Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia

• Published in: British journal of dermatology (1951) Vol. 146; no. 1; pp. 134 - 137
• Main Authors: Karg, E., Bereczki, C., Kovacs, J., Korom, I., Varkonyi, A., Megyeri, P., Turi, S.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Science, Ltd 01.01.2002; Blackwell; Oxford University Press
• Subjects: Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Child, Preschool; chylous reflux; Diseases in Twins; Diseases of the lymphatic vessels; Female; Humans; intestinal lymphangiectasia; Lymphangiectasis, Intestinal - complications; Lymphangiectasis, Intestinal - diagnosis; Lymphedema - complications; Lymphedema - diagnosis; Lymphocytes; lymphoedema; Medical sciences; Toes; Vaginal Discharge - complications; Vaginal Discharge - diagnosis; vaginal lymphorrhoea; xanthoma; Xanthomatosis - complications; Xanthomatosis - diagnosis; Human; Xanthomatosis; Skin disease; Digestive system; Gut; Vagina; Cardiovascular disease; Metabolic diseases; Lipids; Enzymopathy; Lymphatic vessel disease; Case study; Association; Lymphangiectasis; Lymphedema; Hyperlipemia; Dyslipemia; Child
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1046/j.1365-2133.2002.04477.x

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.