Scleroderma of the Hand: Evaluation and Treatment

J Am Acad Orthop Surg. 2020 Aug 15;28(16):e686-e695. doi: 10.5435/JAAOS-D-19-00547.

Abstract

Scleroderma is derived from Latin meaning hard skin. It is an uncommon, noninflammatory connective tissue disorder characterized by increased fibrosis of the skin and in certain variants, multiple other organ systems. Scleroderma involves a spectrum of pathologic changes and anatomic involvement. It can be divided into localized and systemic scleroderma. Hand involvement is common and can include calcium deposits within the soft tissues, digital ischemia, and joint contracture. Nonsurgical management consists of lifestyle modifications, biofeedback, therapy for digital stiffness/contracture, and various pharmacologic medications. When nonsurgical measures are unsuccessful, certain surgical options may be indicated, each with their inherent advantages and pitfalls. Patients with scleroderma who are undergoing surgical intervention pose unique difficulties because of their poorly vascularized tissue and deficient soft-tissue envelopes, thus increasing their susceptibility to wound healing complications and infection. Some subgroups of patients are frequently systemically ill, and specific perioperative measures should be considered to reduce their surgical risk. The spectrum of hand manifestations seen in patients with scleroderma will be reviewed with the focus on evaluation and management.

Publication types

  • Review

MeSH terms

  • Calcinosis
  • Hand* / pathology
  • Hand* / surgery
  • Humans
  • Interdisciplinary Communication
  • Orthopedic Procedures / methods*
  • Patient Care Team
  • Scleroderma, Localized / diagnosis
  • Scleroderma, Localized / pathology
  • Scleroderma, Localized / surgery*
  • Scleroderma, Localized / therapy
  • Scleroderma, Systemic / diagnosis
  • Scleroderma, Systemic / pathology
  • Scleroderma, Systemic / surgery*
  • Scleroderma, Systemic / therapy