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.
Acanthosis nigricans in hereditary syndromesL83
DefinitionThis section has been translated automatically.
ClassificationThis section has been translated automatically.
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).
EtiopathogenesisThis section has been translated automatically.
Hereditary factors that lead to increased levels of growth factors that activate keratinocytes and fibroblasts at the cellular level S.a.u. Acanthosis nigricans.
ManifestationThis section has been translated automatically.
Mostly occurring between birth and puberty.
Clinical featuresThis section has been translated automatically.
Acanthosis nigricans, mostly weak expression, rarely mucosal infestation.
HistologyThis section has been translated automatically.
S.u. Acanthosis nigricans.
TherapyThis section has been translated automatically.
Symptomatically in cooperation with endocrinologists (often acanthosis nigricans runs parallel to the systemic disease), see Acanthosis nigricans.
LiteratureThis section has been translated automatically.
- 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
- Jabbour SA (2003) Cutaneous manifestations of endocrine disorders: a guide for dermatologists. At J Clin Dermatol 4: 315-331
- Schroeder B, Ding X, Pfaff-Amesse T (2002) From HAIR-AN to eternity. J Pediatr Adolesc Gynecol 15: 235-240
- Schwartz RA (1994) Acanthosis nigricans. J Am Acad Dermatol 31: 1-22
- Torley D, Bellus GA, Munro CS (2002) Genes, growth factors and acanthosis nigricans. Br J Dermatol 147: 1096-1101
- Groll A, Hirschowitz BI (1966) Steatorrhea and familial deafness in two siblings. Clin Res 14: 47