A rare association of B cell lymphoma and ectodermal dysplasia presenting with protein-losing enteropathy

Summary

This patient, known to have ectodermal dysplasia, was referred to the hospital to investigate the cause for his significant pedal oedema and hypoproteinaemia. Investigations ruled out protein loss from the kidney and there was no evidence of chronic liver disease. Protein-losing enteropathy became a diagnosis of exclusion. To investigate it further, he underwent an oral gastroduodenoscopy and a computed tomography scan of the abdomen, which showed an abnormal duodenal mucosa and extensive retroperitoneal lymphadenopathy. Biopsies confirmed this to be grade II follicular non-Hodgkin lymphoma. The lymphadenopathy was causing obstructive hydronephrosis, which required a nephrostomy. He received a course of steroids and chemotherapy. His condition, however, deteriorated and he died.