Abstract
IMPORTANCE: Immunoglobulin A (IgA) epidermolysis bullosa acquisita (EBA) is an autoimmune skin blistering disease with IgA autoantibodies directed against type VII collagen. There is debate whether it should be considered part of the clinical spectrum of linear IgA bullous dermatosis (LABD) or a separate disease entity. OBJECTIVE: This cohort study aimed to define the clinical features and treatment responses of IgA EBA and anti-BP180-driven LABD, and to compare the prevalences of IgA EBA anti-BP180 LABD and classic IgG-mediated EBA in an autoimmune diagnostic laboratory database. DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study and case series study included demographic, immunopathologic, and serologic data from 300 patients diagnosed with IgA EBA, IgG EBA, or LABD. Furthermore, clinical features and treatment responses of IgA EBA were analyzed in a case series including 4 patients with IgA EBA. All patients from the database of the autoimmune diagnostic laboratory at the Department of Dermatology, University of Lübeck, Germany, who were diagnosed with IgA EBA, LABD, or IgG EBA between October 2010 and July 2019 were included. Four patients diagnosed with IgA EBA between October 2015 and January 2018 are described in detail. MAIN OUTCOMES AND MEASURES: The clinical course of IgA EBA was observed before and during different treatments. RESULTS: The database search yielded 21 cases of IgA EBA (12 females [57%]/9 males [43%]), 222 cases of LABD (111 females [51%]/106 males [49]), and 57 cases of IgG EBA (29 females [50%]/28 males [48%]). The median (range) age of each cohort was 64 (4-81) years for IgA EBA, 56 (3-92) years for IgG EBA, and significantly older compared with IgG EBA (P = .002) for those with LAPD (median [range], 70 [1-94] years). The patients with IgA EBA exhibited heterogeneous clinical presentations that significantly differed from those of anti-BP180 LABD. All 4 patients with IgA EBA described in detail were first treated with dapsone, but only 1 responded to this treatment. The others required treatment with high-dose dexamethasone, rituximab, and/or intravenous immunoglobulins to achieve partial clinical remission. CONCLUSIONS AND RELEVANCE: Overall, the findings of this cohort study and small case series suggest that IgA EBA may be more common than expected and may require more intensive systemic treatment than LABD, suggesting it should be considered a separate disease entity.