Summary Background Anti‐p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin γ1. The clinical course of anti‐p200 pemphigoid in patients remains poorly investigated. Objectives We aimed to describe the clinical and immunological features and the course of a series of patients with anti‐p200 pemphigoid. Methods We conducted a retrospective study by immunoblotting detection of sera on 200‐kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients. Results A total of 14 patients with a mean age 81·6 ± 6·5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including eczematous, urticarial, prurigo‐like, dyshydrosis‐like and rosette‐like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200‐kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme‐linked immunosorbent assay detected recombinant human laminin γ1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow‐up time of 12·6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy. Conclusions Many patients with anti‐p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected. What's already known about this topic? Anti‐p200 pemphigoid is a recently described rare autoimmune subepidermal blistering disease. The diagnosis of anti‐p200 pemphigoid requires immunoblotting detection of serum antibodies directed against a 200‐kDa dermal protein, corresponding to laminin γ1. Anti‐p200 pemphigoid has been associated with psoriasis and patients are thought to have a favourable prognosis. What does this study add? Patients with anti‐p200 pemphigoid had heterogeneous clinical presentation, which did not allow clear differentiation between anti‐p200 pemphigoid and other autoimmune bullous diseases of the dermoepidermal junction. Relapses were frequently observed despite the use of systemic treatments in most patients. We observed a high mortality rate, which was close to that of bullous pemphigoid. Linked Comment: Geller and Sprecher. Br J Dermatol 2016; 175:676–677.