A Unique Case of Lupus Erythematosus Profundus Presenting With Localized Scarring Alopecia and Perioral Swelling in a Pediatric Patient

Lupus erythematosus profundus, also known as lupus panniculitis, is a rare subtype of cutaneous lupus erythematosus characterized by inflammatory involvement of the deep dermis and subcutaneous adipose tissue. It is predominantly reported in adults, whereas pediatric presentations remain uncommon. A five-year-old Middle Eastern girl presented with a two-month history of progressive localized alopecia involving the left parietal scalp, associated with perioral swelling predominantly affecting the upper lip. Examination showed an alopecic patch measuring approximately 4 × 2 cm with reduced follicular openings, suggestive of early scarring alopecia, and diffuse upper lip swelling with surrounding mild erythema. Trichoscopy demonstrated decreased follicular density and areas of absent follicles. Laboratory evaluation revealed elevated inflammatory markers with negative antinuclear antibody testing and normal complement levels. Histopathological examination of scalp and nasolabial fold biopsies demonstrated predominant lobular lymphocytic and histiocytic panniculitis extending into the subcutaneous fat, consistent with lupus erythematosus profundus. Treatment with oral hydroxychloroquine led to significant improvement in lip swelling and stabilization of the scalp lesion, with subsequent hair regrowth on follow-up. Lupus erythematosus profundus is an uncommon but important cause of localized alopecia and facial swelling in children. Early clinicopathologic recognition and timely treatment are essential to reduce the risk of permanent scarring and disfigurement.