[Simple epidermolysis bullosa. Anatomo-clinical considerations with respect to 7 cases].
Seven patients, belonging to seven different families, are reported. The clinical picture is dominated by the bullous lesions which appeared during the delivery or soon after birth; in one patient (case 4) the lesions first appeared after the first year of life. Besides the bullae there were erosions, crusts, residual macules but no millia cysts or scars. The nails were affected in several patients; the mucosae were affected in 2 patients. The histological aspects of the lesions are described; in all the cases it was possible to establish the position of the cleavage in regard to the functional membrane. The patients were treated with many different kinds of treatment which included topical and systemic corticotherapy; recently alpha-tocopherol (Roche) was assayed; none of the medicaments showed efficacy. The management of the patients relies on protective measures in order to avoid the formation of new lesions; gentle and time consuming nursing care is necessary to treat the patients. This must be explained to the parents of the patients as the care will rely on them. It is important to explain also the basic cutaneous defect that provokes the skin trouble and the necessity to avoid trauma and the possibility of the appearance of a new child with the defect in any subsequent pregnancy. The situation improves with age and this form of E.B. is compatible with a normal adult life.
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D004820:Epidermolysis Bullosa / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D014810:Vitamin E / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D014810:Vitamin E
Epidermólise bolhosa simples. Considerações anatomo-clínicas a respeito de sete casos.