[Manifestation of epidermolysis bullosa acquisita (EBA) in the ENT area]

TY - JOUR

T1 - [Manifestation of epidermolysis bullosa acquisita (EBA) in the ENT area]

AU - Bloching, M

AU - Dippel, E

AU - Jovanovic, S

AU - Hess, Markus

AU - Zouboulis, C C

PY - 1999

Y1 - 1999

N2 - 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.

AB - 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.

M3 - SCORING: Zeitschriftenaufsatz

VL - 47

SP - 497

EP - 501

JO - HNO

JF - HNO

SN - 0017-6192

IS - 5

M1 - 5

ER -