Introduction. Hereditary sclerosing poikiloderma is a genodermatosis with dominant autosomal transmission and variable penetration. The first case was described by Weary in 1969 in 7 members of two black families. Case report. A 10-year-old girl had localized regional poikiloderma of the fingers and club toes. These lesions were associated secondarily with linear symmetric bands of sclerotic tissue in the axillary regions. On the X-ray examinations of the distal phalanges of the fingers and the toes showed a proximal growth foyer and absent ungueal phalanges, excepting in the fourth finger of the left hand. Capillaroscopy of the supra-ungueal fold of the fingers showed abnormal capillary circulation. Histology and ultrastructural examinations did not reveal any pathognomonic alterations. Discussion. This case is the first reported in a white patient. The radiological aspect and the results of the capillaroscopy of the fingers and the toes have not been reported previously in this rare genodermatosis. Inheritance of this genodermatosis is poorly defined.
|Translated title of the contribution||Hereditary sclerosing poikiloderma of Weary|
|Number of pages||3|
|Journal||Annales de Dermatologie et de Venereologie|
|Publication status||Published - 1995|
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