Selected skin diseases with systemic involvement. 2013

Marcelo Ruiz, and Pilar Valdés, and Kenneth Tomecki
Department of Dermatology, Hospital Clínico Universidad de Chile, Santiago, Chile; Department of Dermatology, Cleveland Clinic, Cleveland, OH, USA.

The skin is often a window to systemic disease that is available to the trained eye of the dermatologist. Herein, we focus on four dermatoses with associated systemic conditions of interest: scleromyxedema and monoclonal gammopathy, nephrogenic systemic fibrosis in the setting of renal insufficiency, dermatitis herpetiformis and celiac disease, and psoriasis as a risk factor for cardiovascular disease. Dermatologists can play a crucial role in recognizing the cutaneous manifestations linked with these conditions. Identifying the related underlying disorder will contribute to appropriate diagnosis and improved management.

UI MeSH Term Description Entries
D010265 Paraproteinemias A group of related diseases characterized by an unbalanced or disproportionate proliferation of immunoglobulin-producing cells, usually from a single clone. These cells frequently secrete a structurally homogeneous immunoglobulin (M-component) and/or an abnormal immunoglobulin. Gammapathy, Monoclonal,Gammopathy, Monoclonal,Monoclonal Gammopathies,Paraimmunoglobulinemia,Paraimmunoglobulinemias,Paraproteinemia,Plasma Cell Dyscrasias,Monoclonal Gammapathies,Monoclonal Gammopathy,Cell Dyscrasia, Plasma,Dyscrasia, Plasma Cell,Monoclonal Gammapathy,Plasma Cell Dyscrasia
D011565 Psoriasis A common genetically determined, chronic, inflammatory skin disease characterized by rounded erythematous, dry, scaling patches. The lesions have a predilection for nails, scalp, genitalia, extensor surfaces, and the lumbosacral region. Accelerated epidermopoiesis is considered to be the fundamental pathologic feature in psoriasis. Palmoplantaris Pustulosis,Pustular Psoriasis of Palms and Soles,Pustulosis Palmaris et Plantaris,Pustulosis of Palms and Soles,Psoriases
D002318 Cardiovascular Diseases Pathological conditions involving the CARDIOVASCULAR SYSTEM including the HEART; the BLOOD VESSELS; or the PERICARDIUM. Adverse Cardiac Event,Cardiac Events,Major Adverse Cardiac Events,Adverse Cardiac Events,Cardiac Event,Cardiac Event, Adverse,Cardiac Events, Adverse,Cardiovascular Disease,Disease, Cardiovascular,Event, Cardiac
D002446 Celiac Disease A malabsorption syndrome that is precipitated by the ingestion of foods containing GLUTEN, such as wheat, rye, and barley. It is characterized by INFLAMMATION of the SMALL INTESTINE, loss of MICROVILLI structure, failed INTESTINAL ABSORPTION, and MALNUTRITION. Gluten Enteropathy,Sprue, Celiac,Sprue, Nontropical,Celiac Sprue,Gluten-Sensitive Enteropathy,Sprue,Disease, Celiac,Enteropathies, Gluten,Enteropathies, Gluten-Sensitive,Enteropathy, Gluten,Enteropathy, Gluten-Sensitive,Gluten Enteropathies,Gluten Sensitive Enteropathy,Gluten-Sensitive Enteropathies,Nontropical Sprue
D003874 Dermatitis Herpetiformis Rare, chronic, papulo-vesicular disease characterized by an intensely pruritic eruption consisting of various combinations of symmetrical, erythematous, papular, vesicular, or bullous lesions. The disease is strongly associated with the presence of HLA-B8 and HLA-DR3 antigens. A variety of different autoantibodies has been detected in small numbers in patients with dermatitis herpetiformis. Duhring's Disease,Duhring Disease,Disease, Duhring,Disease, Duhring's,Duhrings Disease
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D051437 Renal Insufficiency Conditions in which the KIDNEYS perform below the normal level in the ability to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of PROTEINURIA) and reduction in GLOMERULAR FILTRATION RATE. Kidney Insufficiency,Kidney Failure,Renal Failure,Failure, Kidney,Failure, Renal,Failures, Kidney,Failures, Renal,Insufficiency, Kidney,Kidney Failures,Kidney Insufficiencies,Renal Failures,Renal Insufficiencies
D053718 Scleromyxedema A connective tissue disorder characterized by widespread thickening of SKIN with a cobblestone-like appearance. It is caused by proliferation of FIBROBLASTS and deposition of MUCIN in the DERMIS in the absence of thyroid disease. Most scleromyxedema cases are associated with a MONOCLONAL GAMMOPATHY, immunoglobulin IgG-lambda. Lichen Myxedematosus,Papular Mucinosis,Mucinosis, Papular,Myxedematosus, Lichen
D054989 Nephrogenic Fibrosing Dermopathy A chronic, acquired, idiopathic, progressive eruption of the skin that occurs in the context of RENAL FAILURE. It is sometimes accompanied by systemic fibrosis. The pathogenesis seems to be multifactorial, with postulated involvement of circulating fibrocytes. There is a strong association between this disorder and the use of gadolinium-based contrast agents. Nephrogenic Systemic Fibrosis,Dermopathies, Nephrogenic Fibrosing,Dermopathy, Nephrogenic Fibrosing,Fibroses, Nephrogenic Systemic,Fibrosing Dermopathies, Nephrogenic,Fibrosing Dermopathy, Nephrogenic,Fibrosis, Nephrogenic Systemic,Nephrogenic Fibrosing Dermopathies,Nephrogenic Systemic Fibroses,Systemic Fibroses, Nephrogenic,Systemic Fibrosis, Nephrogenic

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