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Bullous pemphigoid

Işın Sinem Bağcı, Orsolya N Horváth, Thomas Ruzicka, Miklós Sárdy
Autoimmunity Reviews 2017, 16 (5): 445-455
28286109
Bullous pemphigoid (BP) is the most common autoimmune bullous disorder which is characterized by autoantibodies against hemidesmosomal proteins of the skin and mucous membranes. Collagen XVII and dystonin-e have been identified as target antigens. BP affects mostly the elderly. The incidence of the disease is increasing gradually and is associated with high morbidity and mortality. Clinically, BP is characterized by an intensely pruritic eruption with widespread bullous lesions. The clinical diagnosis can be challenging in the setting of atypical presentations. Diagnosis of BP relies on the integration of clinical, histological, immunopathological, and serological findings. The treatment is mainly based on topical and/or systemic glucocorticoids, but anti-inflammatory antibiotics and steroid sparing adjuvants are useful alternatives. Localised and mild BP can be treated with topical corticosteroids alone.

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