Skip Navigation LinksHome > July 2013 - Volume 35 - Issue 5 > Paraneoplastic Scleroderma-Like Tissue Reactions in the Sett...
American Journal of Dermatopathology:
doi: 10.1097/DAD.0b013e31827adbc8
Original Study

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

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Abstract

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.

© 2013 Lippincott Williams & Wilkins

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