[ XXXX of @entity73316 type].

@entity1 @entity1032 @entity73316 @entity6437 @entity814 @entity12458 @entity4243 @entity194
A familial case of @entity73316 , occurring in a mother and her daughter, is reported. A 12-year old @entity1032 @entity1 was seen with a history of repeated ecchymoses despite normal hemostasis. On clinical examination, the skin was diffusely atrophic with @entity194 and wrinkling most pronounced on the extremities. The venous pattern was noticeable on the upper aspect of the anterior trunk and the extremities. The feet were small and the nose was pinched. "Cigarette paper" scars and molluscoid @entity6437 were seen over the elbows and the knees. Some skin hyperextensibility was noted and @entity12458 of the fingers was demonstrated. The fingernails, hair, dentition, and bone roentgenograms were normal. The mother, a 34-year old @entity1 , presented with the same abnormalities and reported the same condition in her mother. A younger brother was normal and there was no history of consanguinity. In both cases, the skin biopsy specimens showed @entity194 with irregular increase of the elastic staining material in the mid dermis. Ultrastructural studies showed a widened endoplasmic reticulum within the fibroblasts. Biochemical studies, performed in the daughter, showed complete lack of collagen III in the dermis. This, together with the visceral complications occurring in the mother and in previously reported @entity1 , suggests that @entity73316 represents a clinical variant of @entity4243 . Its association with @entity814 in the daughter is considered to be coincidental.
