The vanishing touch: Unveiling the tuft erosion in scleroderma

Hui Jiang; Yangzhong Zhou

doi:10.1515/rir-2024-0008

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The vanishing touch: Unveiling the tuft erosion in scleroderma

Hui Jiang und Yangzhong Zhou

Veröffentlicht/Copyright: 31. März 2024

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Aus der Zeitschrift Rheumatology and Immunology Research Band 5 Heft 1

A 52-year-old woman presented to our clinic complaining of stiffness and shortened fingers. She had a 30-year history of swollen fingers, sclerodactyly, fingertip ulcers, telangiectasias, and subcutaneous calcification. In the last three years, she developed dyspnea, exercise intolerance, and gastroesophageal reflux. Physical examination revealed flexion contractures of the interphalangeal joints, and the distal part of her fingers disappeared. (Figure 1A, B). Laboratory tests showed positive anticentromere antibody. Hands X-ray revealed acro-osteolysis presenting a picture like a “pencil-incup” ^[1] Bilateral wrist X-ray showed mild joint space narrowing, while the metacarpophalangeal joints appeared unaffected (Figure 1C). Chest computer tomography (CT) disclosed severe interstitial lung disease and extensive destruction across both lungs (Figure 1D, E). The patient was treated with prednisone 30 mg daily, combined with mycophenolate mofetil 0.5 g twice daily, tadalafil 10 mg daily, and pirfenidone.

Figure 1

A, B: Flexion contractures of the interphalangeal joints. C: Hands X-ray presented acro-osteolysis. D, E: CT scan presented severe interstitial lung disease.

Acro-osteolysis, a typical complication of scleroderma, is caused by chronic ischemic changes of the digital tuft due to an extended period of Raynaud’s syndrome.^[2,3] Acroosteolysis may lead to diminished tactile sensitivity and undermine the structural integrity of fingers, metaphorically described as “The Vanishing Touch”. Early recognition and treatment may potentially prevent extensive bone tissue destruction.

Address for correspondence: Yang-Zhong Zhou, Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College; Beijing, China.

Funding statement: None.

Acknowledgement

The authors thank the patient for providing original source of image.

Author Contributions

Hui Jiang contributed to the original draft writing. Yangzhong Zhou contributed to paper revising and editing.
Informed Consent

The authors declare that the patient has given the consent for the images and other clinical information to be reported in the journal.
Ethical Statement

The study was approved by the Ethics Committee of PUMCH (Beijing, China; approval number: JS-2038).
Conflict of Interest

The authors declare that they have no competing interests.
Data availability statement

No additional data was generated. The data of this case are available from the corresponding author upon reasonable request.

References

[1] Avouac J, Guerini H, Wipff J, et al. Radiological hand involvementin systemic sclerosis. Ann Rheum Dis. 2006;65:1088–1092.Suche in Google Scholar

[2] Kemp SS, Dalinka MK, Schumacher HR. Acro-osteolysis. Etiologic and radiological considerations. JAMA. 1986;255:2058–2061.Suche in Google Scholar

[3] Avouac J, Mogavero G, Guerini H, et al. Predictive factors of hand radiographic lesions in systemic sclerosis: a prospective study. Ann Rheum Dis. 2011;70:630–633.Suche in Google Scholar

Received: 2023-09-04

Accepted: 2024-03-04

Published Online: 2024-03-31

This work is licensed under the Creative Commons Attribution 4.0 International License.

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