CONGENITAL MULTISEGMENTAL LYMPHATIC DYSPLASIA WITH SYSTEMIC INVOLVEMENT – EVALUATING PRIMARY LYMPHOEDEMA

A ten-year old girl presented with unilateral swelling of right half of her body since birth. She developed itchy, oozing, painful skin lesions over right lower leg for the past three months. Clinical examination and investigations revealed unilateral lymphoedema with elephantiasis nostras verrucosa cutis and visceral involvement in the form of pericardial effusion, ascites and intestinal lymphangiectasia. She was diagnosed as a case of multisegmental lymphatic dysplasia with systemic involvement. We report this rare case of primary lymphoedema, highlighting the approach to a case of primary lymphedema.