DOI: 10.12890/2024_004632

A rare cutaneous manifestation of systemic sclerosis

Authors

  • Joelle Sleiman Department of Internal Medicine, Staten Island University Hospital, New York, USA
  • Taimur Aslam Department of Internal Medicine, Staten Island University Hospital, New York, USA
  • Anastasia Slobodnick Department of Rheumatology, Staten Island University Hospital, New York, USA
  • Alina G. Bridges Department of Dermatology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York, USA

jsleiman@northwell.edu

Keywords

Systemic sclerosis, scleroderma, cutaneous manifestations, nodular scleroderma

Abstract

A 43-year-old male with a history of intravenous drug use and alcohol consumption presented to the emergency department with three-month history of failure to thrive. The patient exhibited a constellation of constitutional symptoms including cough, weight loss, fatigue, decreased appetite, nausea and vomiting. The skin examination revealed multiple subcutaneous hyperpigmented, indurated plaques and nodules on the trunk and arms. Laboratory evaluation revealed abnormal autoimmune tests, anaemia, elevated inflammatory markers and radiological evidence of interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH). An excisional biopsy from a skin lesion demonstrated dermal sclerosis consistent with scleroderma. The patient was diagnosed with diffuse systemic scleroderma with cutaneous findings consistent with nodular or keloidal scleroderma variant. This case highlights a rare cutaneous variant of systemic scleroderma called nodular or keloidal scleroderma.

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References

  1. Adigun R, Goyal A, Hariz A. Systemic Sclerosis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-.
  2. Denton CP, Khanna D. Systemic sclerosis. Lancet 2017;390:1685–1699.
  3. Papara C, De Luca DA, Bieber K, Vorobyev A, Ludwig RJ. Morphea: the 2023 update. Front Med 2023;10:1108623.
  4. Danczak-Pazdrowska A, Cieplewicz P, Zaba R, Adamski Z, Polanska A. Controversy around the morphea. Adv Dermatol Allergol 2021;38:716–720.
  5. Krieg T, Takehara K. Skin disease: a cardinal feature of systemic sclerosis. Rheumatology (Oxford) 2006;48:iii14–8.
  6. Starnoni M, Pappalardo M, Spinella A, Testoni S, Lattanzi M, Feminò R, et al. Systemic sclerosis cutaneous expression: management of skin fibrosis and digital ulcers. Ann Med Surg (Lond.) 2021;71:102984.
  7. Le EN, Junkins-Hopkins JM, Sherber NS, Wigley FM. Nodular/keloidal scleroderma: acquired collagenous nodules in systemic sclerosis. J Rheumatol 2012;39:660–661.
  8. Srisuttiyakorn C, Aunhachoke K. Scleroderma with nodular scleroderma. Case Rep Dermatol 2016;8:303–310.
  9. Richarz NA, Olivé A, García-Patos V, Quirant B, Fernández Figueras MT, Bielsa I. A review of the clinically distinguishing features of nodular or keloidal scleroderma in systemic sclerosis. Australas J Dermatol 2020;61:e269–e273.
  10. Careta MF, Romiti R. Localized scleroderma: clinical spectrum and therapeutic update. An Bras Dermatol 2015;90:62–73.
  11. Kurzinski K, Torok KS. Cytokine profiles in localized scleroderma and relationship to clinical features. Cytokine 2011;55:157–164.

Published

2024-10-30

Issue

Vol. 11 No. 11 (2024)

Categories

How to Cite

1.
Sleiman J, Aslam T, Slobodnick A, Bridges AG. A rare cutaneous manifestation of systemic sclerosis. EJCRIM [Internet]. 2024 Oct. 30 [cited 2026 Nov. 3];11(11). Available from: https://ejcrim.com/index.php/ejcrim/article/view/4632