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Paraneoplastic Scleroderma-Like Tissue Reactions in the Setting of an Underlying Plasma Cell Dyscrasia: A Report of 10 Cases

Magro, Cynthia M. MD*; Iwenofu, Hans MD; Nuovo, Gerard J. MD

American Journal of Dermatopathology: July 2013 - Volume 35 - Issue 5 - p 561–568
doi: 10.1097/DAD.0b013e31827adbc8
Original Study

Introduction: Systemic plasma cell dyscrasias have diverse manifestations in the skin and include an inflammatory paraneoplastic process. We encountered cases of scleroderma and eosinophilic fasciitis in the setting of an underlying plasma cell dyscrasia.

Materials and Methods: Ten cases of scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia were encountered. The biopsies were stained for Transforming growth factor (Transforming growth factor) beta, IgG4, kappa, and lambda.

Results: Patients presented with a sclerodermoid reaction represented by eosinophilic fasciitis (5 cases), morphea (3 cases), and systemic scleroderma (2 cases). The mean age of presentation was 70 years with a striking female predominance (4:1). Acral accentuation was noted in 8 cases. In 6 of the cases, the cutaneous sclerosis antedated (4 cases) by weeks to 2 years or occurred concurrently (2 cases) with the initial diagnosis of the plasma cell. The biopsies showed changes typical of eosinophilic fasciitis and/or scleroderma. In 5 cases, light chain–restricted plasma cells were present on the biopsy. There was staining of the plasma cells for Transforming growth factor beta in 3 out of 5 cases tested.

Conclusions: In any older patient presenting with a sudden onset of eosinophilic fasciitis or scleroderma especially with acral accentuation, investigations should be conducted in regards to an underlying plasma cell dyscrasia.

*Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY

Department of Pathology, The Ohio State University, Columbus, OH.

Reprints: Cynthia M. Magro, MD, Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, Room F-309, 1300 York Avenue, Box 58, New York, NY 10065 (e-mail: cym2003@med.cornell.edu).

The authors have no funding or conflicts of interest to declare.

© 2013 by Lippincott Williams & Wilkins.