Litcius/Paper detail

Linear <scp>IgA</scp> bullous dermatosis of childhood: Retrospective single‐center cohort

Katherine Wang, Julia S. Lehman, Dawn Marie R. Davis

2024Pediatric Dermatology11 citationsDOI

Abstract

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

Topics & Concepts

MedicineRetrospective cohort studyCohortMucous membranePediatricsSingle CenterDiseaseReferralDermatologySurgeryInternal medicinePathologyFamily medicineAutoimmune Bullous Skin DiseasesCoagulation, Bradykinin, Polyphosphates, and AngioedemaUrticaria and Related Conditions