Journal of Symptoms and Signs

2012; Volume 1, Number 1

Review Articles

Spiders on the skin: Spider angioma

Vishal Sharma, MD1, Sourabh Aggarwal, MBBS2, Deepak Vadada, MD3
Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India1; Department of Medicine, University College of Medical Sciences, New Delhi,India2; Department of Gastroenterology, Lenox Hill Hospital, New York, USA3 Corresponding Author: Vishal Sharma, 1819, Gobind Nagar, Subhash Road, Chheharta, Amritsar, Punjab, India. E-mail: docvishalsharma@gmail.com

Abstract
Spider angiomas are cutaneous manifestations usually described with liver disease. They result from dilatation of an arteriole with multiple radiating vessels emerging from it. Pressure on the central arteriole can make the entire lesion disappear which reappears once it is released. The lesions are typically seen in the territory of superior vena cava. The number of lesions varies but more than a couple must prompt concern about underlying liver disease. Histologically, it consists of central arteriole with radial branching of telangiectasia in the periphery. Different theories have advocated increased levels of vascular endothelial growth factor, basic fibroblast growth factor, estrogen/testosterone ratio and substance P as reason for their development. Usually they remain asymptomatic in a majority of subjects and do not need therapy. If treatment is needed for cosmetic purposes, it may include electrocoagulation or laser. These lesions may disappear with improvement in underlying hepatic disease or after liver transplantation. Keywords: spider angioma; cirrhosis; alcoholic liver disease; viral hepatitis; liver
Received: April 3, 2012; Accepted: April 13, 2012; Published online: May 3, 2012

Introduction
Skin is often believed to provide a peep into the human body and careful examination of skin may yield important diagnostic and prognostic information. Perhaps nowhere else is this aphorism better suited than in respect to the spider angiomas and their relation to hepatic diseases [1]. Hepatic disease can have a number of cutaneous findings (Table 1) [2]. Spider angiomas are peculiar manifestations usually described with liver disease and have been variably named as spider nevus, vascular spider, nevus araneus, arterial spider tche http://www.intermedcentral.hk/

stellaire, etoile vasculair or merely as spiders [1, 3]. First described by Erasmus Wilson, the lesions were described in detail by William Bennett Bean [3].

Clinical diagnosis
Spider angiomas are so described for their resemblance to legs of a spider (Figure 1). The lesions result from dilatation of an arteriole with multiple radiating vessels emerging from the central arteriole. Pressure on the central arteriole, typically with a pinhead, can make the entire lesion disappear while the lesion reappears once
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Spider angioma the pressure is released (Figure 2)[4].Occasionally a central pulsation can be noted. The lesions are typically seen in the territory of superior vena cava although the reason is not entirely clear. Therefore the locations usually are the face, neck, and upper part of the trunk, and arms[1]. Other locations from where the lesions have been reported include pleural and sub-pleural locations[5]. Another report has reported esophageal spider naevi in hepatitis C virus related cirrhosis[6]. They have also been reported in the stomach and colon[7, 8]. A report indicated that they may not occur in the retina[9]. Unusual locations like at the knees and distribution like unilateral only distribution have also been reported[10, 11]. The bleeding occuring from Dieulafoys lesion in cirrhotics has also been explained to be a result of bleeding from an underlying spider [8]. Interestingly, even the pulmonary abnormalities that accompany cirrhosis and manifest as hepatopulmonary syndrome have been blamed on pulmonary spider like lesions and described as hepato-pulmonary-cutaneous syndrome [12].The number of lesions vary but more than a couple of lesion must prompt concern about underlying liver disease [4]. Some indicate that even up to five spiders can occur in a normal population [13]. Histologically the lesions consist of central arteriole with radial branching of telangiectasia in the periphery [14]. pregnancy. A higher estradiol/testosterone ratio has been noted in plasma of patients with cirrhosis. This was primarily due to lower free testosterone levels in those cirrhotic patients who had spiders [19]. The increased peripheral conversion of androgens to estrogens may also play a role. In another study of patients with cirrhosis, a higher estradiol/testosterone ratio was noted in patients vis--vis healthy controls [20]. It is believed that relative estrogen excess mediates vasodilatation and results in these vascular lesions. However another study refuted these findings and reported a normal hormonal profile. This study also noted an increased level of substance P in those patients who had spiders [21]. As circulating plasma substance P is partially inactivated in liver, the increase levels may be due to increasing hepatic dysfunction. Substance P might be responsible for the genesis of vascular spiders by causing vasodilatation[21].
Table 1. Cutaneous manifestations and the related liver disease
[2]

