calcinosis, hand, Raynaud syndrome, scleroderma, systemic sclerosis, upper extremity



  1. Cohen, Joshua M. MD
  2. Sibley, Rachel A. MD
  3. Chiu, Ernest S. MD, FACS
  4. Sharma, Sheel MD


GENERAL PURPOSE: To provide information about the pathophysiology, diagnosis, and treatment options for systemic sclerosis.


TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care.


LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant should be better able to:


1. Describe the pathophysiology, signs, symptoms, and diagnosis of systemic sclerosis.


2. Outline the evidence-based medical and surgical management of systemic sclerosis.




OBJECTIVE: To perform a targeted review of systemic sclerosis, including epidemiology, pathophysiology, diagnosis, signs and symptoms, and medical and surgical management of upper extremity manifestations.


DATA SOURCES AND STUDY SELECTION: An electronic literature review was conducted using PubMed for all publication dates through October 2017. Searches were performed using combinations of terms including "systemic sclerosis," "scleroderma," "management," "upper extremity," "hypercalcinosis," "Raynaud's phenomenon," "sympathectomy," and "digital ulcers." Only full-length articles written in English that discussed the management of upper extremity scleroderma were used.


DATA EXTRACTION AND SYNTHESIS: The epidemiology, pathophysiology, diagnosis, upper extremity manifestations, and medical and surgical management of systemic sclerosis were reviewed. The case described in this article reports the utility of microsurgical interventions in the treatment of medically refractory upper extremity systemic sclerosis.


CONCLUSIONS: Systemic sclerosis is a rare rheumatologic disease that greatly impacts quality of life. Medical management is the mainstay of treatment, propelling an improvement in the dismal 10-year cumulative survival rate from 54% in the 1970s to 66% in the 1990s. However, the pathophysiology of this disease is still poorly understood, and when medical management fails and the disease inevitably progresses, surgical approaches are critical.