| Abstract | | A 16-day-old boy was admitted to our clinic with localized blisters on the neck, cheeks, earlobes, and oral cavity and with erythema on the toes, in addition to poor weight gain and respiratory distress. A physical examination revealed several erythematous plaques with tense bullae, multiple vesicles, and erosions on the left toes, neck, earlobes, and face as well as erosive lesions on the anterior part of the oral cavity, lips, and buccal mucosae. A bronchoscopic examination revealed bullous lesions in the upper respiratory tract and on the epiglottis. A skin biopsy suggested a diagnosis of bullous pemphigoid (BP). Because of the severe mucosal involvement, further investigations including various immunological techniques were performed. The case was diagnosed as BP associated with linear IgA bullous disease (LAD). Complete remission without any scarring was achieved after three weeks of oral methyl prednisolone treatment. A correct differential diagnosis of bullous diseases is important for determining the prognosis and expected response to treatment. To our knowledge, this is the first case of BP associated with LAD reported in literature.
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