SKIN-PICKING DURING PRADER-WILLI-SYNDROME

Introduction. The Prader-Labhart-Willi syndrome was first described in 1956. Prader-Labhart-Willi syndrome is the most common genetic form o f human obesity and the incidence of Prader-Labhart-Willi syndrome has been estimated to 1 in 10 000 or 25 000 live births. Skin-picking was frequently reported in Prader-Labhart-Willi syndrome and two patients who displayed repetitive skin picking are described. Observations. Tw o childrens (6 year-old girl and 7 year old boy) were examined and not ed superficial ulcers of their arms and Iegs. This cutaneous lesions w ere induced by children themselves. Skin-picking, in our cases, were a ssociated with behavior problems (temper tantrums, violence). Conclusi ons. Skin-picking appears to occur in the great majority of patients w ith Prader-Labhart-Willi syndrome and constitutes a minor criteria of diagnosis. Hypopigmentation in Prader-Labhart-Willi syndrome appears t o be as common as previously features. Significant differences in hair color, sun sensitivity and complexion were found between theses patie nts with chromosome 15 deletion and those with normal chromosome. Asso ciation between obesity (onset before 6 years) and skin picking consti tute a sign for diagnosis of Prader-Labhart-Willi syndrome.