Dorfman-Chanarin syndrome.
Indian J Dermatol Venereol Leprol
;
2007 Jan-Feb; 73(1): 36-9
Article
in English
| IMSEAR
| ID: sea-52577
ABSTRACT
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.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Portal System
/
Skin
/
Syndrome
/
Vacuoles
/
Fibrosis
/
Female
/
Humans
/
Child, Preschool
/
Ichthyosis, Lamellar
/
Hepatocytes
Language:
English
Journal:
Indian J Dermatol Venereol Leprol
Year:
2007
Type:
Article
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