« Back to Inflammatory Disorders

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?

Name the other organ systems that may be involved in primary or myeloma-associated amyloidosis.

Mucous membrane involvement with macroglossia occurs in 20% of cases. Hepatomegaly is found in about 50% of cases. Cardiac involvement may manifest as a restrictive cardiomyopathy or constrictive pericarditis. Peripheral nerve involvement results in paresthesias, peripheral neuropathy, and median nerve entrapment (carpal tunnel syndrome). Proteinuria is found in 80% to 90% of patients at some time during their course. Renal failure usually develops late in the disease course but may be a cause of death.

Prokaeva T, Spencer B, Kaut M, et al: Soft tissue, joint, and bone manifestations of AL amyloidosis: clinical presentation, molecular features, and survival, Arthritis Rheum 56:3858–3868, 2007.

Silverstein SR: Primary, systemic amyloidosis and the dermatologist: where classic skin lesions may provide the clue for early diagnosis, Dermatol Online J 11:5, 2005.