[Epidermolysis bullosa dystrophica inversa, a review and case report].
An additional case of epidermolysis bullosa dystrophica inversa is reported. A review of the literature shows that until now reports about probably 15 patients from seven families were published, although the first 13 patients were not investigated by electron microscope [3]. By blistering beneath the basal lamina similar to the Hallopeau-Siemens type these patients develop bullae predominantly at the trunk and the inverse sites of the axillar and genitoanal regions continuously or at close intervals starting shortly after birth. The mucous membranes of the mouth, the esophagus, and the cornea and conjunctiva of the eye are very often involved. By bacterial infection of the skin lesions the patients develop hpochrome anemia. The web-like structures in the esophagus following blistering can be successfully treated by careful dilatation because of the low tendency to scar formation. An autosomal recessive mode of inheritance seems to be most likely.
D000293:Adolescent; D000328:Adult; D004359:Drug Therapy, Combination; D004820:Epidermolysis Bullosa / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D004940:Esophageal Stenosis / Q000209:etiology / Q000473:pathology; D005260:Female; D005672:Fusidic Acid / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D008297:Male; D003012:Microbial Collagenase / Q000008:administration & dosage; D008854:Microscopy, Electron; D008875:Middle Aged; D010375:Pedigree; D012867:Skin / Q000473:pathology
D005672:Fusidic Acid; D003012:Microbial Collagenase
Epidermolysis bullosa dystrophica inversa; Ubersicht und Bericht eines Falles.