title: Acute and regressive scleroderma concomitant to an acute CMV primary infection.
abstract: OBJECTIVE
  To describe the pathophysiological mechanisms involving cytomegalovirus (CMV) primary infection and natural killer (NK) cell expansion in the development of localized scleroderma.
  
  
  RESULTS
  A 43-year-old woman presented acute erythematous discoloration and skin thickening concerning face, neck, trunk, abdomen, and the four limbs, predominantly in proximal areas. Our case did not respond to systemic sclerosis criteria diagnosis. However, skin and muscle biopsy revealed early scleroderma associated with capillary thrombi, and tissue infiltration with NK cells (CD56+/Granzyme B). Scleroderma was attributed to CMV primary infection responsible for cytolytic hepatitis (7-fold over the limit) and circulating NK cell excess. After 6 months of prednisone and a 2-year follow-up, a complete resolution of symptoms was observed.
  
  
  CONCLUSIONS
  Our observation suggests a potential triggering role of CMV primary infection in the development of scleroderma. Histological features from our observation addresses the role of CMV and NK cells in the development of endothelial damage and fibrotic process.
authors.affiliation: St Eloi Hospital, Montpellier 1 University, Internal Medicine Department, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Radjiv
authors.initials: R
authors.lastname: Goulabchand
authors.affiliation: Gui de Chauliac Hospital, INSERM 475 and CHU 34295 Montpellier, Hematology and Pathology Department, 82 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Lakhdar
authors.initials: L
authors.lastname: Khellaf
authors.affiliation: St Eloi Hospital, Montpellier 1 University, Internal Medicine Department, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Amandine
authors.initials: A
authors.lastname: Forestier
authors.affiliation: Gui de Chauliac Hospital, INSERM 475 and CHU 34295 Montpellier, Hematology and Pathology Department, 82 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Valerie
authors.initials: V
authors.lastname: Costes
authors.affiliation: St Eloi Hospital, Montpellier 1 University, Virology Department, 82 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Foulongne
authors.affiliation: St Eloi Hospital, Montpellier 1 University, Internal Medicine Department, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France.
authors.forename: Alain
authors.initials: A
authors.lastname: le Quellec
authors.affiliation: St Eloi Hospital, Montpellier 1 University, Internal Medicine Department, 80 avenue Augustin Fliche, 34295 Montpellier Cedex 5, France. Electronic address: p-guilpain@chu-montpellier.fr.
authors.forename: Philippe
authors.initials: P
authors.lastname: Guilpain
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001706:Biopsy; D003586:Cytomegalovirus Infections / Q000150:complications*; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D007150:Immunohistochemistry; D007694:Killer Cells, Natural / Q000276:immunology; D008853:Microscopy; D009132:Muscles / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012594:Scleroderma, Localized / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology*; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D011241:Prednisone
keywords: Connective tissue diseases; Cytomegalovirus; Natural killer cells; Scleroderma; Systemic sclerosis