Are You Confident of the Diagnosis?
What you should be alert for in the history
Patients most often present complaining of the cosmetic appearance of telangiectasias. The unilateral nevoid telangiectasia (UNT) can be acquired or congenital. Often the telangiectasias appear during puberty or pregnancy. Less commonly, the eruption is congenital and appears in the neonate or infant.
Characteristic findings on physical examination
Characteristically, spider telangiectasias with central puncta and ‘legs’ extending out from the center occur in a unilateral dermatomal or ‘Blashkoid’ distribution. These superficial dilated vessels are also described as appearing ‘thread like’ in some patients or as telangiectatic macules. These blanchable lesions may have a white ring around them, also called an anemic halo. The common distribution is the face, neck, arm or thorax (trigeminal, C3-T1 dermatomes) (Figure 1, Figure 2). Hepatic disease and hyperthyroidism has also been associated with UNT.
Expected results of diagnostic studies
The history and exam is often adequate to make the diagnosis. If a biopsy is performed, dilated vessels in the papillary dermis will be apparent.
Conditions in the differential diagnosis include:
Linear atrophoderma of Moulin (telangiectatic variant) – UNT has no atrophy
Hereditary benign telangiectasia – UNT is unilateral and not inherited
Hereditary hemorrhagic telangiectasia – UNT characteristically does not involve mucous membranes or cause hemorrhage
Universal angiomatosis – bleeding in organs is not characteristically found in UNT
Generalized essential telangiectasia – UNT is unilateral
Pigmented purpuric dermatosis – UNT is usually seen on the upper half of the body, purpuric dermatosis is often seen on the legs and is often bilateral
Angioma serpiginosum – often seen on the buttocks and lower limbs with irregular lesions
Who is at Risk for Developing this Disease?
Unilateral nevoid telangiectasia is relatively rare. It is generally not considered hereditary though one familial case has been described. The congenital form is more common in boys while the acquired form is more common in woman.
What is the Cause of the Disease?
The etiology of UNT is unknown though there are many theories. One theory supposes that there is mosaicism associated with distribution of estrogen receptors leading to differential response to hormone fluctuations. There are cases in the literature of UNT associated with other anomalies in the same distribution, a phenomenon attributed to “twin spotting”. Twin spotting depends upon mosaicism associated with somatic recombination.
Other possible factors involved in the pathophysiology include hemodynamic disturbances, neural alteration, angiogenic factors, and changes in supportive connective tissue and vasculature.
Systemic Implications and Complications
UNT can develop during puberty, pregnancy or the use of hormonal birth control in women. Although it may not always present in a first pregnancy, if it does, it will usually recur in subsequent pregnancies. It is also associated with liver disease including alcoholism (with and without cirrhosis), hepatitis B and C, portal hypertension and metastatic carcinoid to the liver.
Hyperthyroidism may also be associated.
There is a case report of UNT associated with underlying melorheostosis
With the proper history, no work up is necessary. When no clear associated condition is elicited, it may be prudent to send blood tests for hepatitis serologies and for thyroid studies (thyroid stimulating hormone).
There are no medical treatments. Withdrawal of hormonal supplements like birth control pills may lead to resolution.
Pulsed dye laser was used with more than 75% clearance in 50% of patients. Use can be associated with transient hypo and hyperpigmentation. Published laser parameters are: spot size 7mm; fluence 5-7.5 J/cm2 ; wavelength 585nm; pulse duration 450µs. Sessions were repeated every 4-6 weeks and required 2-5 treatments.
Optimal Therapeutic Approach for this Disease
This is a cosmetic complaint and needs to be approached that way. Changing method of birth control may help in a patient using oral contraceptives if that is otherwise appropriate to their medical care and life style. A pregnant patient should be counseled to wait and see if the lesions lessen post-partum as there may even be complete resolution.
For those distressed enough from the appearance and requesting therapy, use of pulsed dye laser is optimal. Patients should expect it will take two to five treatments to reach optimal results. There may be transient purpura, and months of hyperpigmentation or hypopigmentation.
Patients with UNT do not need to be monitored or followed for the skin condition (though obviously if they have liver or thyroid pathology the appropriate specialist will need to follow them). Patients being treated with lasers need to be informed that it is possible new telangiectasias will develop and only the ones that can be currently visualized can be treated. However, patients with long standing stable lesions are unlikely to develop new telangiectases.
