Autoimmune bullous diseases (AIBD) comprise a group of disorders in which autoantibodies target structural proteins of the intracellular junctions of the skin and mucous membranes or dermal-epidermal junction, leading to bullae formation. They are divided into intraepidermal AIBD, which include pemphigus vulgaris (PV) and its variants, and subepidermal AIBD, which include bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA bullous disease (LABD), dermatitis herpetiformis (DH), epidermolysis bullosa acquisita (EBA), bullous lupus erythematosus (BLE), and lichen planus pemphigoides (LPP). Among them, LABD and DH are most often reported in children, while others are uncommonly seen. 1-5 Data describing the spectrum of AIBD among children are scarce. 4-10 Due to
Febrile ulceronecrotic Mucha-Habermann disease is a rare fulminant variant of pityriasis lichenoides et varioliformis acuta, characterized by a rapidly progressive course with predominant ulceronecrotic lesions associated with fever and systemic manifestations. It carries a great morbidity and is potentially fatal. The exact pathogenesis is not clear, and it has been proposed to be the result of hypersensitivity reaction to an infection. We report a patient with febrile ulceronecrotic Mucha-Habermann disease in a 12-year-old boy in whom the condition was most likely precipitated by parvovirus infection, and he showed a favorable response to a combination of prednisolone with narrow band ultraviolet B (NB-UVB) phototherapy.
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