title: Paraneoplastic scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia: a report of 10 cases.
abstract: BACKGROUND
  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.
  
  
  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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY 10065, USA. cym2003@med.cornell.edu
authors.forename: Cynthia M
authors.initials: CM
authors.lastname: Magro
authors.forename: Hans
authors.initials: H
authors.lastname: Iwenofu
authors.forename: Gerard J
authors.initials: GJ
authors.lastname: Nuovo
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D004802:Eosinophilia / Q000473:pathology; D005208:Fasciitis / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007145:Immunoglobulin kappa-Chains / Q000032:analysis; D007146:Immunoglobulin lambda-Chains / Q000032:analysis; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D010265:Paraproteinemias / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D011379:Prognosis; D012594:Scleroderma, Localized / Q000473:pathology; D012595:Scleroderma, Systemic / Q000378:metabolism / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D012867:Skin / Q000737:chemistry / Q000473:pathology*; D016212:Transforming Growth Factor beta / Q000032:analysis
chemical_list: D014408:Biomarkers, Tumor; D007074:Immunoglobulin G; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D016212:Transforming Growth Factor beta