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Keratoma Hereditaria Mutilans (Vohwinkel's Disease) with Congenital Alopecia Universalis (Atrichia Congenita)
Author(s) -
Bhatia Krishan Kumar,
Chaudhary Satdev,
Pahwa Ujjal Singh,
Mehrotra Girish Chander
Publication year - 1989
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1989.tb01255.x
Subject(s) - scalp , alopecia universalis , medicine , dermatology , hair shaft , dermis , thickening , hair disease , pathology , hair follicle , chemistry , polymer science
A family is described in which a boy and two girls had features of keratoma hereditaria mutilans (Vohwinkel's disease) and congenital absence of hair. None of them had any hair at birth or developed any subsequently. They had congenital, nonscarring alopecia universalis. The skin over the palms and soles showed progressive thickening, which led to mutilating deformities of the hands and fingers. Histopathological examination of the scalp skin revealed no changes in the epidermis or dermis with normal sebaceous glands and identificable hair structures; the skin on the palms showed a tremendously thickened horny layer. Both conditions remained unresponsive to various topical and systemic remedies used in the past. One of the girls had a meningocoele of the dorsolumbar region and died postoperatively. The simultaneous appearance of two rare hereditary diseases in siblings of one family is being reported for the first time.