TAILIEUCHUNG - Chapter 055. Immunologically Mediated Skin Diseases (Part 10)

Scleroderma often eventuates in development of an expressionless, masklike facies. Morphea is characterized by localized thickening and sclerosis of skin, usually affecting young adults or children. Morphea begins as erythematous or flesh-colored plaques that become sclerotic, develop central hypopigmentation, and demonstrate an erythematous border. In most cases, patients have one or a few lesions, and the disease is termed localized morphea. In some patients, widespread cutaneous lesions may occur, without systemic involvement. This form is called generalized morphea. Most patients with morphea do not have autoantibodies. Skin biopsy of morphea is indistinguishable from that of scleroderma. Linear scleroderma is a limited. | Chapter 055. Immunologically Mediated Skin Diseases Part 10 Scleroderma often eventuates in development of an expressionless masklike facies. Morphea is characterized by localized thickening and sclerosis of skin usually affecting young adults or children. Morphea begins as erythematous or flesh-colored plaques that become sclerotic develop central hypopigmentation and demonstrate an erythematous border. In most cases patients have one or a few lesions and the disease is termed localized morphea. In some patients widespread cutaneous lesions may occur without systemic involvement. This form is called generalized morphea. Most patients with morphea do not have autoantibodies. Skin biopsy of morphea is indistinguishable from that of scleroderma. Linear scleroderma is a limited form of disease that presents in a linear bandlike distribution and tends to involve deep as well as superficial layers of skin. Scleroderma and morphea are usually quite resistant to therapy. For this reason physical therapy to prevent joint contractures and to maintain function is employed and is often helpful. Diffuse fasciitis with eosinophilia is a clinical entity that can sometimes be confused with scleroderma. There is usually the sudden onset of swelling induration and erythema of the extremities frequently following significant physical exertion. The proximal portions of extremities arms forearms thighs legs are more often involved than are the hands and feet. While the skin is indurated it is usually not bound down as in scleroderma contractures may occur early secondary to fascial involvement. The latter may also cause muscle groups to be separated and veins to appear depressed. These skin findings are accompanied by peripheral blood eosinophilia increased erythrocyte sedimentation rate and sometimes hypergammaglobulinemia. Deep biopsy of affected areas of skin reveals inflammation and thickening of the deep fascia overlying muscle. An inflammatory infiltrate composed of eosinophils

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