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Paraproteinemia-Associated Scleredema Treated Successfully With Intravenous Immunoglobulin

A. Brooke Eastham, MD1,2,3; Alisa N. Femia, MD2,3; Nicole F. Velez, MD1,2,3; Hedy P. Smith, MD4; Ruth Ann Vleugels, MD, MPH2,3
[+] Author Affiliations
1Harvard Combined Dermatology Residency Program, Boston, Massachusetts
2Department of Dermatology, Brigham and Women’s Hospital, Boston, Massachusetts
3Harvard Medical School, Boston, Massachusetts
4Division of Hematology, Department of Medicine, Tufts Medical School, Boston, Massachusetts
JAMA Dermatol. 2014;150(7):788-789. doi:10.1001/jamadermatol.2013.8835.
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Scleredema is a fibromucinous connective tissue disease characterized by symmetric, nonpitting edema and induration of the face, neck, and trunk. Although the pathogenesis remains elusive, associations with infection, diabetes mellitus, and paraproteinemia have been established.1 Paraproteinemia-associated scleredema is typically characterized by a progressive course, for which no standard therapeutic protocol exists.2 To our knowledge, the patient described herein represents the first reported case of scleredema with paraproteinemia successfully treated with intravenous immunoglobulin (IVIG).

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Figure.
Limited Range of Motion in a Woman With Scleredema

A, This image demonstrates marked restriction in shoulder abduction before intravenous immunoglobulin (IVIG) therapy. B, This image demonstrates significant improvement in shoulder abduction following IVIG therapy.

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