Cutaneous mastocytosis in a patient with primary Sjögren's syndrome.
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.
Kalay Erbay
Shebnem
S
Division of Rheumatology and Clinical Immunology, University of Florida, Gainesville, 32610-0021, USA.
Stewart
Carol
C
Hassanein
Ashraf
A
Fletcher
Andrew
A
Bhattacharyya
Indraneel
I
Cohen
Donald
D
Wesson
Stanton K
SK
Weinstein
Jason
J
Lyons
Robert
R
Reeves
Westley H
WH
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
D039563:Histamine H1 Antagonists, Non-Sedating; D006635:Histamine H2 Antagonists; D017336:Loratadine; D011899:Ranitidine