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Case Reports in Medicine
Volume 2010, Article ID 782595, 4 pages
http://dx.doi.org/10.1155/2010/782595
Case Report

Hypotension, Syncope, and Fever in Systemic Mastocytosis without Skin Infiltration and Rapid Response to Corticosteroid and Cyclosporin: A Case Report

1Department of Internal Medicine, Hacettepe University Medical School, Sıhhiye, 06350 Ankara, Turkey
2Department of Endocrinology and Metabolism, Hacettepe University Medical School, 06350 Ankara, Turkey
3Department of Hematology, Hacettepe University Medical School, 06350 Ankara, Turkey
4Department of Dermatology, Hacettepe University Medical School, 06350 Ankara, Turkey

Received 29 August 2010; Accepted 6 December 2010

Academic Editor: Hermann E. Wasmuth

Copyright © 2010 Didem Ozdemir et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a small proportion, there is extracutaneous involvement without skin infiltration. Other manifestations are flushing, tachycardia, dyspepsia, diarrhea, hypotension, syncope, and rarely fever. Various medications have been used but there is not a definite cure for systemic mastocytosis. The principles of treatment include control of symptoms with measures aimed to decrease mast cell activation. We describe a case of systemic mastocytosis presenting with hypotension, syncope attacks, fever, and local flushing. In bone marrow biopsy, increased mast cell infiltration was demonstrated. She had no skin infiltration. A good clinicopathological response was obtained acutely with combination therapy of glucocorticoid and cyclosporine.