View metadata, citation and similar papers at core.ac.uk brought to you by CORE provided by Elsevier - Publisher Connector Polymorphous Light Eruption Erhard Holzle, M.D., Gerd Plewig, M.D., Renate von Kries, M.D., Percy Lehmann, M.D. Department of Dermatology, University of Diisseldorf, Diisseldorf, F. R. G. Polymorphous light eruption (PLE) is a common photo­ cells, and spongiosis in the lower epidermis. The most dermatosis of unknown etiology. It affiicts mainly fair­ important differential diagnoses are solar urticaria, pho­ skinned patients, with a preponderance of young females. tosensitive erythema multiforme, and lupus erythemato­ There is, however, no absolute restriction as to age, sex, sus. or race. The action spectrum of PLE is under debate. Repro­ Clinical variants include the papular, vesiculo-bullous, duction of skin lesions has been reported with UVB, UV A, and hemorrhagic variety, as well as plaque, erythema mul­ and, rarely, visible light, with UVA probably being the tiforme-like, and insect bite (strophulus)-like types. Skin most effective part of the spectrum. lesions appear only in certain exposed areas hours or a few More important than treatment of PLE is prophylaxis. days after intense sunshine, and are nearly always mono­ UV A- and UVB-effective sunscreens are of some help. morphous in the same patient. The rash subsides sponta­ Phototherapy and especially photochemotherapy (psoralen neously within several days without leaving scars. + UVA; PUV A) offer effective ways to decrease light The histopathologic picture is characteristic and shows sensitivity. Systemic treatment with chloroquine or f3-car­ a perivascular lymphocytic inftltrate in the upper and mid­ otene has been disappointing. ] Invest Dermatol 88:325-385, dle corium with subepidermal edema, vacuolization of basal 1987 t was recognized in the early 19th century that certain ecze­ several morphologic variants. Our experience is based on obser­ matous reactions were caused by sunlight. Willan [1] was vations in more than 250 patients in the years from 1977 to 1985 the first to use the term "eczema solare." In 1879 Sir Jonathan [8-11). Hutchinson [2] presented 14 patients under the diagnosis I CLINICAL FEATURES "summer prurigo. " There is reason to believe that "prurigo aestivalis," "prurigo adolescentium," and "acne prurigo" syn­ Polymorphous light eruption is a very common photodermatosis, onymously designated similar sun-induced skin disorders. Rasch, but the true prevalence among the population is not known. In in 1900 [3], simplified the terminology by introducing the term a survey of 271 apparently healthy subjects, 10% gave a history "polymorphous light eruption (PLE). " This designation was widely consistent with PLE [12]. Many persons are aware of a transient accepted to characterize urticarial, papular, vesicular, and ecze­ "sun allergy" but never visit a dermatologist because they know matous reactions precipitated by light [4]; however, it did not how to manage the problem by themselves. Individual suscep­ clarify the nosology of photodermatoses. tibility varies greatly, and only the most severely afflicted patients A variety of sunlight-evoked eruptions, such as solar urticaria, are seen by physicians. In many patients, PLE appears during the hydroa vacciniforme, porphyrias, photoallergic dermatitis, pho­ first days of vacationing on the beach or in high altitudes. When tosensitive erythema multiforme, and lupus erythematosus (LE) the patient returns home the eruption has resolved spontaneously fell under this diagnosis. It was only during the past decades that and the pliysician who is asked for help can only conceive a these differential diagnoses have been separated and polymor­ diagnosis by the patient's history. phous light eruption has been defmed as a clinical entity [5-8). Polymorphous light eruption seems to occur most frequently Until now, however, it remains difficult, if not impossible, to among fair-skinned populations. Large series are reported from distinguish in certain patients between polymorphous light erup­ Scandinavian countries [13,14], but the eruption is not confmed tion and light-induced subacute or discoid cutaneous lupus ery­ to a certain race or skin color [7]. It has been reported in Blacks, thematosus, or photosensitive erythema multiforme. Orientals, and in Native North and Latin Americans [15,16]. This review describes the growing knowledge about PLE. We Among the latter, the familial occurrence is a characteristic fea­ put forward our concept of the disease as a distinct entity with ture. This peculiar photodermatosis might represent an entity different from PLE observed in whites [17]. Inheritance in an autosomal-dominant mode with a reduced penetrance has been Reprint requests to: Prof. Dr. E. Holzle, Universitatshautklinik, Moor­ reported in 56% of patients evaluated in Finland [18] and in 75% enstr. 