Cutaneous mastocytosis in a patient with primary Sjögren's syndrome

Shebnem Kalay Erbay, Carol Stewart, Ashraf Hassanein, Andrew Fletcher, Indraneel Bhattacharyya, Donald Cohen, Stanton K. Wesson, Jason Weinstein, Robert Lyons, Westley H. Reeves

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Mast cells have been linked to rheumatoid arthritis (RA) and are essential to the pathogenesis of RA-like disease in a mouse model. We describe a 34-year-old woman who developed Sjögren's syndrome concurrently with telangiectasia macularis eruptiva perstans (TMEP), a rare form of cutaneous mastocytosis. The patient had sicca symptoms with an abnormal minor salivary gland biopsy and decreased salivary flow, peripheral neuropathy, an 80 pound weight loss, and a macular erythematous rash that exhibited superficial perivascular mast cell infiltrates on biopsy of lesional skin. This case further underscores the link between mast cells and the development of autoimmunity.

Original languageAmerican English
Pages (from-to)1697-1700
Number of pages4
JournalJournal of Rheumatology
Volume33
Issue number8
StatePublished - Aug 2006
Externally publishedYes

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

Keywords

  • Autoimmunity
  • Cutaneous mastocytosis
  • Mast cells
  • Sjögren's syndrome

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