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Recognizing and treating cutaneous signs of liver disease

Cleveland Clinic Journal of Medicine. 2009 October;76(10):599-606 | 10.3949/ccjm.76A.08113
October 1, 2009|Cleveland Clinic Journal of Medicine
Ribhi Hazin, MD;Tarek I. Abu-Rajab Tamimi, MD;Jamily Y. Abuzetun, MD;Nizar N. Zein, MD, FAASLD
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Ribhi Hazin, MD
Harvard University, Faculty of Arts & Sciences, Cambridge, MA

Tarek I. Abu-Rajab Tamimi, MD
Department of Gastroenterology and Hepatology, Cleveland Clinic

Jamily Y. Abuzetun, MD
Department of Internal Medicine, Creighton University Medical Center, Omaha, NE

Nizar N. Zein, MD
Mikati Foundation Endowed Chair in Liver Diseases; Chief, Section of Hepatology; Medical Director of Liver Transplantation, Department of Gastroenterology and Hepatology, Transplantation Center, Cleveland Clinic

Address: Nizar N. Zein, MD, Department of Gastroenterology and Hepatology, A30, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH 44195; e-mail zeinn@ccf.org

ABSTRACTCutaneous changes may be the first clue that a patient has liver disease. Recognizing these signs is crucial to diagnosing liver conditions early. Here we describe the spectrum of skin manifestations that may be found in various liver diseases.

KEY POINTS

  • Pruritus due to liver disease is particularly resistant to therapy. Cholestyramine (Questran) 4 g/day, gradually increased to 24 g/day, is one option. If the pruritus does not respond or the patient cannot tolerate cholestyramine, rifampin (Rifadin) can be tried.
  • Spider angiomas, Bier spots, and “paper-money” skin are all superficial vascular problems that may be related to liver disease.
  • Cutaneous lesions often accompany alcoholic cirrhosis and have been detected in up to 43% of people with chronic alcoholism. The combination of spider angiomas, palmar erythema, and Dupuytren contracture is common in alcoholic cirrhosis.
  • Although porphyria cutanea tarda is associated with liver disease in general, recent studies show that patients with hepatitis C are at particularly high risk.

SUPERFICIAL VASCULAR SIGNS

Spider angiomas

Figure 2. Spider angiomas on the neck of an elderly patient with liver failure. Note the characteristic central vessel and symmetrically radiating thin branches.
Spider angiomas, or spider nevi, are collections of dilated blood vessels near the surface of the skin.20 They appear as slightly raised, small, reddish spots from which fine lines radiate outward, giving them a spider-like appearance (Figure 2).21,22

Spider angiomas can occur anywhere on the body, but they occur most often on the face and the trunk.21,23 A key feature is that they disappear when pressure is applied and reappear when pressure is removed.23,24 Biopsy is rarely necessary for diagnosis.

These lesions occur with elevated estrogen levels, such as in cirrhosis, during estrogen therapy, or during pregnancy.25–28 Although spider angiomas are common in pregnant women and in children, adults with spider angiomas deserve a workup for liver dysfunction.29

Given their innocuous nature and asymptomatic course, spider angiomas themselves require no medical treatment.

Bier spots

Bier spots are small, irregularly shaped, hypopigmented patches on the arms and legs. They are likely due to venous stasis associated with functional damage to the small vessels of the skin.30

Since Bier spots are a sign of liver disease, they must be distinguished from true pigmentation disorders. A key distinguishing feature is that Bier spots disappear when pressure is applied. Also, raising the affected limb from a dependent position causes the hypopigmented macules of Bier spots to disappear, which is not the case in true pigmentation disorders.10,30

Paper-money skin

Paper-money skin (or “dollar-paper” markings) describes the condition in which the upper trunk is covered with many randomly scattered, needle-thin superficial capillaries. It often occurs in association with spider angiomas. The name comes from the resemblance the thread-like capillaries have to the finely chopped silk threads in American dollar bills.10,31 The condition is commonly seen in patients with alcoholic cirrhosis and may improve with hemodialysis.31

PALMAR ERYTHEMA

Palmar erythema is a florid, crimson coloration of the palms of the hands and the fingertips. It can occur anywhere on the palm and fingers but is most common on the hypothenar eminence. It can occur in a number of liver conditions but most often with cirrhosis.32 Hepatic compromise, as seen in alcoholic liver disease, disrupts the body’s androgen balance, causing local vasodilation and erythema.32,33 Although the exact mechanism remains unknown, research suggests that prostacyclins and nitric oxide play a role, as both are increased in liver disease.32,33

XANTHELASMA

Xanthelasma—a localized cholesterol deposit beneath the skin and especially beneath the eyelids—is a common manifestation of hypercholesterolemia. Xanthelasma often presents as a painless, yellowish, soft plaque with well-defined borders,34 which may enlarge over the course of weeks.

Several liver diseases can lead to various forms of secondary dyslipoproteinemia.35 The most common dyslipoproteinemias in liver disease are hypertriglyceridemia and low levels of high-density lipoprotein cholesterol, and both of these often accompany fatty liver disease.36 Hypercholesterolemia is a common feature of primary biliary cirrhosis and other forms of cholestatic liver disease.37 Studies suggest that the total plasma cholesterol level is elevated in as many as 50% of patients with compromised liver function.38

Treatment. The underlying hyperlipidemia is treated with cholesterol-lowering drugs. Laser treatment and surgical excision have proven efficacious in treating the lesions.39

OTHER CUTANEOUS FINDINGS IN LIVER DISEASE

Bleeding and bruising. Liver disease can cause hypersplenism and thrombocytopenia, in addition to a decrease in clotting factors. These may present with a myriad of cutaneous symptoms, including purpura, bleeding gums, and easy bruising and bleeding, even from minor trauma.40–42

Hyperpigmentation of the skin may accompany hemochromatosis, alcoholic liver disease, and cirrhosis.43–45

Figure 3. Onycholysis in a patient with liver disease exhibiting characteristic separation of the nail plate distally.
Hair and nail loss. Patients with hepatocellular dysfunction may develop hair-thinning or hair loss and nail changes such as clubbing, leukonychia (whitening), or onycholysis, affecting the nails of the hands and feet (Figure 3).46,47

“Terry’s nails,” in which the proximal two-thirds of the nail plate turns powdery white with a ground-glass opacity, may develop in patients with advanced cirrhosis.48

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