This patient had congenital lymphangectasia of the skin. Congenital lymphangectasia can affect other parts of the body. The most common form of lymphangectasia is intestinal which was first described by Waldmann.. Dilated lacteals result in lymph leaking into the bowel. This causes a protein losing enteropathy and pedal edema. Syndromes associated with intestinal lymphangectasia include von Recklinghausen syndrome, Turner’s , Kippel Trenaunay Weber, Noonan’s syndrome and Hennekam syndrome(intestinal lymphangectasia with facial anomalies and developmental delay). Other areas affected by lymphangectasia include the cornea, lungs, kidneys and retroperitoneum.
Lymphangectasia can also be acquired. It is caused by damage to deep lymph channels which can occur after mastectomy, radiation, or infection like filariasis, tuberculosis or lymphogranuloma venereum. In the case of tuberculosis lymph node enlargementcan cause delayed lymph drainage and swelling of the areas that would be drained by those nodes occurs as in the case of tuberculosis causing lymphagectasia of the vulva shown below.
Lymph node dissection for cancer can also cause lymphangectasia shown here in a post mastectomy patient.
Our patient was biopsied and the diagnosis was confirmed. No treatment was offered.
Ramesh M, Saldanha C, Kambil S, Dandakrei S. Cutaneous lymphangectasia of the vulva secondary to tuberculosis. Indian Journal of Sexually Transmitted Disease 2013 Jan-June 33(1) 35-37.
Vignes S, Balanger J. Primary intestinal lymphangectasia. Orphanet Journal of Rare Diseases 2008. 3:5.
Waldmann TA, Steinfeld DL. The role of the GI system in idiopathic hypoproteinemia. Gastroenterology 1966. 41: 197-207.
Hennekam RX. Autosomal recessive intestinal lymphangectasia and lymphedema with facial anomalies and mental retardation. Am J Med Gen 1989: 34;593-600