IgA Pemphigus: A Systematic Review

Abstract Background The clinical, histologic, and immunopathologic features of IgA pemphigus have not been studied on a large scale. Objective To synthesize existing data on the epidemiological, clinical, histological, and immunological features of IgA pemphigus. Methods We performed a systematic review using Medline, Embase and Web of Science databases. Case reports and series of patents with IgA pemphigus were included. Results 119 eligible studies comprising 137 patients with IgA pemphigus were included with a mean age of 51.5 ± 21.0 years. Most patients presented with vesicles (80.8%), pustules (75.0%), and circinate plaques (63.6%). Pruritus was present in 65.6% of reported patients. Intercellular deposition of IgA was noted in almost all patients (97.0%) and the remaining 3.0% patients had IgA positivity on indirect immunofluorescence (IIF), or ELISA confirming the diagnosis. IgA circulating intercellular antibodies were detected in only 66.7% patients. IgA gammopathy and ulcerative colitis were associated with IgA pemphigus in 9.5% and 6.6% patients, respectively. Oral dapsone and corticosteroids were the mostly commonly used treatments. Limitations Results are mainly grounded on case reports and small case series. Conclusions The diagnosis of IgA pemphigus may be considered in patients presenting with vesiculopustular eruption and circinate plaques with truncal and extremity involvement.