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
Frequency
United States
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.
Happle R. Loss of heterozygosity in human skin. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):143-64. [Medline].
Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol. Nov 1993;129(11):1460-70. [Medline].
Wilkin JK, Smith JG Jr, Cullison DA, Peters GE, Rodriquez-Rigau LJ, Feucht CL. Unilateral dermatomal superficial telangiectasia. Nine new cases and a review of unilateral dermatomal superficial telangiectasia. J Am Acad Dermatol. Apr 1983;8(4):468-77. [Medline].
Hynes LR, Shenefelt PD. Unilateral nevoid telangiectasia: occurrence in two patients with hepatitis C. J Am Acad Dermatol. May 1997;36(5 Pt 2):819-22. [Medline].
Beacham BE, Kurgansky D. Unilateral naevoid telangiectasia syndrome associated with metastatic carcinoid tumour. Br J Dermatol. Jan 1991;124(1):86-8. [Medline].
Uhlin SR, McCarty KS Jr. Unilateral nevoid telangiectatic syndrome. The role of estrogen and progesterone receptors. Arch Dermatol. Mar 1983;119(3):226-8. [Medline].
Kavak A, Kutluay L. Unilateral nevoid telangiectasia and hyperthyroidism: a new association or coincidence?. J Dermatol. May 2004;31(5):411-4. [Medline].
Kreft B, Marsch WC, Wohlrab J. Unilateral nevoid telangiectasia syndrome. Dermatology. 2004;209(3):215-7. [Medline].
Cliff S, Harland CC. Recurrence of unilateral naevoid telangiectatic syndrome following treatment with the pulsed dye laser. J Cutan Laser Ther. Apr 1999;1(2):105-7. [Medline].
Sharma VK, Khandpur S. Unilateral nevoid telangiectasia--response to pulsed dye laser. Int J Dermatol. Aug 2006;45(8):960-4. [Medline].
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