Phacomatosis pigmentokeratoticaQ84.2

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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HistoryThis section has been translated automatically.

Happle al. 1996

DefinitionThis section has been translated automatically.

Sporadically occurring, binary genodermatosis (Karia DR et al. 2018), which is characterized by the joint occurrence of a nevus spilus (papulosus) with a nevus sebaceus(mosaic dermatosis). A malignant degeneration of both hamartomas has been described (Martínez-Menchón T et al. 2005).

EtiopathogenesisThis section has been translated automatically.

Actuating mutations in the KRAS or HRAS genes can be detected (Ohm A eet al. 2017). S.a. uner Rasopathies.

Note(s)This section has been translated automatically.

Today, this genodermatosis is considered a variant of the Schimmelpennig syndrome. The associated organ changes correspond to those of the Schimmelpenning-Feuerstein-Mims Syndrome.

LiteratureThis section has been translated automatically.

  1. Karia DR et al (2018) Phacomatosis pigmentokeratotica: A very rare twin spotting phenomenon. Indian J Dermatol Venereol Leprol 84:120.
  2. Martínez-Menchón T et al (2005) Phacomatosis pigmentokeratotica: a 20-year follow-up with malignant degeneration of both nevus components. Pediatric dermatol 22:44-47.
  3. Om A et al (2017)Phacomatosis Pigmentokeratotica: A Mosaic RASopathy with Malignant Potential. Pediatric Dermatol 34:352-355.

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer