Xanthomas

 Xanthelasma, eruptive, tuberous, tendinous, planar, plexiform

Etiology/Pathogenesis

• Associated with hereditary lipoproteinemias and occasionally secondary lipoproteinemias
• May also occur in normolipemic patients
image Particularly plexiform xanthoma

Clinical Issues

• Wide age range (children or adults)
• Usually occur in skin and subcutaneous tissue
• Occasionally arise in deep soft tissues (tendon, synovium, bone)
• Classified based on clinical features and gross appearance
• Treatment: Medical therapy or conservative excision
• Excellent prognosis

Microscopic

• Specific classification requires clinicopathologic correlation
• Sheets and aggregates of foamy macrophages
• Variable secondary changes including inflammation, giant cells, fibrosis, and cholesterol cleft formation
• Plexiform xanthoma features multinodular or plexiform growth pattern

Top Differential Diagnoses

• Localized-type tenosynovial giant cell tumor (giant cell tumor of tendon sheath)
• Solitary (juvenile) xanthogranuloma
• Lipidized-type dermatofibroma (fibrous histiocytoma)
• Verruciform xanthoma
• Plexiform fibrohistiocytic tumor
image
XanthelasmaXanthelasmas (and planar xanthomas) consist of a sheet-like infiltrate of foamy macrophages involving the dermis and surrounding adnexal structures. Small areas of chronic inflammation image may be present, but fibrosis and cholesterol clefts are not typical.

image
Eruptive XanthomaEruptive xanthoma is characterized by sheets of macrophages within the dermis. Extravascular lipid deposits with a blue-gray amorphous appearance are often seen image between dermal collagen bundles. A sparse perivascular inflammatory infiltrate image can also be seen.
image
Early Eruptive XanthomaIn contrast to other forms of xanthoma, the cells in eruptive xanthoma are nonfoamy in early lesions. Note the rare foamy macrophages image and extravascular lipid image.
image
Older Eruptive XanthomaOlder eruptive xanthomas accumulate more foamy macrophages image and consist of a mixture of foamy and nonfoamy cells. Again, note areas of lace-like blue-gray extravascular lipid between the dermal collagen bundles image.

TERMINOLOGY

Synonyms

• Plexiform xanthomatous tumor (for plexiform xanthoma)

Definitions

• Reactive mass-forming collection of lipidized macrophages, usually resulting from altered serum lipid levels
image Several forms
– Xanthelasma
– Eruptive xanthoma
– Tuberous xanthoma
– Tendinous xanthoma
– Planar xanthoma
– Plexiform xanthoma

ETIOLOGY/PATHOGENESIS

Hereditary or Nonhereditary

• Associated with hereditary lipoproteinemias and occasionally secondary lipoproteinemias (e.g., diabetes, hypothyroidism, primary biliary cirrhosis)
• May also occur in normolipemic patients
image Particularly plexiform xanthoma

CLINICAL ISSUES

Epidemiology

• Age
image Wide age range (children or adults)

Presentation

• Usually occur in skin and subcutaneous tissue
• Occasionally arise in deep soft tissues (tendon, synovium, bone)
• Classified based on clinical features
image Xanthelasma
– Soft yellow plaques
– Predilection for eyelids and periorbital skin; often bilateral
image Eruptive xanthoma
– Sudden onset of small, yellow papules with erythematous halo
– Predilection for gluteal region, thigh, and shoulders
image Tuberous xanthoma
– Firm yellow subcutaneous nodules and plaques
– Predilection for elbow, knee, gluteal region, and fingers
image Tendinous xanthoma
– Soft tissue mass associated with tendons, ligaments, &/or fascia; predilection for hands, feet, and Achilles tendon

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Apr 24, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Xanthomas

Full access? Get Clinical Tree

Get Clinical Tree app for offline access