Right-sided vascular lesions: answer.

DISCUSSION Unilateral nevoid telangiectasia syndrome (UNTS) is a rare usually acquired vascular disease of unknown etiology, characterized by multiple blanching telangiectasias in a unilateral or Blaschkoid distribution, with preference for the C3 to T1 dermatomes. The first cases were described by Blaschko in 1899, with introduction of the term ‘‘unilateral nevoid telangiectasia’’ by Selmanowitz in 1970. In 1978, Wilkin proposed the term ‘‘unilateral dermatomal superficial telangiectasias. ’’ Less than 100 cases have been described in the literature, though the condition is likely underreported. Most patients are females who are pubertal, pregnant, or exposed to exogenous hormones suggesting a hyperestrogenic state as a precipitating factor. In cases with transient periods of elevated estrogen, the lesions may partially regress when hormone levels return to baseline. Male patients exhibit congenital and acquired disease. Associated underlying conditions include chronic liver disease, hepatitis B and C infections, portal hypertension, and alcoholism. UNTS has also been reported with erythema palmare, metastatic carcinoid syndrome, tuberculosis, bronchitis, cryptorochidism, scleroderma, and hyperthyroidism. Pranteda et al described a 58-year-old woman with UNTS on the right hip and papillary serous cystoadenocarcinoma of the right ovary. Oral and gastric involvement have also been reported. Though the pathophysiology of this condition is unclear, estrogen exposure likely plays a central role. Estrogens are involved in angiogenesis, as evidenced by the development of spider angiomas in the elevated estrogenic state of cirrhosis. Uhlin and McCarty demonstrated increased estrogen and progesterone receptors in lesional skin from a pregnant patient, however, these results have not been replicated. In 2 patients with hepatitis C–associated UNTS, there was no difference in estrogen receptor expression in lesional versus nonlesional skin. However, given that this condition occurs along Blaschko lines, somatic mosaicism may result in exaggerated numbers of estrogen receptors in affected tissue subsequently activated during periods of increased estrogen exposure. A similar mechanism is believed operative in Becker nevus with respect to androgens, the 2 entities having been described concomitantly. A patient with trisomy 16 mosacism, unilateral telangiectasias, hypertrichosis, hyperkeratosis, and hyperpigmentation has been reported lending further plausibility to these theories. Other possible etiologic factors include prostaglandins, angiogenesis factors (vascular endothelial growth factor, epidermal growth factor), and adrenergic receptors, which, if increased in lesional skin could elicit capillary dilatation. Sharma and Khandpur have postulated UNTS may represent hereditary hemorrhagic telangiectasia with incomplete genetic penetrance or de novo postzygotic somatic mutation of endoglin or the ALK-1 gene. Additional hypotheses include altered elastic fibers, neural control of vascular tone, or the presence of a ‘‘laminate capillary. ’’ Considering the bulk of investigative data, it seems most likely the vascular changes result from an increase in their luminal diameter rather than the production of new vessels. Clinically, affected patients develop telangiectasias with a predilection for the face, neck, shoulder, arm, and thorax. Histologically, dilated vascular spaces are noted in the papillary and superior reticular dermis in contrast to angioma serpiginosum in which dilated capillaries with thick walls are found within the dermal papillae. Their appearance is typical of postcapillary venules and endothelial cell proliferation is not present. The lack of D 240 staining in our patient confirms these lesions as telangiectatic in nature rather than lymphatic. A recently described entity, acquired elastotic hemangioma, exhibits some histologic overlap with the findings of UNTS however in the case series by Martorell-Clatayud et al the majority of their case were D2-40 positive. Kreft et al evaluated a 29-year-old pregnant woman with UNTS using laser-Doppler flowmetry and periungual capillary microscopy and demonstrated marked increase in perfusion of her affected right hand. Patients with UNTS should be investigated for a hyperestrogenic state (if not already apparent) from hepatic disease or infection and internal malignancy. Histologic evaluation may be helpful in ruling out other entities such as angioma serpiginosum. UNTS is benign with treatment reserved for cosmetic concerns. Flash–lamp pulse dye laser therapy (585 nm) has been noted to improve the appearance of UNTS with as few as 2 to 4 sessions. Reversible dyschromia may result, and in a few patients, mild textural change may occur. From the *Department of Dermatology, Boston University School of Medicine, Boston, MA; †Departments of Medicine and ‡Pathology, Vanderbilt University, Nashville, Tennessee. The authors have no funding or conflicts of interest to disclose. Reprints: Alan S. Boyd, MD, Departments of Medicine Dermatology and Pathology, Vanderbilt University, Thompson Lane, Suite 26300, Nashville, TN (e-mail: alan.boyd@vanderbilt.edu). Copyright 2012 by Lippincott Williams & Wilkins