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Comedonic discoid lupus erythematous

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Cutaneous manifestations of lupus erythematosus (LE) are mostly distinctive enough to permit a straightforward diagnosis (1). However, misleading presentation occasionally occurs in LE. We herein describe an uncommon case of… Click to show full abstract

Cutaneous manifestations of lupus erythematosus (LE) are mostly distinctive enough to permit a straightforward diagnosis (1). However, misleading presentation occasionally occurs in LE. We herein describe an uncommon case of an LE patient displaying comedones and pitting scars, reminiscent of acne vulgaris. A 54-year-old Chinese male had had a patch of hair loss with comedones on the scalp for 2 years. According to the patient’s report, an erythematous plaque had arisen before the comedones developed. Conventional treatments for acne did not help. He denied any history of malar erythema, oral ulceration, or arthralgia. His medication was non-contributory. Skin examination revealed a patch of cicatricial alopecia on the scalp with an erythematous and telangiectatic setting. In the lesion, there were conspicuous cribriform openings and comedones. The comedones were whitish to brown, of different tones, with diameter ranging from 2 to 3 mm. There were no pustules, nodulocystic lesions, or scars (Figure 1). A skin biopsy showed hyperkeratosis, follicular plugging, and epidermal inclusion cysts. There was an extensive vacuolar degeneration of the basal cells. Additional features noted included heavy perivascular and periadnexal mononuclear cell infiltration (Figure 2). Routine laboratory tests found a normal full blood count. Antinuclear antibody, anti-Sm antibody, and anti-double-stranded DNA antibody screens were all normal. Urinalysis and complement were within normal limits. These clinical and pathological findings were consistent with the diagnosis of discoid lupus erythematous (DLE). He was commenced on oral hydroxychloroquine and was topically treated with tretinoin 0.01% cream for 3 months. The number of comedones reduced, with no improvement of the alopecia patch. DLE is a chronic disorder characterized by scaly, erythematous plaques, which usually affect sun-exposed skin (1). It encompasses a spectrum of mucocutaneous conditions. The most common types include hypertrophic, lichenoid, and mucosal DLE (1). The scalp, face, ears, and other photo-exposed regions are preferentially affected in DLE. If the scalp is affected, it starts as macules, papules, or plaques, and progresses to become an erythematous hairless patch. As the lesions age, hair follicles are destroyed irreversibly, forming conspicuous scarring alopecia (2). Comedonal (acneform) LE is an unusual variant of cutaneous LE, and only 10 cases have been reported previously (2–6). It takes the appearance of acne-like pitting scars with open comedones, and acneform LE is alternatively used to describe the additional presence of inflammatory follicular papules, nodules, and cysts (6). Most cases of acneform LE are females, with the age of onset between 20 and 40 years. It most frequently occurs on the nasolabial fold, in the periocular region, and on the nostril rim (6). We herein described the second case of comedonic DLE involving the scalp. The diagnosis of comedonic DLE was established mainly by the histopathological changes. It features hyperkeratosis, vacuolar degeneration of basal cells, and lymphocytic perivascular and peri-appendageal infiltration of the lymphocytes (1, 4). In the later stages, follicular plugging, atrophic epidermis, and thickening of the basement membrane zone may easily be found (1, 6). Our patient’s histology was consistent with the notion that comedones are part of the primary DLE presentation. If non-definitive histological findings present, direct immunofluorescence of lesional biopsies is helpful in obtaining a correct diagnosis.

Keywords: dle; lupus erythematous; discoid lupus; diagnosis

Journal Title: Scandinavian Journal of Rheumatology
Year Published: 2019

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