eMedicine Specialties > Dermatology > Diseases of the Vessels

Generalized Essential Telangiectasia

Author: David Green, MD, Clinical Assistant Professor, Department of Dermatology, Howard University Hospital
Contributor Information and Disclosures

Updated: Oct 30, 2009

Introduction

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

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.

Frequency

United States

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

Mortality/Morbidity

General health is not affected in patients with telangiectases. No cutaneous changes or internal diseases are associated with generalized essential telangiectasia.

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

Clinical

History

  • The most commonly observed initial clinical presentation of generalized essential telangiectasia is telangiectasia on the feet, ankles, and distal legs. Subsequently, telangiectases appear more proximally on the lower extremities, and they also may develop on the upper extremities and trunk. In a report of 13 patients, 12 had involvement of the lower extremities. Occasionally, telangiectases first become apparent on the upper extremities or trunk.2
  • Bleeding from the ectatic vessels is rare.
  • Usually, no family history exists of a similar disorder; however, some familial cases have been reported.
  • Generalized essential telangiectasia is usually asymptomatic, but tingling burning or numbness is occasionally reported.
  • The age of onset is usually in the fourth or fifth decade, but symptoms may be observed in younger adults.
  • The progressive development of the telangiectases, without spontaneous regression, is the usual course.

Physical

  • In generalized essential telangiectasia, dilated blood vessels represent capillary telangiectases (not venous), ie, they appear red or pink and are usually less than 0.2 mm in diameter, unlike venous telangiectases, which usually appear more blue in color and are greater than 0.2 mm in diameter.
  • Most often, generalized essential telangiectasia presents as numerous discrete pink and red capillaries, appearing punctate, linear, or as a lacework or syncytial network. Occasionally, discrete, well-circumscribed, red macules lend the skin a speckled appearance.
  • Capillaries are usually bilateral and are symmetrically distributed on the skin. When they are numerous enough to become confluent, the skin appears diffusely erythematous, and discerning individual telangiectases becomes impossible.
  • Telangiectases rarely protrude above the normal plane of the skin. Pressure on the skin readily displaces the blood, causing blanching; however, rapid refilling occurs.
  • On infrared photography, the underlying venous vasculature appears normal.
  • Even in the presence of significant cutaneous involvement, mucous membranes and conjunctivae are not usually affected, although extracutaneous involvement of the oral mucosa and conjunctiva has been reported.3,4,5,6,7 No changes occur within the epidermis or dermis.

Causes

  • 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.8,9
  • 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.10

More on Generalized Essential Telangiectasia

Overview: Generalized Essential Telangiectasia
Differential Diagnoses & Workup: Generalized Essential Telangiectasia
Treatment & Medication: Generalized Essential Telangiectasia
Follow-up: Generalized Essential Telangiectasia
References
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References

  1. Rothe MJ, Grant-Kels JM. Nomenclature of the primary telangiectasias. Int J Dermatol. May 1992;31(5):320. [Medline].

  2. McGrae JD, Winkelmann RK. Generalized essential telangiectasia: Report of a clinical and histochemical study of 13 patients with acquired cutaneous lesions. JAMA. Sep 21 1963;185:909-13. [Medline].

  3. Ali MM, Teimory M, Sarhan M. Generalized essential telangiectasia with conjunctival involvement. Clin Exp Dermatol. Nov 2006;31(6):781-2. [Medline].

  4. Gantenbein C, Seiter S, Weik R, Reinhold U, Ruprecht KW, Tilgen W. [Ocular manifestation of progressive essential telangiectases]. J Fr Ophtalmol. Jan 2001;24(1):45-8. [Medline].

  5. Mannis MJ, Bindi CS, Huntley AC. Ocular manifestations of generalized essential telangiectasia. Cornea. Nov 1999;18(6):731-3. [Medline].

  6. Seiter S, Gantenbein C, Ugurel S, Ruprecht KW, Tilgen W, Reinhold U. An oculocutaneous presentation of essential progressive telangiectasia. Br J Dermatol. May 1999;140(5):969-71. [Medline].

  7. Swensson B, Swensson O, Häring G. [Progressive disseminated essential telangiectasia with conjunctival involvement]. Klin Monatsbl Augenheilkd. Feb 1998;212(2):116-9. [Medline].

  8. Person JR, Longcope C. Estrogen and progesterone receptors are not increased in generalized essential telangiectasia. Arch Dermatol. Jul 1985;121(7):836-7. [Medline].

