Acanthosis nigricans in hereditary syndromes L83

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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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.

Classification
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Syndromes associated with Acanthosis nigricans include:

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Etiopathogenesis
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Hereditary factors that lead to increased levels of growth factors that activate keratinocytes and fibroblasts at the cellular level S.a.u. Acanthosis nigricans.

Manifestation
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Mostly occurring between birth and puberty.

Clinical features
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Acanthosis nigricans, mostly weak expression, rarely mucosal infestation.

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

Literature
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  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
  2. Jabbour SA (2003) Cutaneous manifestations of endocrine disorders: a guide for dermatologists. At J Clin Dermatol 4: 315-331
  3. Schroeder B, Ding X, Pfaff-Amesse T (2002) From HAIR-AN to eternity. J Pediatr Adolesc Gynecol 15: 235-240
  4. Schwartz RA (1994) Acanthosis nigricans. J Am Acad Dermatol 31: 1-22
  5. Torley D, Bellus GA, Munro CS (2002) Genes, growth factors and acanthosis nigricans. Br J Dermatol 147: 1096-1101
  6. Groll A, Hirschowitz BI (1966) Steatorrhea and familial deafness in two siblings. Clin Res 14: 47

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Last updated on: 29.10.2020