Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia.
Primary lymphoedema associated with chylous reflux is a very rare clinical entity. We report a 3-year-old girl with unilateral lymphoedema, xanthomatosis and vaginal lymphorrhoea. Biopsy also revealed intestinal lymphangiectasia. This paper also presents a brief review of the literature and draws attention to the significance of the xanthomatous eruption in the diagnosis of a chylous reflux.
Karg
E
E
Department of Paediatrics, University of Szeged, Albert Szent-Györgyi Medical School, PO Box 471, H-6721 Szeged, Hungary. karg@pedia.szote.u-szeged.hu
Bereczki
C
C
Kovacs
J
J
Korom
I
I
Varkonyi
A
A
Megyeri
P
P
Turi
S
S
D002675:Child, Preschool; D004200:Diseases in Twins; D005260:Female; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000150:complications* / Q000175:diagnosis; D008209:Lymphedema / Q000150:complications* / Q000175:diagnosis; D008214:Lymphocytes; D014034:Toes; D019522:Vaginal Discharge / Q000150:complications* / Q000175:diagnosis; D014973:Xanthomatosis / Q000150:complications* / Q000175:diagnosis