  9. Sadick NS, Niedt GW. A study of estrogen and progesterone receptors in spider telangiectasias of the lower extremities. J Dermatol Surg Oncol. Jul 1990;16(7):620-3. [Medline].

  10. Thieu KP, Haynes HA. Generalized essential telangiectasia with predilection for surgical scar. J Am Acad Dermatol. Apr 2009;60(4):710-1. [Medline].

  11. Wells RS, Dowling GB. Hereditary benign telangiectasia. Br J Dermatol. Jan 1971;84(1):93-4. [Medline].

  12. Checketts SR, Burton PS, Bjorkman DJ, Kadunce DP. Generalized essential telangiectasia in the presence of gastrointestinal bleeding. J Am Acad Dermatol. Aug 1997;37(2 Pt 2):321-5. [Medline].

  13. Braverman IM. Ultrastructure and organization of the cutaneous microvasculature in normal and pathologic states. J Invest Dermatol. Aug 1989;93(2 Suppl):2S-9S. [Medline].

  14. Wenzel SM, Hohenleutner U, Landthaler M. Progressive disseminated essential telangiectasia and erythrosis interfollicularis colli as examples for successful treatment with a high-intensity flashlamp. Dermatology. 2008;217(3):286-90. [Medline].

  15. Green D. Sclerotherapy treatment insights. Dermatol Clin. Jan 1998;16(1):195-211. [Medline].

  16. Shelley WB. Essential progressive telangiectasia. Successful treatment with tetracycline. JAMA. May 24 1971;216(8):1343-4. [Medline].

  17. Shelley WB, Shelley ED. Essential progressive telangiectasia in an autoimmune setting: successful treatment with acyclovir. J Am Acad Dermatol. Nov 1989;21(5 Pt 2):1094-6. [Medline].

  18. Shelley WB, Fierer JA. Focal intravascular coagulation in progressive ascending telangiectasia: ultrastructural studies of ketoconazole-induced involution of vessels. J Am Acad Dermatol. May 1984;10(5 Pt 2):876-87. [Medline].

  19. Buscaglia DA, Conte ET. Successful treatment of generalized essential telangiectasia with the 585-nm flashlamp-pumped pulsed dye laser. Cutis. Feb 2001;67(2):107-8. [Medline].

  20. Gambichler T, Avermaete A, Wilmert M, Altmeyer P, Hoffmann K. Generalized essential telangiectasia successfully treated with high-energy, long-pulse, frequency-doubled Nd:YAG laser. Dermatol Surg. Apr 2001;27(4):355-7. [Medline].

  21. Blume JE. Generalized essential telangiectasia: a case report and review of the literature. Cutis. Apr 2005;75(4):223-4. [Medline].

  22. Abrahamian LM, Rothe MJ, Grant-Kels JM. Primary telangiectasia of childhood. Int J Dermatol. May 1992;31(5):307-13. [Medline].

  23. Champion RH. Disorders affecting small blood vessels. In: Rook/Wilkinson/Ebling Textbook of Dermatology. Blackwell Scientific; 1986:1081.

  24. Gentele H, Lodin A. Telangiectasia essentialis generalisata of unknown origin. Acta Derm Venereol. 1957;37(6):465-70. [Medline].

  25. Kint A, Geerts ML, Platevoet D. Generalized essential telangiectasia. Arch Belg Dermatol Syphiligr. Oct-Dec 1972;28(4):377-85. [Medline].

  26. Long D, Marshman G. Generalized essential telangiectasia. Australas J Dermatol. Feb 2004;45(1):67-9. [Medline].

  27. Olsen T. Peripheral vascular diseases, necrotizing vasculitis, and vascular-related diseases. In: Moschella SL, Hurley HJ, eds. Dermatology. Philadelphia: WB Saunders Co; 1985:1049.

  28. Reed WB, Epstein WL, Boder E, Sedgwick R. Cutaneous manifestations of ataxia-telangiectasia. JAMA. Feb 28 1966;195(9):746-53. [Medline].

  29. Taber CW. Taber's Cyclopedic Medical Dictionary. Philadelphia: FA Davis Co; 1997:T-11.

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Further Reading

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Keywords

generalized essential telangiectasia, telangiectasis, GET, red telangiectasis, capillary telangiectasis, blue telangiectasis, venous telangiectasis, telangiectases

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Contributor Information and Disclosures

Author

David Green, MD, Clinical Assistant Professor, Department of Dermatology, Howard University Hospital
David Green, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Facial Plastic and Reconstructive Surgery, American College of Phlebology, American Medical Association, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

Medical Editor

Gregory J Raugi, MD, PhD, Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle
Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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