Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2025 Feb 15;17(2):e79036.
doi: 10.7759/cureus.79036. eCollection 2025 Feb.

Erasmus Syndrome, An Autoimmunity Paradox: A Case Report and Literature Review

Alejandro Arango  1 , Simon Villa-Pérez  2   3 , Jhon Edwar Garcia Rueda  1 , Alejandro Cardona Palacio  4   5 , Roberto Benavides  6
Affiliations
Case Reports

Erasmus Syndrome, An Autoimmunity Paradox: A Case Report and Literature Review

Alejandro Arango et al. Cureus. .

Abstract

Erasmus syndrome (ES) is a rare condition characterized by the link between crystalline silica exposure, with or without silicosis, and systemic sclerosis (SSc). Although first noted over a century ago, its underlying mechanisms remain unclear. However, it is indistinguishable from idiopathic SSc in the general population. Its clinical presentation is heterogeneous, depending on the affected systems, with notable features, including skin fibrosis, microstomia, telangiectasia, Raynaud's phenomenon, arthralgia, and interstitial lung disease. Currently, there is no unified consensus on its treatment; however, organ-specific therapy is a reasonable approach. We report the case of a 43-year-old miner diagnosed with diffuse cutaneous SSc, where ES was diagnosed after an exhaustive history was taken, occupational exposure was characterized, differential diagnoses were excluded, and radiological and histopathological evidence of pulmonary silicosis was presented.

Keywords: erasmus syndrome; ild interstitial lung disease; immunosuppressive agents; silicosis; systemic sclerosis.

PubMed Disclaimer

Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Skin involvement
(A) Microstomia. (B) Area of alopecia in the temporal region. (C) Skin induration and salt and pepper lesions.
Figure 2
Figure 2. Edematous involvement
(A, B) Edema from the wrist to the distal interphalangeal joints, with loss of skin folds on the thumb. (C) Scars from fingertip ulcers.
Figure 3
Figure 3. CT chest
(A, B) Arrows indicating lymphadenopathy with punctate peripheral calcifications (“eggshell calcifications”). (C) Pulmonary parenchyma with bilateral thickening of interlobular septa and nodular lesions. (D) Pulmonary parenchyma with confluent nodular lesions of irregular morphology in the right upper lobe.
Figure 4
Figure 4. Pathology
(A, B) Nodules of fibroblasts and histiocytes with abundant silica. (C) Polarized light shows silica crystals.
See this image and copyright information in PMC

References

    1. Diagnostic classification of interstitial lung disease in clinical practice. Adegunsoye A, Ryerson CJ. Clin Chest Med. 2021;42:251–261. - PubMed
    1. Interstitial lung diseases. Wijsenbeek M, Suzuki A, Maher TM. Lancet. 2022;400:769–786. - PubMed
    1. Interstitial pneumonia with autoimmune features: an update. Valenzuela C, Cottin V, Ancochea J. Arch Bronconeumol (Engl Ed) 2018;54:447–448. - PubMed
    1. Systemic sclerosis-associated interstitial lung disease. Perelas A, Silver RM, Arrossi AV, et al. Lancet Respir Med. 2020;8:304–320. - PubMed
    1. Diffuse sclerodermia: its frequency; its occurrence in stone-masons; its treatment by fibrolysin—elevations of temperature due to fibrolysin injections. Bramwell B. https://pmc.ncbi.nlm.nih.gov/articles/PMC5271266/ Edinb Med J. 1914;12:387–401.

Publication types

LinkOut - more resources