Practice Essentials
Unilateral nevoid telangiectasia (UNT) is a cutaneous condition consisting of congenital or acquired patches of superficial telangiectasias 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.
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.
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.
The following laboratory tests may be necessary:
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Serum pregnancy test, if applicable
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Liver function tests
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Hepatitis profile
Punch biopsy of the involved area reveals dilated capillaries in the superficial dermis.
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.
Advise females that the telangiectasias may worsen during pregnancy or with oral contraceptive use.
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
Regarding frequency in the United States, a review of the literature by Wilkin et al described 46 reported cases [3] ; Hynes reported on100 cases. [4] However, as a benign condition, unilateral nevoid telangiectasia is likely underreported. 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.
Congenital unilateral nevoid telangiectasia may be noted in the neonatal period or soon thereafter. In the acquired form, telangiectasias often appear in puberty or in pregnancy, but they may occur at any age.
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Unilateral nevoid telangiectasia on the neck.
