Soft tissue Tumors - Eosinophilic fasciitis

Home Chapter Home Jobs Conferences Fellowships Books

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
=========================================================================

● 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
=========================================================================

● 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
=========================================================================

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

Treatment
=========================================================================

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

Clinical images
=========================================================================

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

Gross description
=========================================================================

● Woody, firm subcutaneous mass

Micro description
=========================================================================

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

Micro images
=========================================================================

Thickening of fascia with chronic inflammatory infiltrate

Inflammatory infiltrate in deep subcutis

Additional references
=========================================================================

● eMedicine #1, #2

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

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).