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Soft tissue Tumors

Fibroblastic / myofibroblastic tumors

Eosinophilic fasciitis


Reviewer: Komal Arora, M.D. (see Reviewers page)
Revised: 20 July 2012, last major update July 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Rare fibrosing disorder with scleroderma-like induration of distal extremities
● Diffuse fasciitis with fibrosis, eosinophilia and inflammation of skin, subcutaneous tissue and fascia
● Not a WHO diagnosis
● First described by Shulman (Trans Assoc Am Physicians 1975;88:70) [also called Shulman’s syndrome]

Epidemiology
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● Mean age 50 years, 75% women
● Eosinophilic-myalgia syndrome is different, and is associated with contaminated L-tryptophan, myalgia and neuritis (Hum Pathol 1992;23:429)
● Shulman's syndrome tends to involve the subcutis alone and the eosinophilia-myalgia syndrome tends to be a pancutaneous-subcutaneous process (eMedicine, J Am Acad Dermatol 1992;26:95)
● Presence of morphea-like skin lesions is associated with residual fibrosis (Clin Rheumatol 2007;26:1445)

Case reports
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● 76 year old woman (Dermatol Online J 2003;9:33)
● Paraneoplastic phenomenon associated with metastatic colorectal carcinoma (Australas J Dermatol 2008;49:27)

Treatment
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● High dose corticosteroids (Int J Dermatol 2008;47:29)

Clinical images
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Symmetric, thick, sclerotic skin with subtle yellow-orange hue on forearms and upper arms

Gross description
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● Woody, firm subcutaneous mass

Micro description
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● Eosinophils, lymphocytes, mast cells and histiocytes in fibrotic or fibromyxoid stroma of subcutis and fascia

Micro images
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Thickening of fascia with chronic inflammatory infiltrate


Inflammatory infiltrate in deep subcutis

Additional references
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eMedicine #1, #2

End of Soft Tissue Tumors > Fibroblastic / myofibroblastic tumors > Eosinophilic fasciitis


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