Unilateral Nevoid Telangiectasia 

Updated: Mar 09, 2017
Author: Rajani Katta, MD; Chief Editor: Dirk M Elston, MD 

Overview

Background

Unilateral nevoid telangiectasia (UNT) is a cutaneous condition consisting of congenital or acquired patches of superficial telangiectases in a unilateral linear distribution. Described in 1899 by Blaschko, its segmental pattern suggests a mechanism of somatic mosaicism apparent early in life or unmasked in states of relative estrogen excess, such as that in pregnancy or in chronic liver disease.

Also see Ataxia-Telangiectasia and Generalized Essential Telangiectasia.

Pathophysiology

Vascular malformations may result from a somatic mutation during embryologic development giving rise to distinct cell populations in a single individual. Paradominant inheritance, in which an autosomal mutation results in loss of heterozygosity and gives rise to a mosaic patch or twin spots, may explain the rare occurrence of telangiectatic nevi in several family members.[1, 2] Whether these alterations include different concentrations of receptors for vasoactive substances or hormones remains to be determined. Telangiectasias are almost always limited to the skin, and associated gastric telangiectasias reported in one case may have been due to the underlying hepatic dysfunction.

Epidemiology

US frequency

A review of the literature by Wilkin et al in 1983 described 46 reported cases[3] ; according to Hynes, 100 cases were reported by 1997.[4] However, as a benign condition, unilateral nevoid telangiectasia is likely underreported.

Sex

The congenital type, though rare, is more common in males than in females. The acquired form is noted more often in females than in males.

Age

Congenital unilateral nevoid telangiectasia may be noted in the neonatal period or soon thereafter. In the acquired form, telangiectases often appear in puberty or in pregnancy, but they may occur at any age.

Prognosis

In acquired unilateral nevoid telangiectasia, the telangiectasias may improve as the estrogen levels decrease, such as following pregnancy or with the discontinuation of oral contraceptives. In other cases of acquired or congenital unilateral nevoid telangiectasia, the lesions persist.

Patient Education

Advise females that the telangiectasias may worsen during pregnancy or with oral contraceptive use.

 

Presentation

History

Cutaneous lesions are asymptomatic and may go unrecognized. Inquiry into possible stimulus for development may include relation to puberty, pregnancy, contraceptive use, or hepatic diseases.

Physical Examination

Patches of superficial, blanchable telangiectasias may be small to large and few to numerous. They are disposed predominantly in a unilateral linear distribution. Note the image below.

Unilateral nevoid telangiectasia on the neck. Unilateral nevoid telangiectasia on the neck.

The third and fourth cervical dermatomes are the most common sites, but the thoracic dermatomes and scattered distant sites may also be involved.

Causes

The pathogenesis of unilateral nevoid telangiectasia remains unknown.

The occurrence of acquired unilateral nevoid telangiectasia in certain settings characterized by elevations of estrogen suggests hormonal causes. Such settings include puberty, pregnancy, and during oral contraceptive use. The other major setting in which this has been reported to occur is in states of hepatic dysfunction. Unilateral nevoid telangiectasia has been reported in patients with chronic liver disease due to alcoholism or hepatitis C, as well as liver metastases from carcinoid tumor[5]  and colon cancer. [6]

Uhlin et al documented increased levels of estrogen and progesterone receptors in involved areas compared with normal skin[7] ; however, other reports have not been able to reproduce this finding.

Because unilateral nevoid telangiectasia has been noted in cases in which no underlying hormonal or hepatic pathology has been found, other mechanisms probably play a role in the pathogenesis.

Although reported cases often involve a dermatome or a group of dermatomes, distribution following the lines of Blaschko suggests that a postzygotic somatic mutation leads to a distinct cell population in the affected site (mosaicism).

 

DDx

 

Workup

Approach Considerations

Individual case reports have described unilateral nevoid telangiectasia in association with other systemic abnormalities. Such associations include cases associated with hormonal disturbances and hepatic dysfunction. Case reports have described an association with hyperthyroidism[8, 9] and a case of unilateral nevoid telangiectasia occurring in a hepatitis B carrier without evidence of liver dysfunction.

In 2013, Tanglertsampan et al reviewed the literature and found multiple case reports of unilateral nevoid telangiectasia, some of which were associated with other diseases.[9] It is not clear if these are coincidental or not. The authors proposed a new classification system, in which acquired cases may be divided into cases with an association and cases without. Associated conditions included estrogen-related (pregnancy, puberty in women), liver-related (cirrhosis, hepatitis C, hepatitis B), skin-related (possibly pyogenic granuloma), and others (including hyperthyroidism).

Laboratory Studies

The following laboratory tests may be necessary:

  • Serum pregnancy test, if applicable

  • Liver function tests

  • Hepatitis profile

 

Other Tests

Punch biopsy of lesional and perilesional skin for comparison may be considered. Skin biopsy is usually not necessary because the diagnosis is usually established on clinical grounds.

Other testing is not necessary. However, Kreft et al have described the results of laser-Doppler flowmetry in a patient with unilateral nevoid telangiectasia.[10] They were able to demonstrate increased perfusion in areas of unilateral nevoid telangiectasia, even in areas that were clinically unremarkable. They also found that capillary microscopy demonstrated morphologically altered capillaries.

Histologic Findings

Punch biopsy of the involved area reveals dilated capillaries in the superficial dermis.

 

Treatment

Medical Care

In cases of acquired unilateral nevoid telangiectasia, observe for progression or improvement after addressing possible estrogen excess.

In congenital unilateral nevoid telangiectasia or in cases of acquired unilateral nevoid telangiectasia that do not improve, treatment is elective.

If cosmesis is desired, camouflage cosmetics or vascular laser treatment of the involved areas may be helpful.

Surgical Care

Pulsed dye lasers with a wavelength of 585 nm and a pulse width of 450 microseconds offer a safe effective treatment of telangiectases. Use caution in the thinner skin in the neck area to avoid blistering and potential scarring. Cliff and Harland reported their results with the use of pulsed-dye laser in the treatment of 5 patients with unilateral nevoid telangiectasia.[11] They found that pulsed-dye laser was an effective treatment, but they noted a recurrence of lesions in all cases. Sharma and Khandpur, on the other hand, found no recurrence in their 6 Indian patients treated with the 585-nm pulsed dye laser.[12] A moderate response was achieved in all patients, and reversible pigmentary changes were the major adverse effects.

Pulsed-dye lasers with longer wavelengths and pulse widths are now available for larger vessels.

Pulsed KTP (potassium-titanyl-phosphate) 532-nm, long-pulsed alexandrite 755-nm, pulsed diode 800- to 930-nm, long-pulsed Nd:YAG 1064-nm, and filtered broad-spectrum pulsed light sources have been used for vascular lesions, with less postoperative purpura.