title: Hypotension, Syncope, and Fever in Systemic Mastocytosis without Skin Infiltration and Rapid Response to Corticosteroid and Cyclosporin: A Case Report.
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.
authors.affiliation: Department of Internal Medicine, Hacettepe University Medical School, Sıhhiye, 06350 Ankara, Turkey.
authors.forename: Didem
authors.initials: D
authors.lastname: Ozdemir
authors.forename: Selcuk
authors.initials: S
authors.lastname: Dagdelen
authors.forename: Tomris
authors.initials: T
authors.lastname: Erbas
authors.forename: Kemal
authors.initials: K
authors.lastname: Agbaht
authors.forename: Songul
authors.initials: S
authors.lastname: Serefhanoglu
authors.forename: Salih
authors.initials: S
authors.lastname: Aksu
authors.forename: Sibel
authors.initials: S
authors.lastname: Ersoy-Evans