Indian Journal of Dermatology, Venereology and Leprology
Medknow Publications on behalf of The Indian Association of Dermatologists, Venereologists and Leprologists (IADVL)
Vol. 73, No. 1, 2007, pp. 36-39
Bioline Code: dv07010
Full paper language: English
Document type: Research Article
Document available free of charge
Indian Journal of Dermatology, Venereology and Leprology, Vol. 73, No. 1, 2007, pp. 36-39
© Copyright 2007 Indian Journal of Dermatology, Venereology and Leprology.
Case Report - Dorfman-Chanarin syndrome|
Gandhi Vijay, Aggarwal Puneet, Dhawan Jyoti, Singh UshaRani, Bhattacharya SN
A four-year-old girl was brought to the dermatology outpatient department with scaling all over the body since birth. She had history of episodic vomiting and abdominal distension. A dermatological diagnosis of lamellar ichthyosis was made. Abdominal examination revealed a nontender hepatomegaly, fatty liver on ultrasonography and deranged liver function tests. Peripheral blood smear showed lipid vacuoles in the granulocytes consistent with Jordans′ anomaly. Similar lipid vacuoles were seen in the basal layer in skin biopsy. An inflammatory infiltrate, moderate fibrosis in the portal tract and diffuse severe fatty change in hepatocytes were seen in liver biopsy. The patient was diagnosed as a case of Dorfman-Chanarin syndrome.
Congenital ichthyosis, Dorfman-Chanarin syndrome, Jordans′ anomaly
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