HEREDITARY SCLEROSING POIKILODERMA OF WEA RY

Citation
M. Fazio et al., HEREDITARY SCLEROSING POIKILODERMA OF WEA RY, Annales de dermatologie et de venereologie, 122(9), 1995, pp. 618-620
Citations number
9
Categorie Soggetti
Dermatology & Venereal Diseases
ISSN journal
01519638
Volume
122
Issue
9
Year of publication
1995
Pages
618 - 620
Database
ISI
SICI code
0151-9638(1995)122:9<618:HSPOWR>2.0.ZU;2-D
Abstract
Introduction. Hereditary sclerosing poikiloderma is a genodermatosis w ith dominant autosomal transmission and variable penetration. The firs t case was described by Weary in 1969 in 7 members of two black famili es. Case report. A 10-year-old girl had localized regional poikiloderm a of the fingers and club toes. These lesions were associated secondar ily with linear symmetric bands of sclerotic tissue in the axiallary r egions. On the X-ray examinations of the distal phalanges of the finge rs and the toes showed a proximal growth foyer and absent ungueal phal anges, excepting in the fourth finger of the left hand. Capillaroscopy of the supra-ungueal fold of the fingers showed abnormal capillary ci rculation. Histology and ultrastructural examinations did not reveal a ny pathognomonic alterations. Discussion. This case is the first repor ted in a white patient. The radiological aspect and the results of the capillaroscopy of the fingers and the toes have not been reported pre viously in this rare genodermatosis. Inheritance of this genodermatosi s is poorly defined.