Definition

Pruritus is itching, and cholestasis is a medical term that describes a disruption in the bile flow produced by hepatocytes, or "liver cells." Cholestasis has a variety of causes, which can be internal or external to the liver. Medications like erythromycin, steroids, oral contraceptives, and phenothiazines can trigger cholestasis. Itching may occur in 20-25% of cases with cholestasis.

Pruritus is a frequent and disturbing symptom in 80-100% of patients with cholestatic liver disease, which includes primary biliary cholangitis, primary sclerosing cholangitis, and pregnancy-related intrahepatic cholestasis. In cholestatic pruritus, there may be no visible primary skin lesion, but secondary skin abnormalities are caused by scratching to relieve itching.

Causes

The exact cause of cholestatic pruritus is still unknown. Bile salts, bile acids, and bilirubin buildup are thought to play a role in cholestatic pruritus development. However, some cases of cholestatic pruritus do not show elevated levels of bile salts and bile acids. As a result, it is suspected that other triggers may cause cholestatic pruritus.

Some potential triggers of cholestatic pruritus include endogenous opioids, serotonin, elevated levels of lysophosphatidic acid, and autotaxin enzymes.

Risk factor

One of the risk factors for developing cholestatic pruritus is being female. Women account for 90% of all cases of primary biliary cirrhosis, which causes cholestatic pruritus. This disease is thought to be an autoimmune disease that affects the small and medium-sized bile ducts, causing cholestasis. It mostly occurs in women aged 40 years and above.

Symptoms

The symptoms of cholestatic pruritus generally vary, and the itching can be persistent or intermittent, all over the body or in specific areas of the body. Severe itching may occur at night and is typically found on the hands, feet, or in areas that are not covered by clothing.

Diagnosis

Cholestatic pruritus is typically diagnosed through a medical interview, physical examination, and additional tests as needed.

During the medical interview, the doctor will inquire about the nature of your itching, the severity of it, when it occurs, and how long it has persisted. When you visit the doctor, you must disclose any itching symptoms. Furthermore, any history of diseases that you are currently experiencing or have previously experienced must be disclosed to the doctor. In the case of cholestatic pruritus, some liver-related diseases can be the key in the medical interview.

Physical examination may reveal some signs of liver disease, such as jaundice (yellow skin), spider angioma (spider-like lines in the abdominal area), white nails, gynecomastia (enlarged male breasts), xanthelasma, splenomegaly (enlarged spleen), and ascites (accumulation of fluid in the area around the abdomen).

The doctor may perform an alkaline phosphatase and direct or indirect bilirubin test as part of the supporting examination. An increase in both parameters indicates cholestasis.  If both parameters are elevated, the doctor may also order anti-hepatitis C and antimitochondrial antibody tests to confirm the diagnosis of primary biliary cirrhosis and hepatitis C, if cholestasis is suspected. A positive antimitochondrial antibody indicates a 98% risk of primary biliary cirrhosis. Other supporting tests may also be performed to look for other causes of cholestasis if the above parameters are found to be negative in the tests.

Management

The management, treatment, and therapy for cholestatic pruritus vary. According to reports, cholestyramine is the primary drug therapy for cholestatic pruritus. In addition, several other drugs can be used to treat cholestatic pruritus.

However, some temporary treatments for cholestatic pruritus can be performed at home. Some treatments include applying cold compresses to the itchy area, using anti-histamine ointments or over-the-counter itch relievers, and, most importantly, avoiding the itch cycle - scratching as little as possible because scratching can aggravate the itching. Then, using moisturizers at least twice a day, just after showers, is another option for relieving itching at home.

Surgical treatment is sometimes an option if the cause of pruritus is associated with obstructive malignancies in the bile duct or other obstructive diseases. The doctor will recommend placing a stent to widen the obstruction, which may also alleviate the itching. Liver transplantation may also be considered if itching caused by benign cholestasis does not respond to medication.

Complications

Complications may vary. Sleeplessness and even suicidal ideation can be notable complications in some patients with severe itching. Women with poorly managed intrahepatic cholestasis during pregnancy and gestational age of fewer than 33 weeks are at risk of preterm labor.

Other complications that can occur include chronic lichen simplex and prurigo nodularis, which are brown-black pustules caused by long-term scratching, and excoriation, or “scratching marks”, which can lead to infection if wounds on the skin area with cholestatic pruritus.

Prevention

The following steps can be taken to help prevent cholestatic pruritis and its potential complications:

• id using cleansers with a high pH, such as cleansers that contain alcohol
• Use moisturizer at least twice a day after showers to maintain the moisture content on the surface of the skin so that it does not itch easily
• Avoid scratching the itchy skin area because scratching can cause an inflammatory process in the skin
• Keep your fingernails short to reduce the risk of scratching wounds

When to see a doctor?

If you experience persistent itching accompanied by a decrease in your quality of life, such as difficulty sleeping or performing usual activities as a result of itching caused by cholestatic pruritus, you should see a specialist in dermatology and venereology at the nearest healthcare facility for further evaluation. The doctor will conduct a medical interview, physical examination, and other tests to determine the exact diagnosis and appropriate management.

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