Chorioretinal lesions, sea-blue histiocytes and other manifestations in familial chronic granulomatous disease.
Several little-emphasized manifestations of familial chronic granulomatous disease are considered: destructive chorioretinal lesions may be as constant as the pigmented histiocytosis seen in reticuloendothelial organs and could be related to a defect in the phagocytic activity of the retinal pigment epithelium; pigmented histiocytes with the staining characteristics of sea-blue histiocytes may be present in the bone marrow; patients may present with lesions resembling eosinophilic granuloma. Also discussed are some observations related to the sequestration of bacteria within phagocytic cells and the use of continuous antimicrobial therapy.
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D002829:Choroid; D003023:Cloxacillin / Q000627:therapeutic use; D003951:Diagnostic Errors; D004386:Duodenum / Q000000981:diagnostic imaging; D004803:Eosinophilic Granuloma / Q000175:diagnosis; D005128:Eye Diseases / Q000209:etiology*; D006105:Granulomatous Disease, Chronic / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D006811:Humerus / Q000000981:diagnostic imaging; D008206:Lymphatic Diseases / Q000209:etiology; D008297:Male; D010019:Osteomyelitis / Q000209:etiology; D010406:Penicillins / Q000627:therapeutic use; D010585:Phagocyte Bactericidal Dysfunction / Q000150:complications*; D010859:Pigmentation Disorders; D011859:Radiography; D012164:Retinal Diseases / Q000209:etiology; D012886:Skull / Q000000981:diagnostic imaging; D013270:Stomach / Q000000981:diagnostic imaging; D014747:Vinblastine / Q000627:therapeutic use
D000305:Adrenal Cortex Hormones; D010406:Penicillins; D014747:Vinblastine; D003023:Cloxacillin