Sjögren's syndrome with specific cutaneous manifestations and multifocal clonal T-cell populations progressing to a cutaneous pleomorphic T-cell lymphoma

Am J Clin Pathol. 1989 Sep;92(3):357-61. doi: 10.1093/ajcp/92.3.357.


A case is presented of primary Sjögren's syndrome of the parotid gland with specific skin manifestations, consisting of diffuse lymphoid infiltrates with proliferative and metaplastic changes of sweat glands, a histologic picture similar to that seen in the parotid gland. Material of the parotid gland, a submandibular lymph node showing expanded T-cell areas, and several skin biopsies were studied immunohistologically for the presence of clonal B-cell populations. Because of a polyclonal kappa/lambda pattern of the B-lymphocytes, the possibility of a B-cell non-Hodgkin's lymphoma (NHL) was excluded. The initially morphologically benign lymphocytic skin infiltrates (1985) changed into a pleomorphic T-cell lymphoma (1987). Southern blot analysis of the atypical skin infiltrates and of the original material of the parotid gland and submandibular lymph node revealed identical clonal rearrangements of the T-cell receptor B-chain. Rearrangement of Ig genes was not found.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Blotting, Southern
  • Clone Cells
  • DNA
  • Female
  • Gene Rearrangement
  • Humans
  • Immunohistochemistry
  • Lymphoma / genetics
  • Lymphoma / pathology*
  • Nucleic Acid Hybridization
  • Parotid Gland / pathology
  • Sjogren's Syndrome / complications*
  • Skin / pathology
  • Skin Diseases / complications*
  • Skin Diseases / pathology
  • Skin Neoplasms / genetics
  • Skin Neoplasms / pathology*
  • T-Lymphocytes / pathology*
  • T-Lymphocytes / physiology


  • DNA