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.