Generalized essential telangiectasia in Child
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Synopsis

Generalized essential telangiectasia (GET) is a benign condition in which there are progressive, widespread, asymptomatic, blanchable telangiectasias. This condition primarily affects adult women, and the average age of onset is late 30's to late 40's, but pediatric cases have been reported. The limbs, especially distal extremities, are usually initially affected with gradual, proximal progression to more widespread involvement. Conjunctival involvement has also rarely been reported.
Most commonly, telangiectasias start distally and spread proximally in a symmetric pattern. The lesions can also be distributed in localized, diffuse, discrete, or confluent patterns. The affected areas are usually asymptomatic, but there can be associated tingling, burning, or numbness.
Generally, there are no associated systemic symptoms or bleeding; however, there have been reported cases of gastrointestinal bleeding secondary to gastric antral vascular ectasias.
Most commonly, telangiectasias start distally and spread proximally in a symmetric pattern. The lesions can also be distributed in localized, diffuse, discrete, or confluent patterns. The affected areas are usually asymptomatic, but there can be associated tingling, burning, or numbness.
Generally, there are no associated systemic symptoms or bleeding; however, there have been reported cases of gastrointestinal bleeding secondary to gastric antral vascular ectasias.
Codes
ICD10CM:
I78.1 – Nevus, non-neoplastic
SNOMEDCT:
238763007 – Generalized essential telangiectasia
I78.1 – Nevus, non-neoplastic
SNOMEDCT:
238763007 – Generalized essential telangiectasia
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome)
- Hereditary benign telangiectasia – usually inherited in autosomal dominant fashion and often presenting in childhood
- Cutaneous collagenous vasculopathy – distinguished from GET histologically with thickened hyaline material in vessel walls
- Ataxia-telangiectasia syndrome – onset usually in first or second decade
- Telangiectasia macularis eruptiva perstans – rare form of mastocytosis
- Telangiectasias secondary to liver disease
- Systemic sclerosis
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Therapy
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References
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Last Reviewed:11/17/2020
Last Updated:11/22/2020
Last Updated:11/22/2020

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