Malakoplakia of the colon in an infant with severe combined immunodeficiency (SCID) and CHARGE association

We report on malakoplakia of the colon observed in a six month old girl in a setting of severe combined immunodeficiency (SCID) and a malformational s yndrome termed CHARGE association. By the age of six months, hemorrhagic di arrhea had developed, and multiple ulcers were seen at colonoscopy. The bio psy specimen showed ulcerating malakoplakia. Immunodeficiency was primarily reflected by deprivation of CD4 cells in the peripheral blood, and CT scan s failed to detect structures consistent with a normal thymus. There were a lso polylymphadenopathy and chronic erythroderma. The lymph node showed ext reme hypoplasia of the follicular cortex and marked expansion of the paraco rtex. B cell counts progressively declined, and plasma cells were absent bo th in intact mucosa of the colon and in a lymph node. The patient died at e ighteen months of respiratory failure following recurrent airway infections . Pediatric malakoplakia of the colon, though rare, may be regarded as an e xample of opportunistic bacterial infection in an immunocompromised host. C ombined immunodeficiency (CID) has to be considered in such instances, in p articular when malformational syndromes coexist affecting the development o f the thymus.