Primary pigmented micronodular disease of the adrenals.
Primary pigmented micronodular disease is a peculiar form of ACTH-independent Cushing's syndrome characterized by the familial occurrence, the frequent association with malformations and the pathological adrenocortical picture consisting in micronodules with cellular deposition of lipofuscinic pigment. We describe here a case occurring in a 14-year-old girl.
Limone
P
P
Istituto di Medicina Interna, Università di Torino, Italy.
Maccario
M
M
Vigliani
R
R
Isaia
G
G
Massara
F
F
Molinatti
G M
GM
D000273:Adipose Tissue / Q000473:pathology; D000293:Adolescent; D000302:Adrenal Cortex / Q000473:pathology*; D000313:Adrenal Medulla / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000097:blood; D003480:Cushing Syndrome / Q000378:metabolism / Q000473:pathology*; D003907:Dexamethasone; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D006914:Hydroxysteroids / Q000652:urine; D008062:Lipofuscin / Q000032:analysis*; D008214:Lymphocytes / Q000473:pathology; D008797:Metyrapone; D010860:Pigments, Biological / Q000032:analysis*
D006914:Hydroxysteroids; D008062:Lipofuscin; D010860:Pigments, Biological; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone