Unilateral Nevoid Telangiectasia
A 22-year-old woman presented to the dermatology clinic with asymptomatic erythematous patches on her right upper extremity (Figure 1) and right chest of 14 years duration. During the seventh month of pregnancy, these lesions increased in number and size, and became mildly pruritic.
Upon delivery of a healthy child 2 months later, there was no improvement in signs or symptoms. To date, the patient denied previous attempts at treatment.
She was otherwise healthy, taking no medications and had no family members with similar skin findings.
Laboratory tests. Her work-up included a normal serum chemistry panel, normal complete blood count, and normal liver studies. A biopsy of the affected area performed was non-specific and suggestive of sun damage; she was referred to a dermatologist for a consultation.
Physical examination. She had multiple 2 mm blanching erythematous telangiectatic macules with anemic halos. These telangiectasias were scattered on her right upper extremity, most prominent on her extensor forearm, and extended to the right proximal shoulder girdle and right upper chest (Figures 2-4). The left upper extremity and chest were not affected. The diagnosis of unilateral nevoid telangiectasia (UNT) was made based on the patient’s history and physical exam, and another histopathologic exam was not pursued. The patient was not concerned about cosmesis and therefore, did not want treatment.
Discussion. UNT, a vascular dermatosis first described in 1899 by Blaschko, is a rare cutaneous condition characterized by multiple, usually asymptomatic, blanching telangiectasias with pale peripheral rings, referred to as anemic halos.1,2,3 The affected area is unilateral, presenting in a dermatomal or Blaschko-like distribution, and commonly involves the trigeminal or C3-C4 region.1-7 Biopsy may show the characteristic pattern of numerous dilated capillaries in the papillary dermis.1
UNT may be congenital or acquired; the acquired form being more common in women and often associated with hyperestrogenic states, such as puberty, pregnancy, and liver disease.1-7 Work-up can be guided towards ruling out such causes although there are reports of UNT in otherwise healthy people.1-6
The pathogenesis is unknown; the most commonly accepted theory is that an increased estrogen level unmasks a localized increase in estrogen receptors caused by chromosomal mosaicism.1,3,5,7 The telangiectasias may improve as estrogen levels decrease, such as in the postpartum period, and may recur with return of a hyperestrogenic state.4-6 Treatment to improve cosmetic disfigurement may be implemented with pulsed dye laser.1
References:
1.Dadlani C, Kamino H, Walters RF, et al. Unilateral nevoid telangiectasia. Dermatol Online J. 2008;14(10):3.
2.Selmanowitz VJ. Unilateral nevoid telangiectasia. Arch Dermatol. 1972;105(1):131.
3.Hynes LR, Shenefelt PD. Unilateral nevoid telangiectasia: occurrence in two patients with hepatitis C. J Am Acad Dermatol. 1997;36(5 Pt 2):819-822.
4.Tok J, Berberian BJ, Sulica VI. Unilateral nevoid telangiectasia syndrome. Cutis. 1994;53(1):53-54.
5.Uhlin SR, McCarty KS Jr. Unilateral nevoid telangiectatic syndrome. The role of estrogen and progesterone receptors. Arch Dermatol. 1983;119(3):226-228.
6.Kreft B, Marsch WC, Wohlrab J. Unilateral nevoid telangiectasia syndrome. Dermatology. 2004;209(3):215-217.
7.Telangiectasias. In: Bolognia J, Jorizzo J, Rapini R. Dermatology Essentials. Vol 2. St. Louis, MO: Mosby/Elsevier; 2008.