[Bullous and hereditary Weary-Kindler's acrokeratotic poikiloderma (author's transl)].
Two observations of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler are studied. They are transmitted in dominance on two generations. The neonatal presentation of the first observation makes us discuss an epidermolysis bullosa; but the spontaneous regression, the appearance of a reticular pigmentation and the analysis of the paternal dyschromia allow the correction of the diagnosis. Forty-one observations are actually known. The blisters are present in 95 p. 100 of the cases, the dyschromia in 90 p. 100 and the keratosis on 60 p. 100. The trisymptomatic and bisymptomatic forms prevail. The mechanism of the disease is unrecognized but the vacuolization of the basal epidermic cells seems to be at the origin of the bullous eruption.
D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D007642:Keratosis / Q000150:complications / Q000235:genetics* / Q000473:pathology; D008297:Male; D010859:Pigmentation Disorders; D011038:Rothmund-Thomson Syndrome / Q000150:complications / Q000235:genetics*; D012871:Skin Diseases / Q000235:genetics*; D012872:Skin Diseases, Vesiculobullous / Q000150:complications / Q000235:genetics* / Q000473:pathology
Acrokératose poïkilodermique bulleuse et héréditaire de Weary-Kindler.