JMIR Publications

ABSTRACT

Pemphigus vulgaris is a rare autoimmune blistering disorder involving the skin and mucous membranes. The disease may first develop or worsen during pregnancy, when diagnosis and treatment are complicated by maternal and fetal considerations. We report the case of a 37-year-old gravida 3 para 2 woman with poorly controlled type 1 diabetes mellitus and autoimmune hypothyroidism who developed painful oral and lip lesions at 19 weeks of gestation. The lesions were initially treated as oral candidiasis, herpetic lesions, and idiopathic aphthous ulceration. At 27 weeks, she developed a progressive generalized blistering eruption involving the trunk, extremities, oral mucosa, and genital region. Pemphigoid gestationis with secondary bacterial infection was initially suspected, and she was treated with topical corticosteroids, wound care, antimicrobial therapy, and subsequently low-dose oral prednisone. At 34 weeks and 6 days, she presented in labor after several days of reduced oral intake caused by worsening mucosal disease. She underwent emergency cesarean delivery and developed diabetic ketoacidosis requiring intensive care. A male infant weighing 2400 g was delivered with multiple cutaneous blisters. The neonatal lesions were clinically compatible with transient neonatal pemphigus, began healing during the first week of life, and were managed supportively. Postpartum maternal skin histopathology was interpreted as consistent with pemphigus vulgaris. Progressive disease was treated with systemic corticosteroids, topical therapy, a brief course of mycophenolate mofetil that was discontinued after liver enzyme elevation, and rituximab. Clinical remission was documented at 1-year follow-up. This case emphasizes that persistent painful oral erosions followed by generalized blistering during pregnancy should prompt early investigation for pemphigus vulgaris. In pregnant patients with type 1 diabetes, reduced oral intake, infection, corticosteroid exposure, and peripartum stress may collectively precipitate diabetic ketoacidosis, highlighting the importance of coordinated dermatologic, obstetric, endocrine, and neonatal care.