(BMJ)—An 18-mo boy had an erythematous vesiculobullous pruritic rash in the perioral and perineal areas that developed into serosanguineous bullae over ~70% of his body, sparing his mucosa. He was otherwise well and afebrile. Resolving central bullae became circumscribed by small vesicles, giving them a rosette-like appearance. Bx made the dx. What is it?
Bullous pemphigoid
Dermatitis herpetiformis
Pompholyx eczema
Bullous impetigo
Chronic bullous disease of childhood
You are correct. Chronic bullous disease of childhood (CBDC) was confirmed by direct immunofluorescence of biopsied perilesional skin showing linear IgA deposition along the basement membrane zone at the dermoepidermal junction. CBDC, also known as linear IgA bullous dermatosis, is a rare idiopathic or drug-induced IgA-mediated blistering disease. CBDC is the most common autoimmune bullous disorder in children and is often misdiagnosed. The key is recognizing the characteristic “string of pearls” or “rosette” pattern. This patient was treated w/ Dapsone as well as systemic glucocorticoid and azithromycin for additional anti-inflammatory effect. Complete remission was achieved by 8 weeks.
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(BMJ)—A 40-yo woman w/ hx of breast CA treated with surgery, chemo, and radiation on tamoxifen for 2 wks presents w/ her skin peeling off, which began when she dried w/ a towel after a bath. ROS: 1 wk malaise/fatigue. Exam: absence of epidermis on ~40% BSA. What is the dx?
Staph scalded skin syndrome
Pseudoporphyria
Exfoliative dermatitis
Toxic epidermal necrolysis
Paraneoplastic pemphigus
You are correct. The patient was clinically diagnosed w/ toxic epidermal necrolysis (TEN) thought to be secondary to her tamoxifen. Drug reactions are implicated in 80% to 95% of TEN cases. The ideal management for TEN includes treatment under aseptic conditions akin to the management of patients with burns.