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Angiokeratoma of Mibelli in Child
Other Resources UpToDate PubMed

Angiokeratoma of Mibelli in Child

Contributors: Rajini Murthy MD, Chris G. Adigun MD, David Dasher MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Angiokeratoma of Mibelli (AKM), also known as the angiokeratoma of Mibelli of adolescence, is a rare, acquired, localized form of angiokeratoma that typically occurs on acral sites. Females between the ages of 10 and 15 years are most commonly affected. Patients frequently complain of lesions bleeding easily with mild trauma.

There may be an association with perniosis and acrocyanosis. It is theorized that these cyanotic attacks have deleterious effects on vessel walls that lead to subsequent development of vascular ectasia. This etiological theory, though, has been debated. Associated systemic vascular involvement has not been established. However, necrosis of the fingertips in AKM has been described.

Although AKM is considered to be an acquired disease, genetic predisposition is likely, and familial studies have revealed higher incidences among siblings than expected.

Codes

ICD10CM:
I78.8 – Other diseases of capillaries

SNOMEDCT:
62727008 – Angiokeratoma of Mibelli

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Infantile hemangioma
  • Lobular capillary hemangioma (pyogenic granuloma)
  • Angioma serpiginosum
  • Glomangiomas
  • Lymphangioma circumscriptum
  • Non-AIDS Kaposi sarcoma
  • Common acquired nevus
  • Spitz nevus
  • Melanoma
  • Fabry disease (Fabry disease)
  • Fucosidosis

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      Therapy

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      References

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      Last Reviewed:07/04/2022
      Last Updated:07/05/2022
      Copyright © 2024 VisualDx®. All rights reserved.
      Angiokeratoma of Mibelli in Child
      A medical illustration showing key findings of Angiokeratoma of Mibelli : Acral distribution, Hemorrhagic papule
      Clinical image of Angiokeratoma of Mibelli - imageId=73068. Click to open in gallery.  caption: 'Tiny maroon macules and papules on the fingers.'
      Tiny maroon macules and papules on the fingers.
      Copyright © 2024 VisualDx®. All rights reserved.