Vi. Albanova, THE SIGNIFICANCE OF DOMINANTLY INHERITED ONYCHODYSTROPHIA IN THE DIAGNOSIS OF BULLOUS EPIDERMOLYSIS, Vestnik dermatologii i venerologii, (5), 1993, pp. 61-63
Three cases with dominant dystrophic bullous epidermolysis in infants
are described, in which analysis of the family history and examination
of the patients' parents helped specify the diagnosis. The parents de
nied the disease in the family in all the cases. Examinations showed d
ystrophic changes of the nails of the soles and sites of cicatricial a
trophy of the skin on the elbows, knees and ankles. Analysis of the fa
mily history revealed the presence of dominantly inherited onychodystr
ophy, developing at the age of 3-5, in all the families. The author su
ggests the existence of poorly manifest forms of the condition.