Xanthoma striatum palmare

A rare type of xanthoma associated with hyperlipoproteinemia

Classification
External resources

Xanthoma striatum palmare is a rare dermatological condition characterized by the presence of yellowish-orange deposits on the palms of the hands. These deposits are due to the accumulation of lipids, specifically cholesterol and triglycerides, within the skin. Xanthoma striatum palmare is often associated with underlying lipid metabolism disorders, particularly type III hyperlipoproteinemia.

Pathophysiology

Xanthomas are formed when lipids accumulate in the skin, often due to elevated levels of lipoproteins in the blood. In the case of xanthoma striatum palmare, the condition is closely linked to type III hyperlipoproteinemia, also known as dysbetalipoproteinemia. This disorder is characterized by the accumulation of intermediate-density lipoproteins (IDL) due to a defect in the apolipoprotein E (ApoE) receptor binding. The impaired clearance of these lipoproteins leads to their deposition in various tissues, including the skin.

Clinical Presentation

Patients with xanthoma striatum palmare typically present with:

• Yellowish-orange streaks or patches on the palms, particularly along the lines of the skin.
• These lesions are often symmetrical and can vary in size.
• The condition may be accompanied by other types of xanthomas, such as tuberous xanthomas or xanthelasma.

Diagnosis

The diagnosis of xanthoma striatum palmare is primarily clinical, based on the characteristic appearance of the lesions. However, laboratory tests are essential to confirm the underlying lipid disorder. These tests may include:

• Lipid profile to assess levels of cholesterol, triglycerides, and lipoproteins.
• Genetic testing for mutations in the Apolipoprotein E gene.

Management

The management of xanthoma striatum palmare involves treating the underlying lipid disorder. This may include:

• Dietary modifications to reduce cholesterol and triglyceride intake.
• Pharmacological therapy with lipid-lowering agents such as statins, fibrates, or niacin.
• Regular monitoring of lipid levels to assess treatment efficacy.

Prognosis

With appropriate management of the underlying lipid disorder, the prognosis for patients with xanthoma striatum palmare is generally good. The skin lesions may regress with effective lipid control, although this can take several months to years.

Related pages

• Xanthoma
• Hyperlipoproteinemia
• Apolipoprotein E
• Dysbetalipoproteinemia