title: Sparing effect of hemiplegia on skin fibrosis and microvascular involvement: reports of two cases of systemic sclerosis and review of the literature.
abstract: OBJECTIVE
  The sparing effect of hemiplegia in rheumatic diseases has been described, but reports on systemic sclerosis (SSc)-spectrum disorders are unusual. SSc-spectrum disorders are complex diseases of unknown origin characterized by multisystem involvement, skin and organ fibrosis, microvascular alterations, and immunologic abnormalities. We describe two cases of patients with hemiplegia who developed Raynaud׳s phenomenon and skin fibrosis of the non-paretic limb.
  
  
  METHODS
  Clinical, laboratory, and investigation findings of two cases with hemiplegia who developed scleroderma spectrum disorders of the non-paretic limb are presented. A review of the medical literature was performed in PubMed for all articles in English.
  
  
  RESULTS
  A total of 46 reports from 1935 to 2012 were identified, especially on osteoarthritis and rheumatoid arthritis. Only two case reports on patients with SSc describe asymmetric SSc skin involvement and unilateral acro-osteolysis on x-ray images of the non-paretic limb. By contrast, we report the first description of capillaroscopic microvascular changes in patients with hemiplegia and asymmetric SSc skin involvement.
  
  
  CONCLUSIONS
  Our cases point out the potential role of a "cross-talk" between the nervous system and the skin in SSc-spectrum disorders and suggest future directions for research in studies of pathogenesis.
authors.affiliation: Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy. Electronic address: nicola.ughi@gpini.it.
authors.forename: Nicola
authors.initials: N
authors.lastname: Ughi
authors.affiliation: Eastbourne District General Hospital, Eastbourne, East Sussex, UK.
authors.forename: Simon A
authors.initials: SA
authors.lastname: Hervey
authors.affiliation: Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Gualtierotti
authors.affiliation: Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy.
authors.forename: Zeni
authors.initials: Z
authors.lastname: Silvana
authors.affiliation: Centre for Musculoskeletal Research, Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK.
authors.forename: Ariane L
authors.initials: AL
authors.lastname: Herrick
authors.affiliation: Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Ingegnoli
authors.affiliation: Division of Rheumatology, Department of Clinical Sciences and Community Health, Gaetano Pini Orthopedic Institute, University of Milano, Piazza Cardinal Ferrari 1, Milano 20122, Italy.
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Meroni
mesh_terms: D005260:Female; D005355:Fibrosis / Q000473:pathology; D006429:Hemiplegia / Q000150:complications / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology*; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000150:complications / Q000473:pathology*
keywords: Connective tissue disease; Hemiplegia; Nailfold capillaroscopy; Scleroderma; Scleroderma spectrum disease; Systemic sclerosis