Lee HY, Lee SK, Kim H, Kang SW. Extensive multiple calcinosis cutis in seronegative limited cutaneous scleroderma (CREST variant): A case report. World J Clin Cases 2026; 14(25): 123670 [DOI: 10.12998/wjcc.123670]
Corresponding Author of This Article
Hyo Yeol Lee, MD, PhD, Department of Orthopaedic Surgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, 776, 1Sunwhan-ro, Seowon-gu, Cheongju 28644, Chungbuk, South Korea. thebonedoctor@hanmail.net
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Orthopedics
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case-report
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Lee HY, Lee SK, Kim H, Kang SW. Extensive multiple calcinosis cutis in seronegative limited cutaneous scleroderma (CREST variant): A case report. World J Clin Cases 2026; 14(25): 123670 [DOI: 10.12998/wjcc.123670]
World J Clin Cases. Sep 6, 2026; 14(25): 123670 Published online Sep 6, 2026. doi: 10.12998/wjcc.123670
Extensive multiple calcinosis cutis in seronegative limited cutaneous scleroderma (CREST variant): A case report
Hyo Yeol Lee, Sun Kyoung Lee, Hosung Kim, Seo Won Kang
Hyo Yeol Lee, Sun Kyoung Lee, Hosung Kim, Seo Won Kang, Department of Orthopaedic Surgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju 28644, Chungbuk, South Korea
Co-first authors: Hyo Yeol Lee and Sun Kyoung Lee.
Author contributions: Lee HY supervised the whole study progress, revised and refined the manuscript; Lee SK managed the study design and drafted the manuscript; Kang SW and Kim H conducted literature search and review; Lee HY and Lee SK contributed equally to this work.
AI contribution statement: No generative artificial intelligence tools were used in the preparation of this manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Hyo Yeol Lee, MD, PhD, Department of Orthopaedic Surgery, Chungbuk National University Hospital, Chungbuk National University College of Medicine, 776, 1Sunwhan-ro, Seowon-gu, Cheongju 28644, Chungbuk, South Korea. thebonedoctor@hanmail.net
Received: May 26, 2026 Revised: July 19, 2026 Accepted: August 28, 2026 Published online: September 6, 2026 Processing time: 100 Days and 24 Hours
Abstract
BACKGROUND
Calcinosis cutis is a recognized manifestation of limited cutaneous systemic sclerosis (CREST syndrome), but extensive multifocal involvement of large joints and the trunk is rare. Diagnosis may be particularly challenging in patients with negative systemic sclerosis-specific autoantibodies.
CASE SUMMARY
A 59-year-old woman with extensive calcinosis cutis in the prepatellar region, elbows, and trunk, accompanied by Raynaud’s phenomenon. Despite persistently negative autoimmune serology results, a seronegative CREST variant was diagnosed based on calcinosis, Raynaud’s phenomenon, and clinically diagnosed sclerodactyly, together with histopathological evidence of chronic inflammation and fibrosis after excluding other differential diagnoses. Surgical excision of the prepatellar lesion resulted in complete resolution without recurrence. Histopathological examination revealed aggregated lymphocytes and fibrosis surrounding the calcified tissue, consistent with an autoimmune process.
CONCLUSION
CREST syndrome should not be excluded despite negative serology. Surgical excision may benefit symptomatic extensive calcinosis cutis.
Core Tip: This case describes a seronegative CREST variant presenting with widespread calcinosis cutis involving the prepatellar region, both elbows, and trunk, accompanied by Raynaud’s phenomenon and clinically diagnosed sclerodactyly. Surgical excision of the symptomatic lesions provided clinical resolution and enabled histopathological assessment demonstrating chronic lymphocytic inflammation and fibrosis. Negative autoimmune serology alone should not exclude limited cutaneous systemic sclerosis when characteristic clinical manifestations are present.
