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lindagray

Scleroderma : Differential Diagnosis 1

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Localized Scleroderma and Scleroderma-Like Disorders

Morphea, or localized scleroderma, can affect all ages and is more common in women.  It typically presents as patches of yellowish or ivory-colored rigid, dry skin. These are followed by the appearance of firm, hard, oval-shaped plaques with ivory centers that are encircled by a violet ring. These spots generally appear on the trunk, face, and/or extremities. Many patients with localized morphea improve without treatment. Generalized morphea is more rare and serious and involves the skin but not the internal organs.

Linear scleroderma appears as a band-like thickening of skin on the arms or legs. This type of scleroderma is most likely to be on one side of the body but may be on both sides. Linear scleroderma generally appears before age 20.  When it occurs in young children, it may result in the failure of one limb (e.g., an arm or leg) to grow as rapidly as its counterpart.

Diffuse fasciitis with eosinophilia (DFE, also called eosinophilic fasciitis or Shulman’s syndrome) is a rare condition that mimics scleroderma with swelling, stiffness, and decreased flexibility of the limbs associated with skin thickening. Although the symptoms can be widespread and involve the trunk and limbs, in contrast to scleroderma, the fingers, hands, and face are usually not affected. In addition, there is no occurrence of Raynaud’s or GI involvement.

Eosinophilia-myalgia syndrome (EMS) is a rare condition that was first described after 3 patients in New Mexico were found to have an illness with significant myalgia (muscle pain) and an increase in the number of eosinophils (a type of white blood cell).  All three patients had taken supplements containing L-tryptophan, which may have been contaminated.  All told, about 1500 people were affected.  A similar outbreak occurred in Spain in 1981 and affected almost 20,000 people.  As it may have been the result of consuming contaminated rapeseed oil, it was known as toxic oil syndrome (TOS).  About 60% of the patients developed skin thickening that look like skin changes typical for scleroderma patients, although the affected areas were different than what is normally seen with scleroderma and there is no associated Raynaud’s phenomenon.

Scleroderma-like skin changes have also been associated with insulin-dependent diabetes, carcinoid syndrome, myeloma, scleromyxedema, chronic graft-versus-host disease, porphyria cutanea tarda, Werner’s syndrome, progeria, phenylketonuria, bleomycin exposure, local lipodystrophies, nephrogenic fibrosing dermopathy, and POEMS syndrome.

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