Generalized Essential Telangiectasia

Updated: Oct 09, 2015
  • Author: David Green, MD, PA; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

A telangiectasis refers to a visibly dilated blood vessel on the skin or mucosal surface. Telangiectases that develop in the absence of any preceding or coexisting cutaneous or systemic disease are considered to be primary or essential. Telangiectases resulting from or in association with a known disease state are classified as secondary.

Different presentations of primary telangiectases have been arbitrarily classified as distinct syndromes, designated by terms that often are descriptive based on inheritance, age of onset, anatomic distribution, morphology, prognosis, or associated findings. No recognized nomenclature exists for these telangiectatic disorders. Generalized essential telangiectasia refers to one syndrome of acquired primary telangiectases that are so termed because of their widespread anatomic distribution. [1]  See the image below.

Essential generalized telangiectasia. Courtesy of Essential generalized telangiectasia. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/vascular/ess-telang1.jpg).
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Pathophysiology

The pathophysiologic factors causing blood vessel dilatation in generalized essential telangiectasia are yet to be elaborated. Familial cases have been reported with an autosomal dominant pattern of inheritance.

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Epidemiology

Frequency

Neither the incidence nor prevalence of generalized essential telangiectasia is known.

Race

Generalized essential telangiectasia has been reported more commonly in whites, perhaps because of the marked contrast of the vessels on light-complexioned skin.

Sex

Women are affected more commonly than are men, and in one published study of 13 people with generalized essential telangiectasia, 10 of the reported patients were women.

Age

In one report of 13 patients, the average age of onset for generalized essential telangiectasia was 38 years. [2]

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Prognosis

The development of telangiectases may be gradual or rapid. Usually, telangiectases tend to progress to other sites. Lesions persist indefinitely and do not regress spontaneously. General health is not affected in patients with telangiectases. No cutaneous changes or internal diseases are associated with generalized essential telangiectasia.

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