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A 54-year-old man is described, suffering from adult linear IgA bullous dermatosis with involvement of the bronchial mucosa. The main respiratory symptoms were recurring haemoptysis, episodic narrowing of the airways and persistent non-specific bronchial hyperreactivity. On CT scan the trachea had a saber-sheath shape with tracheal ring calcification. Endoscopically the tracheo-bronchial mucosa was diffusely purpuric and hyperaemic and also showed pale elevated plaques, bullous lesions and ulceration. Histological examination of biopsies of skin and nasal and tracheo-bronchial mucosa showed subepithelial blister formation associated with an accumulation of polymorphonuclear cells at the epithelial-subepithelial junction, and linear IgA deposits on direct immunofluorescence.
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Verhelst et al. (1987) studied this question.
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