Surgical Management of Digital Calcinosis in CREST Syndrome

Abstract

As a limited form of sclerodermy, CREST syndrome is characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which determine the acronym CREST. Calcinosis is a particularly difficult entity to treat given the paucity of effective options described in the literature. Treatment of finger calcinosis has a wide range of possibilities depending on the extent of calcifications and the involvement of deep structures. From a surgical point of view, whereas simple removal is adequate in minor outpatient cases, a radical debridement in the major and more painful cases seems required. A cover flap is needed particularly in the thumb due to its great functional importance, also if the fingertip is not involved. The authors recommend the kite flap for the dimensions, the tissue quality, and the possibility of giving sensation to the reconstructed area. With this surgical option, the transferred skin is soft, sensate, and the right fit. Usually, no further operations are needed for flap remodeling. The time required for sensory integration is about 2 years, often related to the age of the patient. Debridement and flap reconstruction usually give total resolution of pain, with complete recovery of thumb motion and the thumb-index finger grip.

CREST Syndrome - a Limited Form of Systemic Scleroderma: a Case Report and Literature Review

“…Cutaneous deposition of calcium salts in the skin occurs in a variety of clinical settings, beginning as a calcium phosphate nidus and progressing to hydroxyapatite crystal formation within a collagen matrix (43). Dystrophic calcifi cation is due to accumulation of calcium apatite crystals, with normal levels of serum calcium, phosphorus, and alkaline phosphatase (44). Calcium deposits develop in the skin, as subcutaneous nodules or massive nodes, mainly in the elbows, knees, fi ngers and toes, but also in other body areas (45).…”

“…Treatment of fi nger calcinosis has a wide range of possibilities depending on the extent of calcifi cations and the involvement of deep structures. From a surgical point of view, simple removal is adequate in minor outpatient cases, whereas radical debridement is required in the major and more painful cases (44). Laser surgery may reduce telangiectasias, and amputation is sometimes necessary in gangrene.…”

Management of Calcinosis Associated with Systemic Sclerosis

“…Surgical excision is treatment of choice for larger and deeper calcium deposits, especially when they are located over tendons, blood vessels, and nerves, whose integrity and function need to be preserved. [59] In the Mayo Clinic experience, all 11 patients who underwent surgical excision alone responded (eight with complete response), as well as 16 out of 17 patients who received medical and surgical therapy (14 with complete response). In contrast, only 7 of 19 patients treated with medical therapy alone had any response (one with complete response) [4••].…”

Fluoroscopy-guided intra-articular steroid injection for sternoclavicular joint arthritis secondary to limited cutaneous systemic sclerosis: a case report

“…SSc is categorized into two groups: limited cutaneous (LcSSc) and diffuse cutaneous (DcSSc) forms. The former (the CREST syndrome) is characterized by calcinosis, Raynaud's phenomenon, sclerodactyly, esophageal dysmotility, and telangiectasia [1]. Although joint involvement is not so common in LcSSc, arthritis is reported to occur at a rate of 15% [23].…”