[Epidermolysis bullosa acquisita: diagnosis by optic immunofluorescent demonstration of junctional antigens and vitamin E treatment].
In a patient with epidermolysis bullosa acquisita the characteristic dermolytic cleavage was demonstrated by electron microscopy and by mapping of antigenic determinants (type IV collagen, laminin, bullous pemphigoid antigen) of the dermal-epidermal junction. The latter method represents a rapid and reliable way to determine the cleavage plane in diseases which display subepidermal blister formation at the light-microscopic level. The classification of epidermolysis bullosa acquisita is still under dispute. Due to its highly characteristic clinical, ultrastructural, and immunologic features and pending further experimental data, epidermolysis bullosa acquisita should be regarded as a separate disease entity; its lumping together with cicatricial pemphigoid, as proposed by some authors appears speculative. Therapy of epidermolysis bullosa acquisita is generally regarded as difficult; following a 3-year course of high dose vitamin E therapy our patient underwent complete clearing; the possibility of a spontaneous remission, on the other hand, cannot be unequivocally ruled out.
D000328:Adult; D004820:Epidermolysis Bullosa / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D000939:Epitopes / Q000276:immunology*; D005455:Fluorescent Antibody Technique; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D012867:Skin / Q000648:ultrastructure; D014810:Vitamin E / Q000627:therapeutic use*
D000939:Epitopes; D014810:Vitamin E
Epidermolysis bullosa acquisita: Diagnostik durch immunfluoreszenzoptische Darstellung von junktionalen Antigenen und behandlung mit Vitamin E.