Pruritus, Itch marks Xanthoma

Cholestatsis Cholestasis Hemochromatosis, Primary

Skin pigmentation Biliary Cirrhosis Papular acrodermatitis (Gianotti Crosti Syndrome) Scarring Paper money skin Palmar Erythema Purpura, Bleed Viral infections like HCV, HBV Porphyrea cutanea tarda Usually chronic liver disease Usually chronic liver disease Coagulopathy HCV Palpable purpura cryoglobulinemia Muhercke Nails[42] Terry Nails/Leukonychia[42] Hypoalbuminemia Cirrhosis Cirrhosis, Hepatopulmonary Clubbing syndrome Azure lunulae [42] Caput medusa [43] Wilson Portal hypertension related

Mechanisms behind spider naevi

The mechanisms responsible for causation of spider naevi are not entirely clear. Many efforts have been made to identify the etiologic factors responsible for occurrence of spiders (Figure 3). In a case-control study, the levels of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) were determined in patients of cirrhosis and healthy controls. The authors noted a significantly elevated level of both these markers in patients with cirrhosis and also that the levels were higher amongst those cirrhotics who had spider angimas[15]. The authors speculate that disordered angiogenesis mediated via VEGF and bFGF is responsible for causation of these spiders. VEGF is a glycoprotein responsible for proliferation of endothelium and angiogenesis while FGF also has similar actions [16, 17]. Another study however gave conflicting results [18]. The role of a relative hyper-estrogenic state has also been studied in patients with spider naevi in liver disease. This is also believed to be the reason that they are found in
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Spider angioma

Figure 1. Spider angioma

Figure 2.

Spider naevi disappear when pressure is applied at the centre and reappear at release of pressure

Figure 3.

Flowchart depicting the pathogenetic mechanisms in causation of spider naevi

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Spider angioma

Implications of presence of spider naevi

Prevalence of spider naevi has been reported variably and it has been found to be associated with variety of conditions (Table 2). In a study of children with and without chronic liver disease, presence of spider naevi was noted in 38% of controls and 2.5% of the controls had five or more spiders. Of the 34 patients with chronic liver disease studied only four (11.7%) had five or more spiders [13]. Around half of those with chronic liver disease did not have spiders. Spiders, therefore, can be present normally in children. Another study reported around 22% spiders in normal male children and 30% in normal female children [22]. In normal children the numbers increase till puberty and the spiders are more numerous in pubertal females vis--vis males [22]. These numbers decrease after around 13 years of age and then reach the usual adult prevalence of around 10-15%[1, 22]. One report had indicated that spiders may predate the onset of liver disease, although the report had been criticized [23, 24]. Spiders may be an early feature of alcohol abuse [25]. Other than liver related diseases, spider angiomas have been reported in relation to pregnancy. In a study of 60 pregnant women spider naevi were present in 32 of them [26]. Conversely, an Indian study reported no spider angioma in more than 600 female patients and the authors suggested dark skin color as the possible reason for the findings [27]. These lesions have also been reported in other hyper-estrogenic states including usage of oral contraceptives (OCPs), adenocarcinoma of the uterus and the lung [28]. Intriguingly one report blamed stoppage of OCPs for an increase in spiders blaming it on hormone rebound effect[29]. Spiders have also been noted in hyperviscosity syndromes like Waldenstrm's macroglobulinaemia[30, 31]. A report has also described them in association with endocarditis [32]. They have also been described with hyperthyroidism and rheumatoid arthritis [33, 34]. Spider angiomas are usually of no concern and remain asymptomatic in a majority of subjects. Occasionally they have been implicated in causation of bleeding[8, 35]. Reports have indicated that the presumed Dieulafoy's lesion-like bleeding might be resulting from underlying spider naevi [36]. They also are believed to have important prognostic significance. The discriminative power of spider angioma for presence of fibrosis has been reported to be better than certain biochemical
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parameters including aspartate aminotransferase/platelet ratio [37]. Spider naevi have also been reported as a reliable clinical sign for underlying cirrhosis [4]. In patients with hepatic C infection, the presence of spider naevi indicated both the presence of inflammation and fibrosis [38].

