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Linear IgA Bullous Disease of Childhood: An Experience from Kuwait
Author(s) -
Nanda Arti,
Dvorak Richard,
AlSabah Humoud,
Alsaleh Qasem A.
Publication year - 2006
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2006.00279.x
Subject(s) - medicine , dapsone , disease , incidence (geometry) , population , autoimmune disease , autoimmune hepatitis , glomerulonephritis , pediatrics , immunology , dermatology , physics , environmental health , optics , kidney
Linear IgA bullous disease of childhood is a rare autoimmune blistering disease. We report eight patients with this disease seen in our autoimmune bullous diseases clinic over a span of 12 years. They constituted 89% of the total number of those with linear IgA bullous disease of childhood seen during this period, with an age‐adjusted minimum estimated incidence of 2.3 cases/million population/year. Males outnumbered females by a 1.7:1 ratio. The age at onset ranged between 10.5 months and 13 years, with a mean of 6.8 ± 4.17 years. The majority of patients (62.5%) had moderately severe disease. Fifty percent of patients were observed to have an association with either an autoimmune disease (Crohn disease and post‐streptococcal glomerulonephritis in one each) or an infection ( β ‐hemolytic streptococcal and hepatitis A virus infection in one each). The treatment of choice was dapsone alone or in combination with systemic steroids. Seventy‐one percent of patients achieved complete remission by the end of 2 years. The study highlights the significance of systematic clinicoepidemiologic surveys from different regions.