Unusual Clinical Scenarios to Consider in Patient Management
Although UNT is generally considered to be limited to the skin, a case of gastric telangiectasias in a man with UNT and cirrhosis has been reported. If a patient has evidence of blood loss without other known cause, gastrointestinal imaging to rule out systemic telangiectasias may be warranted.
What is the Evidence?
Abbas, O, Rubeiz, N, Ghosm, S. “Extensive and progressive eruption in a young woman”. Clin Exp Dermatol. vol. 35. 2010. pp. e85-86. (A case report with a brief summary of the epidemiology and associated findings of UNT.)
Anderton, RL, Smith, G. “Unilateral nevoid telangiectasia with gastric involvement”. Arch Dermatol. vol. 111. 1975. pp. 617-621. (A case report of unilateral nevoid telangiectasia in a man with cirrhosis and gastric telangiectasias. Although the gastric telangiectasias were not unilateral, the authors surmise that they may represent a systemic component to UNT.)
Chen, LA, Tsai, TH, Chang, YJ, Su, LH, Hsu, YL, Chan, JY. “Unilateral nevoid telangiectasia – response to long-pulsed Nd:YAG laser and pulsed dye laser: a case report”. Dermatologica Sinica. vol. 28. 2010. pp. 44-45. (A brief report noting that the long-pulsed Nd:YAG laser was not helpful but that UNT does respond to the 595-nm pulsed dye laser.)
Dadlani, C, Kamino, H, Walters, RF, Rosenman, K, Pomeranz, MK. “Unilateral nevoid telangiectasia”. ermatol Online J. vol. 14. 2008. pp. 3(A case report of UNT becoming more prominent during pregnancy with a discussion of the pathophpysiology as well as the differential diagnosis.)
Kim, J, Cho, SB, Cho, S, Bang, D. “Unilateral Nevoid Telangiectasia associated with ipsilateral melorheostosis”. Ann Dermatol. vol. 24. 2012. pp. 206-208. (A case report of unilateral nevoid telangiectasia in a child with melorheostosis diagnosed by xray and bone scintigraphy. There was a limb length discrepancy between the arms. The authors suggest twin spotting may explain the coexistence of the two conditions.)
Mirrer, E, Cipriano, A, McGuire, J. “Unilateral nevoid telangiectasia”. Arch Dermatol. vol. 103. 1971. pp. 320-323. (A case report of unilateral neviod telangiectasia with a review of the differential of primary telangiectasias. A table summarizes the main conditions to be differentiated: hereditary hemorrhagic telangiectasia, generalized essential telangiectasia and universal angiomatosis.)
Pinho, A, Oliviera, H, Gouveia, M, Vieira, R. “Bier spots and unilateral nevoid telangiectasia: more than just a coincidence”. An Bras Dermatol. vol. 90. 2015. pp. 769-70. (A case report of Bier spots and unilateral nevoid telangiectasia with a discussion of twin spotting.)
Selmanowitz, VJ. “Unilateral nevoid telangiectasia”. Ann Int Med. vol. 73. 1970. pp. 87-90. (This brief report of two patients coins the term 'unilateral nevoid telangiectasia' and purports a possible neural mechanism.)
Sharma, VK, Khandpur, S. “Unilateral nevoid telangiectasia – response to pulsed dye laser”. International J Dermatol. vol. 45. 2006. pp. 960-4. (This report from India on the use of pulsed dye laser shows its efficacy in patients with skin types III and IV. The main adverse effect is pigmentary alteration.)
Wilkin, JK, Smith, G, Cullison, DA, Peters, GE, Rodriguez-Rigau, LJ, Feucht, CL. “Unilateral dermatomal superficial telangiectasia: nine new cases and a review of unilateral dermatomal superficial telangiectasia”. J Am Acad Dermatol. vol. 8. 1983. pp. 468-477. (This is a case series of 9 patients and review of all previously reported cases. It provides a classification for this entity: congenital and acquired with subtypes of acquired based on underlying etiologic factor (physiologic estrogen increase or hepatic related).)
Woollons, A, Darley, CR. “Unilateral naevoid telangiectasia syndrome in pregnancy”. Clin Exp Dermatol. vol. 21. 1996. pp. 459-460. (A report of UNT occurring for the first time in a third pregnancy (though only one previous pregnancy went to term).)
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