Do you know this syndrome?

Abstract:

:The Bourneville-Pringle syndrome is an autosomal dominant neurocutaneous disorder characterized by the development of multiple hamartomas in various systems, especially brain, skin, retina, kidney, heart and lung. The case of a patient with brownish plaques on the forehead and temporal region, pink malar and chin papules, and hypopigmented macules on the back and trunk is described. The diagnosis of the Bourneville-Pringle syndrome is based on clinical criteria. Presence of two major criteria, such as facial angiofibromas, forehead fibrous plaques, three or more hypomelanotic macules establish the definitive diagnosis. The diagnosis should be made as early as possible in order to assess and treat the associated complications.

journal_name

An Bras Dermatol

authors

Gontijo GM

doi

10.1590/S0365-05962013000200027

subject

Has Abstract

pub_date

2013-03-01 00:00:00

pages

303-5

issue

2

eissn

0365-0596

issn

1806-4841

pii

S0365-05962013000200303

journal_volume

88

pub_type

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