BP230-Type Bullous Pemphigoid is a Rare Subset of Bullous Pemphigoid Associated With a Favorable Prognosis: A Retrospective Analysis From a Tertiary Referral Center

Bullous pemphigoid (BP) is the most common autoimmune bullous disease. It usually presents with widespread blistering of the skin and intense pruritus; atypical presentations lacking blisters have also been reported. BP is associated with autoantibodies targeting the NC16A domain of Collagen XVII (BP180). Pathogenic activity of BP230-IgG has also been reported in BP. A minority of patients have elevated serum levels of BP230-IgG while being negative for BP180-NC16A-IgG in commercial enzyme-linked immunosorbent assays (ELISAs); whether these patients constitute a different subset of BP is still debated. We set up a monocentric retrospective analysis of clinical characteristics and outcomes of patients diagnosed with BP230-type BP over a period of 3 years. In our series, BP230-type BP was preferentially associated with female sex, but not with specific comorbidities, such as neurological diseases. Regarding prognosis, BP230-type BP ran a benign clinical course, confirming the positive prognostic implication of BP180-IgG negativity in BP.