April 28, 2007

Connective Tissue Disorders

By Babin, Lynn A

Dermatology Nursing Essentials: A Core Curriculum (2nd edition) was written to provde the reader with core knowledge about the specialty of dermatology nursing. Key points from the text are highlighted to test and review your dermatology nursing knowledge. These quick hits of information are no substitute for the indepth analysis provided in the Core Curriculum, but are designed to whet your appetite for the comprehensive content that the Core delivers. References for the information presented here can be found in the Core Curriculum. To order your personal copy of the Core, visit DNA's Web site at www.dnanurse.org or call (800) 454-4DNA.

* Connective tissue disorders (collagen vascular disease) are defined as a group of acquired disorders that have the commonality of immunologic and inflammatory changes in small blood vessels and connective tissue.

* Common features include arthritis, skin lesions, iritis and episcleritis, pericarditis, pleuritis, subcutaneous nodules, myocarditis, vasculitis, and nephritis.

* Lupus is a chronic autoimmune disease resulting in inflammation and tissue damage.

* The exact cause is unknown; however, current research points to interrelated immunologic, environmental, hormonal, and genetic factors.

* Lupus strikes women 10 to 15 times more frequently than men, and occurs most often during childbearing years (see Figure 1).

* Types include cutaneous lupus erythematosus, systemic lupus erythematosus, drug-induced lupus erythematosus, and neonatal lupus erythematosus.

* Diagnosis includes a complete history and physical, including a full-skin assessment. Currently, there is no single definitive laboratory test for lupus.

* Prevention is key to minimizing symptoms, reducing inflammation, and maintaining normal bodily functions.

* Scleroderma (systemic sclerosis) is a chronic, often progressive autoimmune disease, which can cause thickening and tightening of the skin by excessive collagen production.

* Scleroderma varies widely in severity; in some cases, internal organs such as lungs, heart, kidneys, esophagus, and GI tract are seriously damaged (see Figures 2-3).

* The exact cause(s) of scleroderma is unknown, but research is pointing to a "susceptible gene."

* Classifications of scleroderma include localized scleroderma, systemic scleroderma (also called progressive systemic sclerosis), and scleroderma-related disorders.

* Localized scleroderma affects the collagen-producing cells in limited skin areas and usually spares the internal organs and blood vessels.

* In systemic scleroderma, the immune system causes damage to small blood vessels and the collagen-producing cells throughout the body.

* Scleroderma-related disorders occur in patients with other autoimmune disorders and may be referred to as an "overlap syndrome."

* Diagnosis includes a detailed history of signs/symptoms during general systems review that prompted medical services which may indicate progression and severity of disease process.

* There is no cure for scleroderma. Treatment plans are individualized to each patient's type and severity of symptoms.

* Inflammatory myopathies are a group of muscle diseases characterized by inflammation of connective tissue and muscle fibers which can cause extensive necrosis and destruction of the muscle fibers.

* The inflammatory myopathies are dermatomyositis, juvenile dermatomyositis, polymyositis, and inclusion body myositis. Each is unique in its development and treatment.

* Inflammatory myopathies are designated as autoimmune diseases because of the presence of autoantibodies in the serum of those affected.

* There is no known cure for inflammatory myopathies.

* High doses of immunosuppressants and/or steroids have been effective for many patients.

Lynn A. Babin, MSN, RN, CNS, AAS, is an Instructor/Clinical Nurse Specialist, Cincinnati State Technical and Community College, Cincinnati, OH.

Copyright Anthony J. Jannetti, Inc. Apr 2007

(c) 2007 Dermatology Nursing. Provided by ProQuest Information and Learning. All rights Reserved.