Generalized Essential Telangiectasia

Updated: Dec 15, 2017
  • 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, 2] 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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Etiology

Etiology and pathogenesis of generalized essential telangiectasia remain unknown.

Naturally occurring substances, such as estrogen, serotonin, or adrenal corticosteroids, have no known influence on the development or progression of generalized essential telangiectasia. [4, 5]

No reported association with varicose vein disease or other superficial or underlying deep venous insufficiency has been reported.

Localized absence of telangiectases under a wristwatch has been reported. This suggests that sun exposure or mechanical factors may influence the development of telangiectases, as is observed with ataxia-telangiectasia (Louis-Bar syndrome).

A patient reported with generalized essential telangiectasia, having multiple symmetrically distributed telangiectases on upper and lower extremities, had telangiectases covering an appendectomy scar; however, other scars associated with multiple unrelated surgical procedures had none. [6]

Another study posits a connection between cutaneous collagenous vasculopathy and generalized essential telangiectasia, but the authors emphasize the importance of clinicopathological correlation in order for a diagnosis to be made. [7]

One patient exhibited symptoms that might indicate an association between generalized essential telangiectasia, corneal neovascularization, and thoracic aneurysm formation. [8]

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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. [3]

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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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