(BMJ)—A 13-yo girl, diagnosed w/ systemic lupus erythematosus 3y prior, presented w/ progressive painful, round, red plaques on the extremities, trunk, and face. Exam: multiple red plaques w/ tissue sloughing and necrosis. Bx confirmed the dx. What is it?
Cutaneous leukocytoclastic vasculitis
Stevens-Johnson syndrome
Pyoderma gangrenosum
Bullous impetigo
Staphylococcal scalded skin syndrome
You are correct. Keratinocyte vacuolar degeneration on bx confirmed Stevens-Johnson syndrome (SJS). She was also diagnosed w/ Rowell syndrome, a rare subtype of systemic lupus erythematosus (SLE) characterized by SLE, erythema multiforme, speckled ANA-positive test, and positive anti-SSA/Ro and anti-SSB/La. Autoimmune dz is an uncommon cause of SJS. More-common causes include abx, anticonvulsants, HSV, and mycoplasma. SJS tx includes plasma exchange, hemoperfusion, steroids, mycophenolate mofetil, and intravenous immunoglobulins.

BMJ 2019;364:l1257