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Plane xanthomas - Skin
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Plane xanthomas - Skin

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Contributors: Gil Weintraub MD, Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
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Synopsis

Plane xanthomas, or planar xanthomas, are yellow-orange patches or slightly raised papules or plaques that form due to increased lipid deposition.

They are associated with:
  • Elevated serum lipid levels
  • Normal or slightly elevated lipid levels  
Over 50% of cases are due to hyperlipoproteinemia from primary metabolic lipoprotein abnormalities, such as familial hypercholesterolemia (type IIa hyperlipoproteinemia) or familial dysbetalipoproteinemia (type III hyperlipoproteinemia), or secondary causes, such as hepatic cholestasis (eg, primary biliary cirrhosis). The quantitative or qualitative abnormality in serum lipid levels results in aggregation of lipoproteins that are phagocytosed by scavenger macrophages, forming foam cells that coalesce into plane xanthomas.

Normolipemic plane xanthomas, including those associated with paraproteinemias, most commonly multiple myeloma, are less common. Other associated diseases include Waldenström's macroglobulinemia, leukemia, lymphoma, cryoglobulinemia, Sézary syndrome, Castleman's disease, histiocytosis X, exfoliative dermatitis, actinic reticuloid, and photosensitive eczema. It is hypothesized that excess serum proteins bind lipoproteins to form modified lipoprotein complexes that are deposited in the skin and phagocytosed by scavenger macrophages, leading to foam cells and xanthoma formation.

Although plane xanthomas can occur in patients of all ages, they are significantly more common in adults aged 40-60 years. They tend to form along the eyelids, lateral sides of the neck, upper aspects of the trunk, flexural folds, and palms. When seen on the palmar creases of the hands, the condition is classified as palmar xanthoma, which almost exclusively is found in patients with underlying familial dysbetalipoproteinemia.

Recognition of the relationship between plane xanthomas and hyperlipidemia is critical to reducing the risk of atherosclerosis and lipid-related causes of death. In cases of normal serum lipid levels, it is important to regularly screen patients for the development of hematologic and lymphoproliferative disorders, as xanthomas may precede the onset of associated disease by months to years.

Codes

ICD10CM:
E78.2 – Mixed hyperlipidemia

SNOMEDCT:
238953008 – Plane Xanthoma

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Differential Diagnosis & Pitfalls

  • Amyloidosis – Nonpalpable periorbital purpura or smooth, waxy infiltration of palms and volar fingertips; strong association with blood dyscrasias.
  • Diffuse cutaneous mastocytosis – Orange-colored, rough, indurated, leathery skin.
  • Lipoid proteinosis – Thickened skin and mucous membrane with yellow nodules appearing on the face and yellow and waxy papules or plaques diffusely spread across the face, axilla, and scrotum; develops during infancy; persistently hoarse voice.
  • Necrobiotic xanthogranuloma – Reddish-yellow plaques and nodules differentiated by the presence of skin atrophy, ulcerations, and ophthalmic involvement; majority found in older adults and associated with paraproteinemia.
  • Pseudoxanthoma elasticum – Abnormal deposition of calcium on elastic fibers; yellow papules in redundant skin folds and flexural areas such as the neck, axillae, antecubital fossae, and inguinal area; skin manifestations develop in childhood; skin becomes lax.
  • Psoriasis – Well-demarcated silver-scaled plaques; may appear on extensors or intertriginous areas; pinpoint bleeding (Auspitz sign).
  • Sebaceous hyperplasia – Small yellow papules with central umbilication, usually around the face; lesions often have small dilated vessels visible around a rim of yellow.

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Last Updated: 02/04/2016
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Plane xanthomas - Skin
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Plane xanthomas : Flexural, Hypercholesterolemia, Intertriginous, Widespread, Smooth papules, Smooth plaques
Clinical image of Plane xanthomas
A close-up of confluent yellowish papules forming plaques.
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