Dorfman-Chanarin syndrome.
Indian J Dermatol Venereol Leprol
;
2007 Jan-Feb; 73(1): 36-9
Artigo
em Inglês
| 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.
Texto completo:
DisponíveL
Índice:
IMSEAR (Sudeste Asiático)
Assunto principal:
Sistema Porta
/
Pele
/
Síndrome
/
Vacúolos
/
Fibrose
/
Feminino
/
Humanos
/
Pré-Escolar
/
Ictiose Lamelar
/
Hepatócitos
Idioma:
Inglês
Revista:
Indian J Dermatol Venereol Leprol
Ano de publicação:
2007
Tipo de documento:
Artigo
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