[Manifestation of epidermolysis bullosa acquisita (EBA) in the ENT area].
Epidermolysis bullosa acquisita (EBA) is a rare, chronic, acquired bullous autoimmune dermatosis. It is characterized by the formation of IgG autoantibodies against type VII procollagen of anchoring fibrils with subepidermal formation of bullous lesions and consequent scarring. The epidemiology of this disease and its characteristic clinical findings cannot be completely surveyed at present due to the limited number of available publications. In general, bullous lesions form on the entire integument and can also involve mucosa. The development of scar-related adhesions on the mucosa of the upper airways and esophagus can lead to serious complications that are difficult to treat. We report our experience in managing a 44-year-old male patient in whom the diagnosis of EBA was established in 1993 on the basis of multiple persistent bullous lesions involving the eye, nose, skin and oral, pharyngeal and laryngeal mucosa. After failing previous medical and surgical therapies, interdisciplinary management resulted in the control of active lesions with extracorporal phototherapy and cyclosporin A.
Klinik für Hals-, Nasen-, Ohrenkrankheiten, Gesichts- und Halschirurgie, Martin-Luther-Universität Halle-Wittenberg.
M
M
Bloching
E
E
Dippel
S
S
Jovanovic
M
M
Hess
C C
CC
Zouboulis
D000328:Adult; D001323:Autoantibodies / Q000097:blood; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000008:administration & dosage; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D009092:Mucous Membrane / Q000276:immunology / Q000473:pathology; D010038:Otorhinolaryngologic Diseases / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D010348:Patient Care Team; D010789:Phototherapy; D011347:Procollagen / Q000276:immunology
D001323:Autoantibodies; D007074:Immunoglobulin G; D011347:Procollagen; D016572:Cyclosporine
Manifestation der Epidermolysis bullosa acquisita (EBA) im HNO-Gebiet.