Bullous Systemic Lupus Erythematosus in a Male Child With Lupus Nephritis: A Rare Pediatric Presentation.

Bullous systemic lupus erythematosus (BSLE) is a rare blistering manifestation of systemic lupus erythematosus (SLE), characterized by widespread tense bullae resulting from autoantibodies against type VII collagen. It is predominantly seen in women and is exceptionally rare in pediatric males. We report a case of a 14-year-old South Asian male with recurrent tense blisters over the trunk, face, extremities, palms, and mucosa, alongside systemic features including photosensitivity, arthralgia, oral ulcers, and significant renal involvement. Laboratory findings revealed proteinuria, hypoalbuminemia, low complement levels, and positive antinuclear antibodies. Skin biopsy revealed subepidermal blisters with neutrophilic infiltrates, and direct immunofluorescence (DIF) demonstrated linear IgG, IgA, IgM, and C3 deposition at the basement membrane. Renal biopsy revealed membranous glomerulonephritis along with positive DIF showing IgG and C3 deposition. Treatment with corticosteroids, dapsone, and mycophenolate mofetil led to significant clinical improvement. This case emphasizes the need for a high index of clinical suspicion in young males presenting with blistering dermatoses, as BSLE may serve as an early marker of active systemic disease, particularly lupus nephritis. BSLE is considered an important diagnostic red flag, especially in the pediatric population, because its appearance may signal active underlying systemic disease, as seen in our patient with lupus nephritis. Sudden development of widespread tense blisters may coincide with systemic disease flares. Early recognition and aggressive management with immunosuppressants, along with close renal monitoring and long-term follow-up, are essential to reduce irreversible cutaneous and renal complications.