Acanthosis nigricans in hereditary syndromes L83

Acanthosis nigricans is a partial symptom of numerous, mostly autosomal recessive inherited symptom complexes. Most of them are associated with endocrinological disorders, especially diabetes mellitus. Insulin resistance is therefore discussed as a pathogenetic factor for acanthosis nigricans.

Syndromes associated with Acanthosis nigricans include:

• Bloom Syndrome
• Wilson's disease
• Crouzon Syndrome
• Miescher Syndrome
• Prader-Willi syndrome
• HAIR-AN syndrome
• Lipodystrophia generalisata acquisita (see also Seip-Lawrence syndrome)
• Groll-Hirschowitz Syndrome (deafness, gastrointestinal and neurological abnormalities, etc. combined with acanthosis nigricans)
• SADDAN syndrome (achondroplasia, thanatophoric dysplasia, combined with acanthosis nigricans, among others)
• Leprechaunism syndrome (genetic defect of the β cells of the pancreas)
• Rabson-Mendenhall syndrome (genetic defect of insulin action)
• Insulin-resistant diabetes mellitus with acanthosis nigricans
• Insulin-resistant diabetes mellitus with acanthosis nigricans and hypertension
• Familial partial lipodystrophy
• Alström syndrome (retinal degeneration combined with, obesity, diabetes mellitus, neurogenic deafness, acanthosis nigricans and others).

Symptomatically in cooperation with endocrinologists (often acanthosis nigricans runs parallel to the systemic disease), see Acanthosis nigricans.

1. Alström CH et al (1959) Retinal degeneration combined with obesity, diabetes mellitus and neurogenous deafness. A specific syndrome (not hitherto described) distinct from Laurence-Moon-Biedl syndrome. A clinical endocrinological and genetic examination based on a large pedigree. Acta Psychiatrist Neurol Scand 34 (Suppl 129): 1-35
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