Recognizing and treating cutaneous signs of liver disease
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.
Lichen planus
Lichen planopilaris is a subset of lichen planus that causes scaling and atrophy of the scalp and permanent hair loss (Figure 4).73
Interferon-induced vitiligo
Vitiligo is an autoimmune disease in which melanocytes in the skin are destroyed, with resulting depigmentation in affected areas.75 Although it has no specific association with liver disease, it has been linked to treatments for hepatitis C such as interferons.76 Interferon-induced vitiligo often completely resolves when interferon is stopped.77
Typical findings include aggregations of irregularly shaped white patches in a focal or segmental pattern.78 The diagnosis is based on the medical history, physical examination, and sometimes skin biopsy.
HEMOCHROMATOSIS
Hemochromatosis or “bronze diabetes” is a devastating multisystem disease with a relentless course. It is among the most common genetic disorders of metabolism, and results in deposition of iron in tissues and organs throughout the body, including the liver, usually in patients ages 30 to 40.
As iron stores increase in tissues and organs, multiorgan failure and associated complications may ensue. In addition, surplus iron stores can also result in widespread bronze discoloration of skin exposed to the sun. Hemochromatosis also results in loss of body hair, ichthyosiform alterations, and koilonychia.79
Treatments that lower serum iron levels can reverse the cutaneous manifestations of the disorder and minimize the risk of organ failure.
Since the condition is inherited in an autosomal-recessive pattern, family members of patients should consider being screened.80
Hyperpigmentation in hemochromatosis
Hyperpigmentation is an early sign of hemochromatosis, affecting up to 90% of patients. Usually, sun-exposed areas of the body are the most prone and take on a grayish or brownish-bronze hue.81 Cutaneous iron deposits injure vital skin structures, initiating a process that culminates in enhanced melanin production by melanocytes.82 Exposure to ultraviolet light may have synergistic effects with iron, hastening the process of hyperpigmentation. As a result of this synergistic effect, many patients with hemochromatosis notice tanning with minimal sun exposure.
Although organ function can improve immediately with phlebotomy to reduce iron stores, skin hyperpigmentation does not immediately resolve.81,82
Ichthyosiform alterations in hemochromatosis
Ichthyosiform changes, in which the skin takes on the appearance of fish scales,83 can be seen in patients with hemochromatosis.80 Affected areas typically become extremely dry. Treatment includes topical hydrating creams and ointments. Avoiding sunlight is paramount, as sunlight exposure may exacerbate the condition.83
