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  Česky / Czech version Otorinolaryngol. /Prague/, 49, 2000, No. 2, p. 114-116
 
Autoimmune Bullous Diseases of the Larynx 
Čoček A., Hahn A., Průcha I. 

ORL klinika 3. LF UK a FNKV, Praha, přednosta doc. MUDr. Dr. med. Aleš Hahn, CSc.
 


Summary:

       Autoimmune bullous diseases of the larynx are relatively rare. They include in particular pemphigus and pemphigoid. In both instances diseases are involved which affect mainly the skin, as to mucous membranes, most often the buccal mucosa is affected, however also the mucosa 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 cortico therapy. 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 were treated recently at the ENT Clinic of the Third Medical Faculty, Charles University, Prague.

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

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