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Bullous pemphigoid

23 February 2017, by BERNARD Ph.

Bullous pemphigoid (also known as pemphigoid) is the most common chronic blistering autoimmune disease. It accounts for 70 percent of all subepidermal autoimmune skin disorders with over 400 new cases reported every year in France [3, 20]. It is usually a disease of the elderly (mean age in France of about 80 years old). Immunologically, it is caused by autoimmunisation against two proteins which are components of the hemidesmosome adhesion complex with a molecular mass of 230 kDa (called BPAG1 or AgPB230, located in the intracellular portion of the hemidesmosome) and 180 kDa (called BPAG2, AgPB180 or collagen XVII, a collagen and transmembrane structure), respectively [9, 23]. It is a serious disease which continues to have a high mortality rate in France (about 30 percent after one year of treatment) [18, 20, 27]. Clinically, it presents as tense and often large bullae or blisters containing a clear liquid, generally on erythematous skin. It generally causes intense and debilitating itching. The lesions are symmetrical and tend to have a flexural distribution or are located on the anterior/internal surfaces of the thighs and abdomen. Mucosal involvement is rare and tends to be restricted to the oral mucosa (10 to 20 percent of cases). Nikolsky’s sign is absent. Clinically, the mode of onset, spread and progression of the disease are rather variable. Patients present with serial flares and the blisters heal without leaving scars. The systemic effects are variable and depend on the surface covered by the lesions, the severity of the pruritus and also its time of onset.


EADV leaflet published in 2019, produced by the EADV Auto-Immune Bullous Diseases Task Force:

- Bullous Pemphigoid


International Pemphigus & Pemphigoid Foundation 

ERN-SKIN: Bullous Pemphigoid

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