Infection in autoimmune bullous diseases: a retrospective comparative study

Certain autoimmune bullous diseases (AIBD), including pemphigoid and pemphigus, confer increased infection risk. Infections have not been systematically studied in these conditions, however. Little is known about infection risk in these conditions, particularly dermatitis herpetiformis (DH). We aimed to characterize and compare infection patterns and risk factors in patients with pemphigoid, pemphigus, and DH. We retrospectively studied the medical records of Olmsted County, Minnesota, residents who had a diagnosis of AIBD between 1 January 1998 and 1 January 2011. Of 81 patients studied, 54 (67%) had pemphigoid, 11 (14%) had pemphigus and 16 (20%) had DH. Most patients studied developed at least one localized infection (72%) or one systemic infection (83%). Almost one-third of patients (31%) developed infections requiring hospitalization or contributing to death. All patients taking systemic corticosteroids experienced a localized or systemic infection during the follow-up period. Systemic infections were significantly less frequent in patients with DH than those with pemphigoid or pemphigus (P = 0.03), as were systemic infections requiring hospitalization or contributing to death (P = 0.002). Patients with DH were significantly less likely to require systemic corticosteroids (P < 0.001) and significantly more likely to receive dapsone (P = 0.002). The study design was retrospective and a limited number of patients met the inclusion criteria. Patients with AIBD frequently developed localized and systemic infections, a substantial portion of which contributed to hospitalization or death. Patients with DH experienced infections of lesser severity than patients with pemphigoid or pemphigus.