title: Cutaneous mastocytosis in a patient with primary Sjögren's syndrome.
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.
authors.affiliation: Division of Rheumatology and Clinical Immunology, University of Florida, Gainesville, 32610-0021, USA.
authors.forename: Shebnem
authors.initials: S
authors.lastname: Kalay Erbay
authors.forename: Carol
authors.initials: C
authors.lastname: Stewart
authors.forename: Ashraf
authors.initials: A
authors.lastname: Hassanein
authors.forename: Andrew
authors.initials: A
authors.lastname: Fletcher
authors.forename: Indraneel
authors.initials: I
authors.lastname: Bhattacharyya
authors.forename: Donald
authors.initials: D
authors.lastname: Cohen
authors.forename: Stanton K
authors.initials: SK
authors.lastname: Wesson
authors.forename: Jason
authors.initials: J
authors.lastname: Weinstein
authors.forename: Robert
authors.initials: R
authors.lastname: Lyons
authors.forename: Westley H
authors.initials: WH
authors.lastname: Reeves
mesh_terms: D000328:Adult; D015551:Autoimmunity / Q000276:immunology; D001706:Biopsy; D004359:Drug Therapy, Combination; D005260:Female; D039563:Histamine H1 Antagonists, Non-Sedating / Q000627:therapeutic use; D006635:Histamine H2 Antagonists / Q000627:therapeutic use; D006801:Humans; D017336:Loratadine / Q000627:therapeutic use; D008407:Mast Cells / Q000276:immunology / Q000473:pathology*; D034701:Mastocytosis, Cutaneous / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D011899:Ranitidine / Q000627:therapeutic use; D012859:Sjogren's Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology*; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D039563:Histamine H1 Antagonists, Non-Sedating; D006635:Histamine H2 Antagonists; D017336:Loratadine; D011899:Ranitidine