Autoimmune Bullous Diseases of the Larynx

Overview

Autoimmune bullous diseases of the larynx are relatively rare. They include inparticular pemphigus and pemphigoid. In both instances diseases are involved which affect mainlythe skin, as to mucous membranes, most often the buccal mucosa is affected, however also themucosa of the larynx, hypopharynx and oesophagus may be affected. The diagnosis is histological(intraepidermal acantholytic blisters in pemphigus vulgaris and subepidermal blisters in pemphigoid) and by direct immunofluorescence. The main and best known therapeutic method is corticotherapy. The prognosis of untreated disease is bad, it is fatal due to general marasm or septicaemia.Treatment reduces the mortality markedly, the patient’s death is usually associated with complications of prolonged corticotherapy. The authors describe three cases of these diseases which weretreated recently at the ENT Clinic of the Third Medical Faculty, Charles University, Prague.

Key words:
pemphigus vulgaris, pemphigoid, direct immunofluorescence, corticotherapy.