[Vegetative dystrophic epidermolysis bullosa. Urogenital manifestations and treatment with hydantoin].
Dystrophic epidermolysis bullosa has a rare recessive polydysplastic vegetant variant, which was described by Nicolas et al. Two siblings are reported. The male was affected by urethral lesions, an exceptional finding in this disease. Many members in family died of renal insufficiency . A variety of therapeutics was indicated along ten years. Only hydantoins caused a marked involution of the lesions. An interesting feature was that high drug levels in the blood produced not only common side effects, such as macrulia , but also exacerbation of the dermatosis and growth of new lesions, which took on a botriomicoid aspect.
D000293:Adolescent; D004820:Epidermolysis Bullosa / Q000139:chemically induced / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D006827:Hydantoins / Q000009:adverse effects / Q000627:therapeutic use; D008297:Male; D010375:Pedigree; D012008:Recurrence
D006827:Hydantoins
Epidermólisis ampollar distrófica vegetante. Manifestaciones urogenitales y tratamiento con hidantoína.