Clove syndromeQ85.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

CLOVES syndrome; Congenital lipomatous overgrowth - vascular malformation - epidermal naevi - skeletal abnormalities; Congenital lipomatous overgrowth - vascular malformation - epidermal nevi; Hemihyperplasia (multiple) lipomatosis syndrome; OMIM: 612918

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HistoryThis section has been translated automatically.

Friedberg Hermann 1867

DefinitionThis section has been translated automatically.

CLOVE is the acronym for a segmental overgrowth syndrome, "PIK3CA-related overgrowth spectrum" (PROS), and is characterized by the following symptoms: congenital lipomatous overgrowth, vascular malformations, epidermal nevus, skeletal anomalies.

EtiopathogenesisThis section has been translated automatically.

This is caused by germline mutations in the mosaic in the PIKA3Ca gene located on chromsome 3q26.32. The gene codes for the PIK3-alpha protein.

Clinical featuresThis section has been translated automatically.

Particularly typical is a trunk-stressed port wine nevus gfls. combined with venous vascular malformations

Congenital circumscribed large growth especially of the feet, which increases in the course of life. Typical is the "sandal gap".

Lipomatosis, lipomas mainly on the trunk.

Bone changes, especially scoliosis.

Epidermal nevus

Possible are asymmetry of the skull, epilepsy, hemimegalencephaly, lack of beams

LiteratureThis section has been translated automatically.

  1. Eissing M et al (2019) PTEN Hamartoma Tumor Syndrome and Immune Dysregulation. Translated Oncol 12:361-367.
  2. Pilarski R et al (2019) PTEN Hamartoma Tumor Syndrome: A Clinical Overview. Cancers (Basel)11:6

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer