Scleroderma of the Hand: Evaluation and Treatment
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Scleroderma is an uncommon, noninflammatory connective tissue disorder characterized by increased fibrosis of the skin. It involves a spectrum of pathologic changes and anatomic involvement. It can be localized and systemic scleroderma. Hand involvement is common and can include calcium deposits within the soft tissues, digital ischemia, and joint contracture.

Physical examination involves evaluation of soft-tissue integrity, vascular status, contracture, and deformity. When aggravating conditions are present, early clinical manifestations include a white appearing digit because
of a lack of circulation, further progressing to a blue discoloration (second stage) secondary to oxygen desaturation in the remaining blood. In third stage, the digit becomes hyperemic after vasospasm resolves because of the
anoxic stimulus. Calcinosis is common and has well-defined chalky or toothpastelike deposits in the distal tuft or along the length of the extremity. Other common findings are sclerodactyly, inflammation, dry gangrene, ulceration, nail bed petechiae, poor skin turgor, and dystrophic fingertips/nails. Characteristic claw deformities of the digits may develop.

Nonsurgical management consists of lifestyle modifications, biofeedback, therapy for digital stiffness/contracture, and various pharmacologic medications. Avoidance of caffeine ingestion, cold exposure, smoking, and psychological stress should be iterated to minimize digital ischemia. 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.

source: https://journals.lww.com/jaaos/Abstract/2020/08150/Scleroderma_of_the_Hand__Evaluation_and_Treatment.2.aspx
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