SYNDROME UNCOMBABLE HAIR - OBSERVATION OF 6 MEMBERS OF A FAMILY WITH PILI CANALICULI, PILI TORTI, PROGRESSIVE ALOPECIA, ATOPIC ECZEMA AND HAMARTOMAS

  • 1 January 1982
    • journal article
    • research article
    • Vol. 33  (7) , 366-372
Abstract
Six members of 1 family showed the typical clinical picture of uncombable hair (cheveux incoiffables), a syndrome first described by Dupre, Rochiccioli and Bonafe in 1973. In all 5 cases scanning electron microscopical investigations revealed characteristic morphological hair changes including longitudinal grooves as well as angular or kidney-shaped patterns in cross sections. Genetically the abnormality seems to be of autosomal dominant heredity with incomplete penetrance. In addition to uncombable hair some patients showed pili torti, progressive alopecia areata and atopic eczema. Possible relations of this syndrome with other diseases as pili torti, kinky hair disease or hypotrichia congenita are discussed.