Therapy for spider naevi

Usually the spider naevi does not need therapy. If they need to be treated primarily for the cosmetic purposes, the therapy may include electrocoagulation or use of laser. While argon laser was the initial therapy of choice, now the pulsed laser is utilised as it is less painful and scarring is unusual [39]. The lesions may recur after therapy [40]. However these lesions may disappear with improvement in underlying hepatic disease [1]. Spider naevi disappear after liver transplantation [41]. To summarize spider naevi are vascular lesions which may occur normally but may be associated with advanced liver disease and may indicate underlying fibrosis. They usually disappear with treatment of underlying disease. Table 2. Conditions associated with spider nevi Normal Children (20-30%) Adults (15-20%) Cirrhosis Chronic hepatitis Alcoholic hepatitis Pregnancy Oral contraceptives/Estrogen therapy Rare-Hyperviscosity syndromes, Rheumatoid arthritis, OCP withdrawal, Hyperthyroidism, Endocarditis

References
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5. Daimaru, N., et al. [Hypoxemia of liver cirrhosis--an autopsy case study]. Nihon Kyobu Shikkan Gakkai Zasshi, 1990; 28(11): 1504-1510. 6. Nur, F.A., et al. Atypical esophageal vascular lesions observed in liver cirrhosis. Dis Esophagus, 2010; 23(1): E9-E11. 7. Campbell, S. and A. Mee. Gastrointestinal spider nevi. Gastrointest Endosc, 2004; 59(3): 401. 8. Madhira, M.S. and M. Tobi. Isolated gastrointestinal spider nevi: potential clinical significance. Am J Gastroenterol, 2000; 95(10): 3009-3010. 9. Misra, S.P., et al. Spider angiomas are not found in the retina of patients with cirrhosis. J Assoc Physicians India, 1998; 46(8): 711-713. 10. Bharath, S. Atypical spider nevi distribution in liver disease. Arch Intern Med, 1979; 139(10): 1193. 11. Cunliffe, W.J., B. Dodman, and M.J. Butterworth. Unilateral spider naevi. Br J Dermatol, 1972; 87(1): 51-52. 12. Capodicasa, E., F. De Bellis, and C. Muscat. 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Indian J Dermatol Venereol Leprol 2009; 75(4): 403. 25. Bashir, K.R. A necklace of telangiectases: an early clinical sign of alcohol abuse. Can Fam Physician 1984; 30: 2067-2074. 26. Esteve, E., et al. [Physiological cutaneous signs in normal pregnancy: a study of 60 pregnant women]. Ann Dermatol Venereol 1994; 121(3): 227-231. 27. Kumari, R., T.J. Jaisankar, and D.M. Thappa. A clinical study of skin changes in pregnancy. Indian J Dermatol Venereol Leprol 2007; 73(2): 141. 28. Brear, S.G., et al. Hypertrophic osteoarthropathy, spider naevi and oestrogen hyperexcretion associated with adenocarcinoma. Postgrad Med J 1985; 61(719): 827-828. 29. Allgaier, H.P. and H.E. Blum. [Spider naevi after discontinuation of oral contraceptives]. Dtsch Med Wochenschr 1996; 121(20): 676. 30.Torok, L., I. Borka, and G. Szabo. Waldenstrom's macroglobulinaemia presenting with cold urticaria and cold purpura. Clin Exp Dermatol 1993; 18(3): 277-279. 31. Tuppal, R., et al. Eruptive spider nevus-like lesions associated with the hyperviscosity syndrome. Arch Dermatol 1992; 128(6): 860. 32. Cook, T. and C.G. Cobbs. Spider angiomata in endocarditis. Ala J Med Sci 1971; 8(3): 276-278. 33. Kavak, A. and L. Kutluay, Unilateral nevoid telangiectasia and hyperthyroidism: a new association or coincidence? J Dermatol, 2004. 31(5): 411-414. 34. Bland, J.H., R. O'Brien, and R.E. Bouchard, Palmar erythema and spider angiomata in rheumatoid arthritis. Ann Intern Med 1958; 48(5): 1026-1032. 35. Calvo Hernandez, R., J.C. Erdozain Sosa, and J.M. Segura Cabral. [Unusual complication of spider angiomas: hemorrhagic diathesis controlled with antihormonal therapy (tamoxifen)]. Rev Esp Enferm Dig 2006; 98(7): 558-559. 36. Akhras, J., P. Patel, and M. Tobi. Dieulafoy's lesion-like bleeding: an underrecognized cause of upper gastrointestinal hemorrhage in patients with advanced liver disease. Dig Dis Sci 2007; 52(3): 722-726. 37. Niederau, C., et al. 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Cruveilhier-Baumgarten syndrome with caput medusae. N Z Med J 2011; 124(1347): 89-90 Copyright: 2012 Vishal Sharma, et al. This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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