Limited cutaneous systemic sclerosis

Last reviewed: 29 Aug 2022
Last updated: 14 Nov 2018

Summary

Definition

History and exam

Key diagnostic factors

  • Raynaud phenomenon (RP)
  • dysphagia
  • GERD
  • dilated nailbed capillaries
  • bright shiny skin of hands, feet
  • symmetrical swelling, tight fingers
  • prayer sign
  • claw hand deformities
  • ulcers on fingers
  • flexion contracture of hands
  • carpal tunnel syndrome
  • calcinosis
  • large well-demarcated telangiectasia
More key diagnostic factors

Other diagnostic factors

  • skin pigmentation changes
  • family history of connective tissue disease
Other diagnostic factors

Risk factors

  • silica dust exposure
  • family history of scleroderma
  • polyvinylchlorides
More risk factors

Diagnostic investigations

1st investigations to order

  • complete blood count
  • serum creatinine
  • serum antinuclear antibody
  • serum extractable nuclear antigens
  • serum erythrocyte sedimentation rate (ESR)
  • echocardiogram
  • pulmonary function tests (PFTs)
More 1st investigations to order

Investigations to consider

  • peripheral blood smear
  • anticentromere antibody
  • esophageal manometry
More investigations to consider

Treatment algorithm

ACUTE

renal crisis

ONGOING

no renal crisis

Contributors

Authors

Janet E. Pope, MD, MPH, FRCPC
Janet E. Pope

Professor of Medicine

Division of Rheumatology

Department of Medicine

Schulich School of Medicine and Dentistry

University of Western Ontario

Head

Division of Rheumatology

St. Joseph's Health Care

London

Ontario

Canada

Disclosures

JEP has consulted for and/or performed research trials with AbbVie, Actelion, Amgen, BMS, Lilly, Merck, Novartis, Pfizer, Roche, Sandoz, Sanofi, and United Chemicals Belgium. JEP is an author of several references cited in this topic.

Peer reviewers

Virginia Steen, MD

Professor of Medicine

Georgetown University

Washington

DC

Disclosures

VS has received research funding, consulting fees, and honorarium from Actelion and Gilead, which could result in competing interests. VS is an author of a number of references cited in this topic.

Frank Wollheim, MD

Emeritus Professor

Department of Rheumatology

Lund University Hospital

Lund

Sweden

Disclosures

FW is an author of a reference cited in this topic.

Gabriela Riemekasten, PD, Dr. med.

Charité-Universitätsmedizin Berlin

Medizinische Klinik mit Schwerpunkt

Rheumatologie und Klinische Immunologie

Charitéplatz

Berlin

Germany

Disclosures

GR is a consultant for Encysive, Actelion, Bayer, GSK, Roche, and other companies, and received lecture fees from all of these companies involved in systemic scleroderma. GR is an author of a reference cited in this topic.

  • Limited cutaneous systemic sclerosis images
  • Differentials

    • Generalized morphea
    • Eosinophilic fasciitis (Shulman syndrome)
    • Nephrogenic systemic fibrosis
    More Differentials
  • Guidelines

    • Update of EULAR recommendations for the treatment of systemic sclerosis
    More Guidelines
  • Patient leaflets

    Carpal tunnel syndrome

    Raynaud's phenomenon

    More Patient leaflets
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