Une nouvelle localisation de l'angiokératome circonscrit næviforme

Translated title of the contribution: A new location of angiokeratoma circumscriptum

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Angiokeratomas are papular telangiectasias having a common histology of ectasia of the superficial dermal vessels surmounted by a hyperkeratotic epidermis. Patients and methods: The patient was a 9-year-old girl born of non-consanguineous parents after a well-followed pregnancy with problem-free delivery at term. From birth, she had a tumefaction of the left side of the nose and the left half of the upper lip that gradually increased in size without obstructing the nasal orifice and bled easily. Examination revealed the presence of tumefaction of the left nostril and the left half of the upper lip projecting towards the contralateral side especially in the nose. It was soft and painless, with the presence at the surface of dull red keratotic papules of 1 to 2 mm in diameter. Examination of the nasal mucosa revealed the same appearance of papules. Discussion: Angiokeratoma circumscriptum is a rare congenital malformation, the rarest of five types. Since its initial description in 1890, few cases have been reported. However, female predominance has been noted with a male/female sex ratio of 1/3. It appears to be due to a genetic mutation that is probably autosomal, but the site of which is still unknown. In view of the special features of this case, several diagnoses were suggested, including Rendu Osler's disease, superficial lymphangioma and verrucous angioma. Conclusion: The particularity of this case is that it includes the first description of this site, which posed a therapeutic problem, especially concerning the choice of laser type to be used.

Translated title of the contributionA new location of angiokeratoma circumscriptum
Original languageFrench
Pages (from-to)732-735
Number of pages4
JournalAnnales de Dermatologie et de Venereologie
Volume139
Issue number11
DOIs
Publication statusPublished - Nov 2012

Keywords

  • Angiokeratoma
  • Hemangioma
  • Naeviform
  • Vascular malformation

ASJC Scopus subject areas

  • Dermatology

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