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]


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


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]


Kabuki syndrome[37]

Ichthyosis with hypogonadism (Rud’s syndrome)[38]

Primary biliary cirrhosis[39]