruption (PMLE) prevented?Which agents increase the effectiveness of sunscreen for the treatment of polymorphous light eruption (PMLE)?Which medications in the drug class Vitamins are used in the treatment of Polymorphous Light Eruption?Which medications in the drug class Immunomodulators are used in the treatment of Polymorphous Light Eruption?Which medications in the drug class Corticosteroids are used in the treatment of Polymorphous Light Eruption?Which medications in the drug class Antimalarials are used in the treatment of Polymorphous Light Eruption?

Author

Noah S Scheinfeld, JD, MD, FAAD, † Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Disclosure: Nothing to disclose.

Coauthor(s)

Raul Del Rosario, MD, Consulting Staff, Dermatopathology, Mission Hospital at Laguna Beach

Disclosure: Nothing to disclose.

Sophie Shirin, MD, Consulting Staff, Global Dermatology

Disclosure: Nothing to disclose.

Specialty Editors

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD, Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Disclosure: Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD, Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Disclosure: Nothing to disclose.

Additional Contributors

Craig A Elmets, MD, Professor and Chair, Department of Dermatology, Director, Chemoprevention Program Director, Comprehensive Cancer Center, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: University of Alabama at Birmingham; University of Alabama Health Services Foundation<br/>Serve(d) as a speaker or a member of a speakers bureau for: Ferndale Laboratories<br/>Received research grant from: NIH, Veterans Administration, California Grape Assn<br/>Received consulting fee from Astellas for review panel membership; Received salary from Massachusetts Medical Society for employment; Received salary from UpToDate for employment. for: Astellas.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Dr. Ada Winkielman, to the development and writing of this article.

References

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Polymorphous light eruption on the thighs and hand. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/reactions/pmle2.jpg).

Polymorphous light eruption on the arm. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MCJd/pmle-22.JPG).

Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MCJd/pmle-15.JPG).

Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MSJd/pmle-14.jpg).

Polymorphous light eruption on the arm. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MCJd/pmle-22.JPG).

Polymorphous light eruption on the thighs and hand. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/reactions/pmle2.jpg).

Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-1.jpg).

Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-2.jpg).

Polymorphous light eruption pathology showing papillary dermal edema with lymphocytes in the epidermis (exocytosis). Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-3.jpg).

Polymorphous light eruption pathology. The infiltrate is mainly lymphocytic but there may be intermixed eosinophils, neutrophils, and histiocytes. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-4.jpg).

Photograph of a springtime eruption of polymorphous light eruption in a 6-year-old boy. The eruption has occurred in the spring since the patient was aged 5 years, and it resolves completely with no scarring by mid to late summer. High block sunscreens attenuate but do not prevent the eruption. No itching or pain occurs. Courtesy of Dr. Jeremy F. Harrison, FRCA, FFAEM.

Polymorphous light eruption on the thighs and hand. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/reactions/pmle2.jpg).

Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MSJd/pmle-14.jpg).

Polymorphous light eruption on the chest. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MCJd/pmle-15.JPG).

Polymorphous light eruption on the arm. Courtesy of Waikato District Health Board and DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/_resampled/FitWzY0MCw0ODBd/WatermarkedWyIyNTg0MCJd/pmle-22.JPG).

Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-1.jpg).

Polymorphous light eruption pathology showing papillary dermal edema. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-2.jpg).

Polymorphous light eruption pathology showing papillary dermal edema with lymphocytes in the epidermis (exocytosis). Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-3.jpg).

Polymorphous light eruption pathology. The infiltrate is mainly lymphocytic but there may be intermixed eosinophils, neutrophils, and histiocytes. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/e/pmle-figure-4.jpg).