A 72-Year-Old Man with Crusted Erosions on the Scalp

Abstract

Bullous pemphigoid is the most frequent autoimmune subepidermal blistering disease. It is characterized by the presence of tissue-bound and circulating autoantibodies against components of the hemidesmosomes: BP230 (BPAg1) and BP180 (BPAg2). The disease mainly affects elderly individuals, during the eighth decade of life, without gender predilection. Typically, tense blisters on the apparently normal or erythematosus skin are observed. In non-bullous phase of the disease, signs are extremely polymorphic and include excoriated, eczematous papular or urticarial lesions. Skin lesions are accompanied by intense pruritus and are most frequently localized on the trunk and extremities. Rarely the scalp area is affected. Diagnosis of bullous pemphigoid is established on a basis of immunofluorescence tests. Histopathology may be useful. The first-line therapy of bullous pemphigoid consists of potent topical corticosteroids. The combination of nicotinamide and tetracyclines or doxycycline may be a therapeutic option. In refectory cases oral prednisone with steroid-sparking agents such as azathioprine, mycophenolate mofetil or methotrexate are recommended.