title: Mastocytosis and systemic sclerosis: a clinical association.
abstract: BACKGROUND
  Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis. At the early stage of disease when organ involvement and extent of disease are emerging, mast cells may have some role, as implied by both symptoms and histologic evidence.
  
  
  METHODS
  A female patient diagnosed with cutaneous mastocytosis experienced the onset of systemic sclerosis after 15 years followed by the switch of mastocytosis to the systemic phenotype. A literature review on the evidences related to mast-cells activation in systemic sclerosis is presented below.
  
  
  CONCLUSIONS
  For clinicians, more attention must be paid to the potential association between systemic sclerosis and cancer. This case suggests that a proliferative disease in the mast cell compartment-though representing a rare association-may not be completely unexpected in SSc and perhaps excess mast cell activity can serve a pathogenic role in promoting fibrotic disease.
authors.affiliation: Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
authors.forename: Gianluca
authors.initials: G
authors.lastname: Bagnato
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Louisville, Louisville, KY USA.
authors.forename: William Neal
authors.initials: WN
authors.lastname: Roberts
authors.affiliation: Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Sciortino
authors.affiliation: Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
authors.forename: Donatella
authors.initials: D
authors.lastname: Sangari
authors.affiliation: Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, Messina, Italy.
authors.forename: Santa
authors.initials: S
authors.lastname: Cirmi
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Louisville, Louisville, KY USA.
authors.forename: Roneka L
authors.initials: RL
authors.lastname: Ravenell
authors.affiliation: Department of Chemical, Biological, Pharmaceutical and Environmental Sciences, University of Messina, Messina, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Navarra
authors.affiliation: Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.
authors.forename: Gianfilippo
authors.initials: G
authors.lastname: Bagnato
authors.affiliation: School and Division of Allergy and Clinical Immunology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy ; Institute of Applied Sciences and Intelligent Systems (ISASI), Pozzuoli, NA Italy.
authors.forename: Sebastiano
authors.initials: S
authors.lastname: Gangemi
keywords: Fibrosis; Mastcells; Mastocytosis; Systemic sclerosis