[Infantile segmental hemangioma without facial involvement: A cutaneous marker of vascular malformations such as in PHACE syndrome?]

Ann Dermatol Venereol. 2015 Oct;142(10):563-6. doi: 10.1016/j.annder.2015.04.164. Epub 2015 Jun 9.
[Article in French]

Abstract

Background: Herein we report a case of a possible PHACE syndrome without hemangioma of the head but with a large segmental hemangioma of the trunk.

Patients and methods: A 17-year-old female patient with a medical history of transposition of the great arteries with ventricular septal defect diagnosed at 3 days of life and of coarctation of the aorta diagnosed at 14 years was seen in the dermatology department for a long-standing large rectangular, segmental, atrophic and telangiectasic lesion on her back. The lesion appeared to be a sequel of infantile segmental hemangioma of the trunk, and this was confirmed by history-taking.

Discussion: This case raises the question of a link between infantile segmental hemangioma and underlying cardiovascular disorders. Infantile segmental hemangioma could be a marker of an underlying vascular development defect. The presence of infantile segmental hemangioma, regardless of site, should prompt vascular explorations.

Keywords: Hémangiome segmentaire; PHACE; Segmental hemangioma; Tronc; Trunk.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Abnormalities, Multiple / etiology*
  • Adolescent
  • Aortic Coarctation / diagnosis
  • Back
  • Diagnosis, Differential
  • Eye Abnormalities / diagnosis
  • Female
  • Hemangioma, Capillary / etiology*
  • Humans
  • Neurocutaneous Syndromes / diagnosis
  • Skin Neoplasms / etiology*
  • Syndrome
  • Telangiectasis / etiology
  • Transposition of Great Vessels / etiology*
  • Transposition of Great Vessels / pathology

Supplementary concepts

  • PHACE association