Table 1. Syndromes associated with acanthosis nigricans[1, 4]
Insulin Resistance Syndromes |
Acral hypertrophy and muscle cramps[11] |
Acromegaly[12, 13] |
Alström syndrome[14] |
Ataxia-telangiectasia (Louis-Bar syndrome)[15] |
Cushing syndrome[16] |
Diabetes Mellitus Type 2[2, 7, 17] |
Gonadal dysfunction[7] |
Ovarian hyperthecosis[18] |
Polycystic ovary syndrome (PCOS, Stein-Leventhal syndrome)[7] |
Hyperplasia of the adrenal cortex, diabetes mellitus, and hypertrophy of the pineal body (Rabson-Mendenhall syndrome)[19] |
Leprechaunism[20] |
Lipodystrophy[21, 22] |
Congenital lipodystrophic diabetes with acanthosis nigricans (Lawrence-Seip syndrome) |
Congenital generalized lipodystrophy (Seip-Berardinelli syndrome) |
Familial partial lipodystrophy (Kobberling-Dunnigan syndrome) |
Mental retardation, overgrowth, remarkable faces, and acanthosis nigricans (MORFAN)[23] |
Prader-Willi syndrome[24] |
Type A syndrome (Hyperandrogenism, insulin resistance, and AN syndrome, or HAIR-AN)[9] |
Type B syndrome[8] |
Hashimoto’s thyroiditis |
Scleroderma |
Sjögren’s syndrome |
Systemic lupus erythematosus[25] |
Type C syndrome |
Fibroblast Growth Factor Receptor Defect Syndromes |
Beare-Stevenson cutis gyrata syndrome[26] |
Crouzon’s syndrome with acanthosis nigricans[27] |
Severe achondroplasia with developmental delay and acanthosis nigricans (SADDAN)[28] |
Thanatophoric dysplasia[29, 30] |
Other Associations |
Benign encephalopathy[31] |
Congenital adrenal hyperplasia[32] |
Costello syndrome[33, 34] |
Hepatolenticular degeneration (Wilson’s disease)[35] |
Hirschowitz syndrome[1] |
Hypothyroidism[36] |
Kabuki syndrome[37] |
Ichthyosis with hypogonadism (Rud’s syndrome)[38] |
Primary biliary cirrhosis[39] |