Cutaneous deposition diseases. Part II,☆☆,

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Abstract

Part II of the cutaneous deposition disorders focuses on cutaneous calcification and ossification, alkaptonuria and ochronosis, and gout. These disorders have in common the deposition of materials in the dermis or subcutis and often involve metabolic defects in hormonal and enzymatic regulation. The pathogenesis, clinical findings, and treatment of these diseases are discussed. Both the histologic and ultrastructural findings are reviewed. (J Am Acad Dermatol 1998;39:527-44.)

Learning Objective: After reading this review, the practitioner should be familiar with both the cutaneous and systemic manifestations of these disorders.

Section snippets

CUTANEOUS CALCIFICATION

Cutaneous deposition of calcium salts (calcinosis cutis) in the skin and subcutaneous tissue occurs in a variety of clinical settings. It begins as a calcium phosphate nidus and progresses to hydroxyapatite crystal formation within a collagen matrix. Four subsets occur: metastatic calcinosis, dystrophic calcinosis, idiopathic calcinosis, and iatrogenic calcification.

CUTANEOUS OSSIFICATION

Cutaneous ossification (osteoma cutis) involves bone deposition in the skin via osteoblastic organization of new type I collagen fibers.1 Both membranous and enchondral bone formation may be seen.34 It usually occurs in the connective tissue adjacent to a preexisting neoplasm.1 Less commonly it occurs as a result of inflammatory or metabolic disease, or in calcified tissue. Primary osteoma cutis occurs within normal tissue. All are termed metaplastic ossification. Osteoma cutis is also a

ALKAPTONURIA AND OCHRONOSIS

Alkaptonuria is a rare autosomal recessive disorder of homogentisic acid (HGA) catabolism. Its incidence is estimated at 1 in 1 million births,68 with a higher frequency in Eastern Europe and Santo Domingo.69 The human gene responsible for alkaptonuria has recently been localized to chromosome 3q70, 71 but has not been mapped or cloned. The underlying defect is a lack of renal and hepatic HGA oxidase (HGAO) activity.

EXOGENOUS OCHRONOSIS

Exogenous ochronosis refers to focal darkening of the skin most commonly seen after hydroquinone application to lighten the skin (Fig 11).

. Exogenous ochronosis resulting from hydroquinone use.

It typically occurs in dark-skinned patients who use high-strength preparations, but even over-the-counter low concentrations (<2%) may result in this paradoxical darkening of the skin.110 Hydroquinone is available in concentrations of 2% to 10%.111 The hyperpigmentation generally occurs after 6 months of

GOUT

Gout is a metabolic disease resulting from tissue deposition of monosodium urate crystals from supersaturated extracellular fluids. The clinical manifestations include acute gouty arthritis, aggregates of crystal in connective tissues (tophi), urate urolithiasis, and, rarely, gouty nephropathy. The cutaneous manifestations of gout will be discussed. They occur in patients with acute gouty arthritis or tophaceous deposits.

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    The opinions and assertions contained herein are the private views of the authors and not to be construed as official or as reflecting the views of the US Army or the Department of Defense.

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    Reprints are not available from the authors.

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