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Unilateral nevoid telangiectasia

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Unilateral nevoid telangiectasia
Chicky Dadlani MD, Hideko Kamino MD, Ruth F Walters MD, Karla Rosenman MD, Miriam K Pomeranz MD
Dermatology Online Journal 14 (10): 3

Department of Dermatology, New York University


A 30-year-old woman, who was six months pregnant, presented with multiple, blanching, asymptomatic telangiectasies on her right upper extremity for two years. At the onset of her pregnancy, her lesions increased in number and redness. Given the unilateral distribution and worsening during pregnancy, a diagnosis of unilateral nevoid telangiectasia was made. This condition is a rare entity that has been most commonly reported in association with puberty, pregnancy, the use of oral contraceptives, and alcoholic cirrhosis. However, there have been case reports in otherwise healthy individuals.


A 30-year-old woman, who was six months pregnant, presented to the Dermatology Clinic at Bellevue Hospital Center in September, 2007, with a two-year history of multiple, scattered, asymptomatic red spots on her right arm. Over the past six months of her pregnancy, the patient noted an increase in the number and the redness of her lesions. The patient denied any prior treatment. Prenatal vitamins were her only medication. No family members had similar skin findings. Past medical history was not contributory.

Figure 1Figure 2

Physical Examination

Multiple, blanching, scattered, discrete 1-to-3-mm telangiectaseas with a peripheral anemic halo involved her right upper extremity and extended from her shoulder to dorsal hand distally. Additionally, 1-to-2-mm spider telangiectaseas were noted on the dorsal aspect of the left hand.




In the papillary and upper reticular dermis, there are multiple, dilated, thin-walled vessels lined by plump endothelial cells.


Unilateral nevoid telangiectasia (UNT) was first described in 1899 by Blaschko [1]. Many designations have been suggested to describe this entity, which include acquired spider telangiectases, microtelangiectasia essential progressive unilateral [2], unilateral spider nevi, and linear telangiectasias. In 1970, Selmanowitz [3] proposed the name unilateral nevoid telangiectasia, and Wilkin [4] proposed the term unilateral dermatomal superficial telangiectasia in 1978. These terms have been predominantly used since then.

Unilateral nevoid telangiectasia is a rare entity (fewer than 100 reported cases) that can be congenital or acquired. The acquired form has been associated with physiologic conditions, such as pregnancy, puberty, or hormonal therapy. The exact pathogenesis is obscure and unclear. Unilateral nevoid telangiectasia is an asymptomatic vascular dermatosis that is characterized by multiple, unilateral, linearly arranged, blanching telangiectases in a dermatomal or Blaschkoid pattern. A pale ring that is referred to as anemic halo may be observed surrounding the telangiectases. Some authors report that UNT may present as erythematous macules that, upon closer inspection, reveal telangiectases. Sites of predilection include the face, neck, shoulder-arm region, and thorax [5]. The differential diagnosis of UNT includes the telangiectactic variant of linear atrophoderma of Moulin. However, this entity is characterized by cutaneous atrophy, telangiectatic macules, and hyperpigmentation [6].

On histopathology, superficial small vascular channels can be seen in the papillary dermis [5].

Circumstantial evidence suggests a strong association with a hyperestrogenic state. However, there have been case reports in otherwise healthy individuals [3, 7, 10]. Most cases reported to date have occurred in women during pregnancy, puberty, or with the use of oral contraceptives. In men, alcoholic cirrhosis is the most common association. Unilateral nevoid telangiectasia has also been described in patients with hyperthyroidism [10], alcoholism without cirrhosis, and chronic liver diseases, which include hepatitis B and C infections, portal hypertension, and carcinoid syndrome with liver metastases. It has been proposed that UNT may result from a localized increase in estrogen receptors caused by a chromosomal mosaicism that is unmasked at times of relative estrogen excess thus accounting for the distribution that follows Blaschko lines [1].

Telangiectasies can be divided into two groups: primary and secondary. Disorders with primary telangiectasies include: ataxia telangiectasia, hereditary hemorrhagic telangiectasia, nevus araneus, venous lakes, universal angiomatosis, benign generalized essential telangiectasies, and unilateral nevoid telangiectasia. The entities with secondary telangiectasies can be associated but not limited to the following: connective-tissue diseases, telangiectasia macularis eruptiva perstans, basal-cell cancers, xeroderma pigmentosa, rosacea, and post-radiation injury [8]. It is well known that pregnancy may cause an increase in spider angiomas; however, these do not occur in a unilateral distribution. Given the patient's localized eruption, histopathologic features, changes associated with pregnancy, a diagnosis of UNT was favored. It has been suggested that estrogen stimulates abnormal target end organs congenitally distributed in a dermatomal or linear pattern to produce telangiectases [4, 5, 10]. Although no etiology has been agreed upon, other speculated causes, of UNT include hemodynamic disturbances, neural alterations, a role for angiogenic factors, and aberrations in perivascular supportive connective tissue [3, 4, 7, 12, 13]. Pulse dye laser has been a useful modality in treating lesions [13].


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