(142) An atypical presentation of bullous SLE: a diagnostic challenge

Track
Case Reports
Abstract

Linear IgA bullous dermatosis (LAD) encompasses a spectrum of acquired immunobullous disorders that are associated with the isolated deposition of IgA at the basement membrane zone as detected by immunofluorescence. Bullous SLE (BSLE) is a rare immunobullous disorder mediated by anti-COL7 antibodies in the setting of SLE. There are case reports of patients with SLE having an additional diagnosis of linear IgA bullous dermatosis, rather than bullae related to BSLE, largely based on the pattern of direct immunofluorescence. Here we present the case of a pediatric patient initially diagnosed with linear IgA bullous dermatosis, in the absence of any significant systemic symptoms, who subsequently developed severe symptoms of SLE including lupus nephritis and neurological sequelae.

Published in: ASDP 61st Annual Meeting

Publisher: The American Society of Dermatopathology
Date of Conference: November 4-10, 2024