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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?

 
 
 

Compare lichen amyloidosis and macular amyloidosis.


A, Lichen amyloidosis. Numerous pruritic, scaly papules on the anterior shin. B, Nodular amyloidosis demonstrating large waxy nodule on the nose.
Fig. 16.3 A, Lichen amyloidosis. Numerous pruritic, scaly papules on the anterior shin. B, Nodular amyloidosis demonstrating large waxy nodule on the nose.
Lichen amyloidosis is the most common form of localized cutaneous amyloidosis. Lesions are pruritic, flesh-colored to brown papules, often with overlying scale (Fig. 16-3A). Papules may coalesce into verrucous plaques. The shins are the most common site of involvement. In macular amyloidosis, pruritic macular hyperpigmentation occurs most commonly in the interscapular area. The chest or extremities are less commonly involved. The lesions have a characteristic reticulate or rippled appearance. Both of these variants of primary localized cutaneous amyloidosis occur more frequently in patients from the Middle East, Asia, and Central and South America. The etiology of both lichen and macular amyloidosis is unclear but thought to be related to chronic scratching or frictional exposure. An autosomal dominant family history may be found in up to 10% of patients with lichen amyloidosis. Lichen amyloidosis is occasionally associated with multiple endocrine neoplasia type 2A.

Tanaka A, Arita K, Lai-Cheong JE, et al: New insight into mechanisms of pruritus from molecular studies on familial primary localized cutaneous amyloidosis, Br J Dermatol 161:1217–1224, 2009.