scleromyxedema/amyloid

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Coexistence of papular mucinosis and systemic amyloidosis associated with lambda-type IgD paraproteinemia.

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The patient is an obese, 55-year-old woman. She noticed purpura at several sites when she was 49 years old. She visited our clinic with a chief complaint of exertional dyspnea at 51 years of age. Physical examination revealed localized edema in the left chest wall and lower abdomen with translucent

Fatal recurrent dermatoneuro syndrome associated with systemic AL amyloidosis.

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A male patient is presented with long-lasting paraproteinemia of monoclonal IgG λ, who suffered from recurrent, and until the last one, mostly reversible episodes of dermatoneuro syndrome, described exclusively in scleromyxedema. The skin biopsy revealed λ-light chain amyloid deposition instead of

Scleromyxedema with corneal deposits.

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Scleromyxedema (Arndt-Gottron syndrome) is a rare cutaneous disease in which hyaluronic acid is deposited in the dermis. The authors describe a patient with scleromyxedema and corneal deposits. A corneal biopsy demonstrated hyaluronic acid deposition in the corneal stroma and amyloid P component in

Monoclonal gammopathy of cutaneous significance: review of a relevant concept.

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Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Amyloidosis, macroglobulinoderma

Skin disorders in association with monoclonal gammopathies.

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Monoclonal gammopathy represents a condition characterized by clonal proliferation and accumulation of immunoglobulin producing B-cells. A variety of skin disorders are associated with an increased level of monoclonal immunoglobulin proteins. These skin disorders can be divided into two groups. The

Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum.

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A variety of cutaneous manifestations has been described in multiple myeloma including extramedullary cutaneous plasmacytomas, cutaneous amyloidosis, pyoderma gangrenosum, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, scleromyxedema, Sweet's syndrome, subcorneal pustular dermatosis,

A case report and differential diagnosis of pruritic pretibial skin lesions

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Pretibial pruritic papular dermatitis (PPPD) is a clinical entity first described in 2006. The etiology is uncertain; however, gentle chronic rubbing is likely to be the reason for the skin reaction. Pretibial pruritic lesions may reflect many different systemic diseases and dermatoses. We present a

Parallel studies on collagen hydroxyproline and hydroxylysine in human skin biopsies.

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Studies on hydroxyproline and hydroxylysine, the two amino acids characteristic of collagen and related glycoproteins, were undertaken on biopsies of pathologic and clinically normal human skin. No statistically significant differences between clinically normal skin of mamma, thorax, axilla, femur,

Lich myxedematosus. A dermal infiltrative disorder responsive to cyclophosphamide therapy.

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Lichen myxedematosus is an uncommon, chronic, cutaneous disease of mucin deposition that has resisted a variety of therapies. The discovery of a unique, basic, homogeneous globulin in the sera of many of the patients with this disorder raised questions concerning the relationships between lichen

Case of suspected connective tissue disease presenting as chronic edema or swelling of face, neck, and upper trunk.

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An adequate biopsy that includes the subcutaneous tissue is very helpful in the evaluation of patients with suspected connective tissue disease. However, the histologic features must often be correlated with the clinical features and other laboratory tests for a definitive diagnosis. An objective

Myeloma, paraproteinemias, and the skin.

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This review focuses on those systemic diseases or syndromes associated with monoclonal plasma cell disorders that may present with important cutaneous manifestations. Amyloidosis, POEMS syndrome, cutaneous plasmacytoma, xanthomas, benign hypergammaglobulinemic purpura of Waldenström, and

Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance.

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Background

Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations.

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