5, D-4000 Diisseldorf 1, F.R.G. of Canadian Inuit patients [19]. In other series, genetic predis­ Abbreviations: position ranged from 3-45%. Jansen [18] suggested that patients DLE: discoid lupus erythematosus in other reports might not have been questioned as thoroughly LE: lupus erythematosus and, therefore, genetic predisposition in general might be under­ MED: minimal erythemal dose PLE: polymorphous light eruption estimated. PUV A: photochemotherapy (psoralen + UV A) There is agreement that the time of onset is most commonly SCLE: subacute cutaneous lupus erythematosus during the early adult life [7,13], but the eruption may also start UVA: ultraviolet A in childhood. In children the face and ears frequently are involved. UVB: ultraviolet B Similar cases have been described under the designation ''juvenile 0022-202X/87/S03.50 Copyright © 1987 by The Society for Investigative Dermatology, Inc. 32s VOL. 88, NO. 3 MARCH SUPPLEMENT 1987 POLYMORPHOUS LIGHT ERUPTION 33$ spring eruption" [20,21], which is probably part of the PLE spec­ borne contact dermatitis. Prurigo associated with sun exposure trum. Actinic prurigo [22,23], however, is in our view a separate might by seen in actinic prurigo, which represents an entity sep­ disorder that bears resemblance to the hereditary variant of PLE arate from PLE. The reason we do not observe eczematous lesions among North, Central, and South American Natives (18,24]. in our patients might be the lack of chronic exposure to high­ Features that distinguish these disorders from PLE are involve­ intensity solar radiation. In the F. R. G. the summer season is ment of non-sun-exposed areas, lack of complete clearing during short and longer periods of sunny weather conditions are rare. the winter season, and presence of chronic eczematous lesions. In our patients [8,11] the papular type, along with the hem­ In our experience, 90% of PLE patients are women. This is in orrhagic and insect bite (strophulus)-like varieties, the plaque accordance with the findings of Clorius and Jung [6] and Jansen type, the vesiculobullous type, and an erythema multiforme-like [13]. Others have found a preponderance of men [25,26] or an type make up the spectrum of PLE. We do not share the opinion equal sex distribution [27,28]. The reason that, in some studies, that the clinical picture might be polymorphous in the same in­ women are more commonly afflicted is not well understood. It dividual [5,6] or that combination or transition between the var­ is possible diat the preponderance of females does not reflect the ious types might occur [7]. In our series skin lesions were mon­ true prevalence, which might be equal, but rather might reflect omorphous in any one patient. a higher rate of reporting of the rash by women. Papular Type It is the most common type of PLE. Small Lesions of PLE are confined to sun-exposed body sites. In papules or papulovesicles are disseminated or densely aggregated contrast to others [5,7,13] we have neither observed lesions on on a patchy erythema (Figs lA,2A). areas covered by clothing nor a tendency of the eruption to gen­ eralize. In our experience, PLE is confined to certain sites of Hemorrhagic Type Rarely, the papular lesions are hemor­ predilection even when large areas are sun-exposed. In order of rhagic. decreasing frequency of involvement, these sites are: V-neck, Insect bite (Strophulus)-like Type This is a peculiar, rare dorsal forearm and back of hands, upper arms, face, shoulders, variety of the papular type. The lesions are few, scattered, and thighs,and lower legs. Variations among different patients occur. consist of small urticarial papules topped by a tiny vesicle. In exquisitely sensitive individuals larger parts of the trunk are involved, too. Jansen [13] notes that lips and eyes are frequently Plaque Type This is the second most common variety in our involved, and reports that patients experience general symptoms, patients. It occurs frequently on the face [7] and resembles sub­ like chills, headache, fever, and nausea after sun exposure. acute cutaneous lupus erythematosus (SCLE), with its sharply The reaction is of a delayed type. Lesions appear from several demarcated, erythematous, elevated, often urticarial plaques. hours to a few days after intense sun exposure. A characteristic Erythema Multiforme-like Type It shows typical target le­ sequence of events is reported by most patients, and the dynamics sions and occurs less frequently. ofthe lesions can be observed when PLE is experimentally evoked by phototesting. Itching occurs first; it is followed by patchy Vesiculobullous Type It consists of tense vesicles and small erythema.