SEARCH

SEARCH BY CITATION

Unilateral nevoid telangiectasia (UNT) is a rare condition characterized by telangiectasias distributed in a linear, unilateral pattern. The etiopathogenesis is not completely understood, although it is believed that high levels of estrogens are behind its development.1 It can be congenital or acquired. In these cases, it is related to hyperestrogenism.1

We report two cases of UNT in association with chronic liver disease without cirrhosis.

The first refers to a 41-year-old woman who presented with multiple telangiectasias on her right upper thorax and shoulder (Fig. 1a) for five months. She had been submitted to a hepatic transplant four years before, after a fulminant hepatitis due to hepatitis B virus (HBV) infection, which was followed by treatment with lamivudine. Laboratory tests including complete blood cell count and biochemistry were unremarkable. The coagulation panel was within the normal ranges. Antibodies to HBV, which had been negative for the last four years, were positive, although DNA viral charge was within normal values. The hepatic ultrasound showed no cirrhosis. A skin biopsy was made revealing multiple dilated, thin-walled vessels in the papillary and upper reticular dermis (Fig. 1b,c).

image

Figure 1.  (a) Patient 1: telangiectasias on right upper thorax and shoulder. (b) Multiple, dilated vessels in the papillary and upper reticular dermis (H&E, 4×). (c) Detail of dilated vessels (H&E 20×). (d) Patient 2: telangiectasias on left shoulder

Download figure to PowerPoint

The second case refers to a 34-year-old man with hepatitis C virus (HCV) infection diagnosed three years before. The patient denied any prescribed or over-the-counter medication. He presented multiple telangiectasias over his left shoulder (Fig. 1d), which had appeared a few years before and had increased in number over the last few months. The laboratory tests were unremarkable, except for positive anti-HCV antibodies, although the RNA viral charge was within normal values. A skin biopsy showed dilated vessels in the superficial dermis.

Unilateral nevoid telangiectasia is a vascular dermatosis that was first described in 1899 by Zeisler and Blaschko.2 It represents a unilateral and linear proliferation of superficial telangiectasias.1 It can be congenital or acquired. The congenital type is transmitted in an autosomal dominant pattern and is believed to be influenced by maternal estrogens.2 The acquired type frequently occurs in situations related to an increased estrogens’ production, such as puberty, pregnancy, the use of oral contraceptives, or chronic liver diseases. It has also been described in patients with hyperthyroidism, alcoholism, carcinoid syndrome with liver metastases, and even in healthy individuals.2

The most accepted theory is that it may result from a localized increase in estrogen receptors caused by a somatic mosaicism,3 which explains the distribution in a segmental pattern. These lesions become unmasked in situations of estrogen excess.3 Estrogens promote endothelium-dependent relaxation by increasing nitric oxide and prostacyclin and inhibiting the mechanisms of vascular smooth muscle contraction, contributing to the formation of telangiectasias.4

Clinically it manifests as an asymptomatic, segmental distribution of telangiectasias, frequently found in C3–C4 or the trigeminal dermatomal areas.1 The histopathology is characterized by a normal-appearing epidermis with underlying superficial dermal telangiectatic vessels.3 As this is a benign condition, there is no need for treatment. Nevertheless, the treatment of choice is the pulsed-dye laser therapy.3 In most cases, the cosmetic result is excellent.5

In both our patients, the history of chronic hepatic disorders along with the characteristic distribution of the lesions and the histopathological confirmation of superficial telangiectasias confirmed the diagnosis of UNT. In both cases, estrogen levels were not performed, which would be helpful to support the diagnosis, if increased.

Hyperestrogenism in liver diseases is attributed to reduced hepatic catabolism of estrogens and weak androgens. The weak androgens are converted to estrogenic compounds in peripheral tissues, thereby adding to the burden of circulating estrogens. Hyperestrogenism is much more evident when extrahepatic portal hypertension due to cirrhosis is present, as the portal-systemic shunts permit these hormones to bypass the liver.6

References

  1. Top of page
  2. References
  • 1
    Afsar FS, Ortac R, Diniz G. Unilateral nevoid telangiectasia with no estrogen and progesterone receptors in a pediatric patient. Indian J Dermatol Venereol Leprol2008; 74: 163164.
  • 2
    Jordão JM, Haendchen LC, Berestinas TC, Faucz LRL. Acquired unilateral nevoid telangiectasia in a healthy man. An Bras Dermatol2010; 85: 912914.
  • 3
    Wenson SF, Jan F, Sepehr A. Unilateral nevoid telangiectasia syndrome: a case report and review of the literature. Dermatol Online J2011; 17: 2.
  • 4
    Reslan OM, Khalil RA. Vascular effects of estrogenic menopausal hormone therapy. Rev Recent Clin Trials2011; 25. PMID: 21864249.
  • 5
    Sharma VK, Khandpur S. Unilateral nevoid telangiectasia – response to pulsed-dye laser. Int J Dermatol2006; 45: 960964.
  • 6
    Rubin R, Strayer DS. The liver and biliary system. In: Rublin R, Strayer DS, eds. Rubin’s Pathology, 5th edn. Philadelphia, PA: Lippincott Williams & Wilkins, 2008: 631.