Generalized Essential Telangiectasia 

Updated: Dec 15, 2017
Author: David Green, MD, PA; Chief Editor: Dirk M Elston, MD 

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).

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.

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]

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]

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.

 

Presentation

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.[3]

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 Examination

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, as shown in 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).

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.[9, 10, 11, 12, 13] No changes occur within the epidermis or dermis.

Complications

No physical complications are associated with generalized essential telangiectasia; however, the diffuse nature of the capillary telangiectases can be a source of emotional distress to patients.[14]

 

DDx

Diagnostic Considerations

Generalized essential telangiectasia is distinguished from other diseases, such as hereditary hemorrhagic telangiectasia, by the apparent absence of any preceding or coexisting cutaneous or systemic disease.[15]

No visceral involvement and no hemorrhagic tendency are noted, although one report describes a patient with generalized essential telangiectasia and gastrointestinal tract bleeding resulting from the presence of a watermelon stomach.[16]

Conjunctival telangiectases are uncommon in generalized essential telangiectasia but have been reported.[9, 10, 11, 12, 13]

The telangiectatic form of mastocytosis known as telangiectasia macularis eruptiva perstans manifests as telangiectases overlying brown macules that may be generalized in distribution.

Although angiokeratomas of Fabry disease are usually papular, they have been observed as flat and may resemble the speckled appearance of the discrete red macules sometimes seen in generalized essential telangiectasia.

Differential Diagnoses

 

Workup

Laboratory Studies

Laboratory test results in generalized essential telangiectasia patients are normal.

Histologic Findings

Microscopically, capillary telangiectases consist of thin-walled ectatic vessels in the dermis, usually in the superficial portion subjacent to the epidermal-dermal junction. They are devoid of muscular, elastic, and adventitial components, and the endothelial cells are alkaline phosphatase negative, all typical features of the venous portion of the capillary loop. Ultrastructural analyses of the vessels are consistent with the telangiectases developing from the existing microvasculature—no evidence of neovascularization is apparent.[17]

These findings are in contrast to those found in the walls of normal terminal arterioles and capillary loops and the telangiectases associated with dermatomyositis, in which the endothelium is alkaline phosphatase positive. These features indicate that the telangiectases of generalized essential telangiectasia are derived from the venous portion of the capillary loop or that the anatomic construction has been altered and the biochemical activity, ie, alkaline phosphatase, has been lost as a consequence of the disease.

In one report, no estrogen or progesterone receptors were present in measurable levels in the single patient with generalized essential telangiectasia in whom tests were performed. Estrogen and progesterone receptors have not been found in randomly occurring telangiectases of the lower extremity.

 

Treatment

Medical Care

Capillary telangiectases (red telangiectases, usually < 0.2 mm in diameter) in generalized essential telangiectasia, as with randomly occurring capillary telangiectases of the lower extremity, are relatively refractory to treatment. No consistently effective treatment can remove the capillary telangiectases of generalized essential telangiectasia, although case reports describe success using photothermal coagulation with laser and intense pulsed light (IPL).[18]

Capillary telangiectases should be distinguished from venous telangiectases (blue telangiectases, usually >0.2 mm in diameter). While venous telangiectases are quite responsive to sclerotherapy, capillary telangiectases are usually resistant to sclerotherapy.[19] In fact, attempting sclerotherapy to remove capillary telangiectases carries a high risk of local development of new capillary telangiectases. These new capillaries often appear as pink or red patches because the capillaries are so numerous. These patches of capillary telangiectases—a well-recognized effect of sclerotherapy—are known as telangiectatic capillary mattes, and this adverse effect of treatment is known as telangiectatic capillary matting.

In one case report, a 39-year-old woman with a 7-year history of progressive generalized essential telangiectasia who was treated empirically using tetracycline noted a decrease in the telangiectases within 3 weeks of beginning oral tetracycline, with complete resolution within 3 months.[20] Treatment using tetracycline had been initiated empirically in this patient because of the vascular resemblance to tetracycline-responsive rosacea. The mechanism of action of the amelioration remains obscure.

In another report, a patient with widespread telangiectases along with autoimmune thyroiditis, progressive muscle weakness, and small varicose veins of the legs had complete clearing of the telangiectases after treatment using oral acyclovir.[21] Involution was noted within 3 weeks, with almost complete clearance at 2 months. Treatment was continued for 5 months without recurrence. Acyclovir had no effect on the larger ectatic veins, muscle weakness, or thyroiditis. Acyclovir was initiated because of the possibility that a viral infection may have been the underlying cause of an autoimmune syndrome.

Another report noted the disappearance of generalized telangiectases with ketoconazole[22] ; however, in most patients affected by generalized essential telangiectasia, no improvement occurs using these or other medications.

Another report indicated success in treating generalized essential telangiectasia with 6-mercaptopurine, which may indicate the condition represents a state of increased angiogenesis.[23]

Cover-up cosmetic makeup and self-tanning lotion can be used to conceal telangiectases. Makeup provides some relief to patients who are self-conscious about their appearance.

Surgical Care

Treatment can be attempted with a laser that specifically targets vascular lesions, including long-pulse Nd:YAG (532 nm), long-pulse frequency-doubled Nd:YAG (1064 nm), or flashlamp-pumped pulsed dye laser (585 nm).[24, 25] However, capillary telangiectases of the lower extremity, unlike those on the face, are not as responsive to photothermal coagulation with currently available vascular lasers. Multiple treatments are usually required, and, often, many of the treated capillaries remain.