Telangiectasia nevoide unilateral adquirida em homem hígido

Abstract

Unilateral nevoid telangiectasia is a rare vascular dermatosis, characterized by areas of superficial telangiectases distributed in a linear unilateral pattern. It was described in 1899 by Zeisler and Blascko. Several theories where developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in the estrogen levels. There are two types: congenital and acquired. The latter is associated with hepatopathies in male patients. The acquired form is rarely observed in healthy men, with a few cases reported in the medical literature, and its etiology is unknown. This study reports the case of a healthy young man with acquired unilateral nevoid telangiectasia, without any comorbidities, clinical and /or laboratory findings indicative of hyperestrogenism. Keywords: Estrogens; Liver Diseases; Telangiectasis Resumo: A Telangiectasia Nevoide Unilateral é uma dermatose vascular rara, caracterizada por áreas de telangiectasia superficial, em uma distribuição linear unilateral, descrita, em 1899, por Zeisler e Blaschko. Diversas teorias foram desenvolvidas para explicar sua etiopatogenia, sendo a provável (e a mais fundamentada) a relação com elevação dos níveis de estrogênio. Há duas formas: a congênita e a adquirida. Esta, geralmente, se relaciona a hepatopatias, em pacientes do sexo masculino. Em homens hígidos, a ocorrência da forma adquirida é rara, com poucos relatos na literatura, sendo sua causa desconhecida. Este trabalho tem como objetivo relatar um caso de telangiectasia nevoide unilateral adquirida em homem jovem, sem comorbidades, alterações clínicas e/ou laboratoriais sugestivas de hiperestrogenismo. Palavras-chave: Estrogênios; Hepatopatias; Telangiectasia INTRODUÇÃOUnilateral nevoid telangiectasia is a rare vascular dermatosis, characterized by areas of superficial telangiectases distributed in a linear unilateral pattern, described in 1899 by Zeisler e Blaschko.1 Since then, less than a 100 cases have been described in the medical literature 2 . Several theories were developed in order to explain its etiopathogenesis. The most widely accepted is the one which establishes its probable association with an increase in the estrogen levels. There are two types: congenital and acquired. The first is prevalent in men through dominant autosomal inheritance and the second in women, in association with physiological and pathological conditions of hyperestrogenism. 1The acquired type, in male patients, is generally related to hepatopathies . The occurrence of this type among healthy men is rare and its cause remains unknown.

Case for diagnosis

“…3 There is controversy in regard to the existence of a somatic mutation during embryogenesis in the congenital form or, in the acquired form, the elevation of estrogen levels or vasoactive substances that may, in theory, be revealing of a state of hidden mosaicism. 4-6 Only one publication to date has identified the elevation of estrogen receptors in lesional skin. 7 Others have proposed the role of a still unidentified vasoactive substance.…”

Unilateral nevoid telangiectasia, diagnostic and therapeutic challenge

“…The Unilateral Nevoid Telangiectasia (UNT) was described by Zeisler and Blaschko in 1899, but its current denomination was defined by Selmanowitz in 1970. [1][2][3] The term nevóide refers to the similarity with other congenital cutaneous lesions blaschkoide and does not indicate a specific morphology of telangiectasia. 4 It is a rare vascular dermatitis defined by areas of superficial telangiectasia with unilateral distribution, arborescent, non-confluent and without central vessel.…”

“…4 It is a rare vascular dermatitis defined by areas of superficial telangiectasia with unilateral distribution, arborescent, non-confluent and without central vessel. 1,2,5 The most accepted etiopathogenesis is associated with high levels of estrogen. 1 This pathology is classified as congenital and acquired, being the first one more prevalent in men, by autosomal dominant inheritance occurring during or after the neonatal period.…”

“…1,2,5 The most accepted etiopathogenesis is associated with high levels of estrogen. 1 This pathology is classified as congenital and acquired, being the first one more prevalent in men, by autosomal dominant inheritance occurring during or after the neonatal period. The second one has a greater predilection for women during their fertile age and is linked to hyperestrogenism or elevation of vasoactive substances.…”

“…The second one has a greater predilection for women during their fertile age and is linked to hyperestrogenism or elevation of vasoactive substances. 1,2,6…”