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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?

 
 
 

Name the various types of amyloidosis.

Amyloidosis may be classified according to clinical presentation and type of amyloid fibril protein deposition (Table 16-1). The amyloid in the macular and lichenoid variants is derived from degenerated tonofilaments of keratinocytes. Nodular amyloidosis is formed from light-chain–derived AL protein produced locally by plasma cells. It cannot be distinguished from primary systemic amyloidosis, and therefore, systemic disease should be excluded in all patients with nodular amyloidosis. There are also rare forms of hereditary systemic amyloidoses that have less frequent skin manifestations.





Table 16-1. Classification of Amyloidosis
CLINICAL DISORDER AMYLOID PROTEIN PRECURSOR AMYLOID PROTEIN
 
Primary systemic amyloidosis
Immunoglobulin light chain
  AL
 
Myeloma-associated amyloidosis
Immunoglobulin light chain
  AL
 
Secondary systemic amyloidosis
Serum amyloid A lipoprotein
  AA
 
Primary localized cutaneous amyloidosis
   
 
Macular amyloidosis
Keratinocyte tonofilaments
  -
 
Lichen amyloidosis
Keratinocyte tonofilaments
  -
 
Nodular amyloidosis
Immunoglobulin light chain
(produced locally by plasma cells)
  AL