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Deposition Disorders

»How is “deposition disorder” defined?
»What is amyloid?
»How is amyloid identified?
»Name the various types of amyloidosis.
»What are the cutaneous manifestations of primary or myeloma-associated systemic amyloidosis? How often do they occur?
»Name the other organ systems that may be involved in primary or myeloma-associated amyloidosis.
»Compare lichen amyloidosis and macular amyloidosis.
»How does nodular amyloidosis present? With what is it associated?
»In what setting is secondary systemic amyloidosis seen?
»What are the systemic manifestations of secondary systemic amyloidosis?
»What is lipoid proteinosis?
»What is colloid milium?
»Which histologic feature or “deposit” is common to all porphyrias?
»Which porphyria classically demonstrates the largest deposits? What are its cutaneous features?
»Name some of the cutaneous mucinoses.
»Describe the clinical lesions seen in pretibial myxedema and its disease associations.
»Describe the clinical lesions seen in lichen myxedematosus.
»What serum abnormality has been associated with scleromyxedema?
»Describe the clinical lesions in scleredema and its disease associations.
»What is a digital mucous (myxoid) cyst?
»What substance is elevated in gout?
»Where is the uric acid deposited in gout? What are the resulting clinical manifestations?
»How is gout treated?
»How many types of calcinosis cutis are there?
»What underlying medical conditions have been associated with metastatic calcinosis cutis?
»What is calciphylaxis and who develops it?
»What is osteoma cutis?

 
 
 

Where is the uric acid deposited in gout? What are the resulting clinical manifestations?


A, Gouty tophi. Tophaceous deposits of gout overlying digits. B, Aspirate from gouty tophus demonstrating diagnostic birefringent gout crystals with polarization. (Courtesy of the Fitzsimons Army Medical Center teaching files.)
Fig. 16.5 A, Gouty tophi. Tophaceous deposits of gout overlying digits. B, Aspirate from gouty tophus demonstrating diagnostic birefringent gout crystals with polarization. (Courtesy of the Fitzsimons Army Medical Center teaching files.)
Uric acid crystals in gout are most commonly deposited in the synovium, soft tissues, and skin. The most common site is the synovium of joints, producing acute gouty arthritis. The metatarsophalangeal joint of the great toe is classically involved. Uric acid deposition in the skin and soft tissues results in gouty tophi, which are seen in 20% to 50% of patients. Common sites of involvement include the helix of the ear, elbows, and digits (Fig. 16-5A). These gouty tophi may ulcerate and discharge monosodium urate crystals that appear as a thick chalky material. Under light microscopy, these crystals are needle-shaped and birefringent (Fig. 16-5B).

Thissen CA, Frank J, Lucker GP: Tophi as first clinical sign of gout, Int J Dermatol 47(Suppl 1):49–51, 2008.