• J Rheumatol · Aug 2006

    Case Reports

    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, and Westley H Reeves.
    • Division of Rheumatology and Clinical Immunology, University of Florida, Gainesville, 32610-0021, USA.
    • J Rheumatol. 2006 Aug 1; 33 (8): 1697-700.

    AbstractMast 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.

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