Anti-p200 pemphigoid is an autoimmune bullous disorder, first described in 1996. Clinically, it presents with tense blisters, erosions, crusts and erythematous macules/plaques, in addition to mucosal involvement in about 40% of cases, resembling bullous pemphigoid or the inflammatory variant of epidermolysis bullosa acquisita. As in other pemphigoid diseases, autoreactive IgG and/or C3 bind along the cutaneous basement membrane zone. Serum autoantibodies recognize a 200 kDa protein of the dermoepidermal junction that label the dermal side of human salt-split skin by indirect immunofluorescence microscopy. Subsequently, laminin γ1 has been described as a target antigen of anti-p200 pemphigoid. Laminin β4 has also recently been identified as a target antigen. A standardized, highly specific and sensitive detection system for serum anti-laminin β4 IgG is now widely available. This allows for the diagnosis of anti-p200 pemphigoid outside of specialized centres. Current in vitro and ex vivo data point to laminin β4 as the pathophysiologically relevant autoantigen, while in vivo data to clarify the functional relevance of anti-laminin β4 IgG are still lacking. Here, we summarize the current knowledge about the epidemiology, clinical presentation, diagnosis, target antigens, pathophysiology and treatment of this pemphigoid disease.
Autoimmune blistering diseases are a type of rare skin condition. They involve antibodies directed against the structure of the skin. This review focuses on ‘anti-p200 pemphigoid’. This condition was first described in 1996. Anti-p200 pemphigoid often looks similar to other autoimmune blistering diseases. It can share both clinical signs and immunological features with other conditions. This means that anti-p200 pemphigoid can be easily misdiagnosed without specific testing. In anti-p200 pemphigoid, antibodies have a target within the skin. This target was first reported as a ‘200 kDa’ protein found in extracts of human skin. The target was then described as ‘laminin γ1’. Most recently, ‘laminin β4’ has been identified as another autoantigen. This plays a crucial role in diagnosing anti-p200 pemphigoid. It is also central in understanding its underlying mechanisms. This review article summarizes the current knowledge about anti-p200 pemphigoid. We provide an overview of the epidemiology and clinical presentation of the disease. We also discuss the diagnosis, target antigens, pathophysiology and treatment of the condition.
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