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Abstract

Background Discoid lupus erythematosus (DLE) represents the most common variant of chronic cutaneous lupus
erythematosus, a skin-specific autoimmune disorder within the broader lupus erythematosus spectrum. Clinically,
DLE is characterized by erythematous, scaly plaques with areas of hyperpigmentation. These lesions predominantly
occur on sun-exposed areas, particularly the scalp and cheeks. Involvement of the genital skin is rare and poses a
significant diagnostic challenge due to its clinical overlap with other inflammatory and infectious dermatoses. To
date, only twelve cases involving genital skin have been documented in the literature, most described in female
patients, with a particular tendency for the vulva to be affected. Case Presentation We present the case of a 60-year-
old female with a past medical history of systemic sclerosis and Raynaud’s syndrome, who presented with well-
demarcated, erythematous, keratotic plaques with atrophy, involving the inguinal creases and medial buttocks,
associated with pruritus and recent-onset of pain when sitting. The rash had been present for years. Clinical
differential diagnoses included lichen sclerosus, lichen simplex chronicus, and psoriasis. Histopathologic
examination revealed orthokeratosis and parakeratosis, epidermal hyperplasia, thickened basement membrane,
dilated blood vessels, and perivascular inflammation. Plasmacytoid dendritic cells were present in the dermis and
highlighted by CD123 immunohistochemical stain, supporting the diagnosis of DLE. Systemic evaluation was
negative for systemic lupus erythematosus. The patient was started on treatment with topical corticosteroids.
Conclusion This case highlights the importance of including DLE in the differential diagnosis of chronic genital
dermatoses and reinforces the value of histopathologic evaluation in such cases.