Linear cutaneous lupus erythematosus/discoid lupus erythematosus in an adult

Linear cutaneous lupus erythematosus (LCLE) is rare. The LCLE was proposed by Abe et al. [1] for discoid lupus erythematosus (DLE) with a linear configuration in 1988. It occurs mainly in children and young adults. Lesions appear as linear unilateral erythematous plaques following the Blaschko lines and are observed most frequently on the face, although the neck, trunk and extremities may also be affected [2]. Neither photosensitivity nor progression to systemic LE is observed [3]. To our knowledge, only 9 cases in adults have been reported in the literature. We report here the tenth case in an adult.
A 32-year-old woman presented with a 1-month history of slightly pruritic plaque on her left jaw and neck. These lesions first appeared on her jaw and spread to the left side of the neck in a linear arrangement. There was no history of trauma around the lesions, nor of intensive exposure to sunlight. The patient reported no fever, chills or photosensitivity. Her past medical history was unremarkable and there was no similar condition reported in her family members.
On physical examination, she had linear, slightly atrophic, reddish-brown plaque on her left jaw and neck (Figure 1 A) which did not follow the lines of Blaschko strictly (Figure 1 B). Laboratory investigations revealed positive antinuclear antibodies (ANA) with a titer of 1 : 80 (normal: < 1 : 40) and a granular fluorescence pattern, decreased complement C4 of 0.13 g/l (normal: 0.17–4 g/l) and decreased leukocyte of 3.62 × 109/l (normal: 4–10 × 109/l). Liver function tests, blood urea, creatinine and urine analysis were within normal limits. Antibodies against doublestranded DNA and Sm were negative. Skin biopsy from the lesion on the jaw revealed epidermal atrophy, follicular plugging and liquefaction degeneration in the basal layer of the epidermis. In the underlying dermis, inflammatory infiltrates of lymphocytes around the adnexal and vascular structures were observed (Figures 2 A, B). Alcian blue staining did not reveal obvious mucin depositions in the dermis (Figure 2 C). Direct immunofluorescence was not performed. Based on the clinical, laboratory, and histological findings, a diagnosis of LCLE was made. The patient was injected with 1 ml of a betamethasone injection and was also treated with desonide cream and tacrolimus 0.1% ointment for 1 month, the lesions stopped spreading and became darker (Figure 1 C).
The LCLE is a highly unusual variation of discoid lupus...


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