Indian Journal of Dermatology
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Year : 1994  |  Volume : 39  |  Issue : 4  |  Page : 73-75

Menkes' kinky hair syndrome a case report


Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012

Correspondence Address:
S Dhar
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh-160012

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ABSTRACT: Menkes'-Kinky disease, also known as trichopoliodystrophy, is an X-linked recessive disorder first described in 1962 by Menkes et al 1. The basic defect of copper metabolism incriminated in this disease was discovered 10 years later by Danks et al 2. In this disorder affected infants appear normal at birth but gradually develop all the features over a few months to years. The condition is incompatible with life and death usually occurs by 4 year of age 3. The incidence of premature birth is 50 percent 4. We herein describe this rare condition in a patient recently seen by us.


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