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Year : 2022  |  Volume : 67  |  Issue : 3  |  Page : 290-292
Erasmus syndrome: A rare occupational disease

Department of Dermatology, İstanbul Training and Research Hospital, İstanbul, Turkey

Date of Web Publication22-Sep-2022

Correspondence Address:
Vildan Manav
Department of Dermatology, İstanbul Training and Research Hospital, İstanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_778_20

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Erasmus syndrome is a rare syndrome accompanied by interstitial lung disease and scleroderma caused by silica exposure. There are a limited number of case reports in the literature. Awareness of this rare syndrome is important and occupational exposure should be questioned in patients presenting with scleroderma in our country, silica exposure is quite frequent and it should be kept in mind. Here, we report a case of a 41-year-old male patient presenting with interstitial lung disease, scleroderma, and serpiginous supravenous hypermelanosis caused by silica exposure who worked in the denim grinding for a short period of three months.

Keywords: Erasmus syndrome, scleroderma, serpiginous supravenous hypermelanosis, silicosis

How to cite this article:
Manav V, Avcı EB, Erdem O, Polat AK, Leblebici C, K Aksu AE. Erasmus syndrome: A rare occupational disease. Indian J Dermatol 2022;67:290-2

How to cite this URL:
Manav V, Avcı EB, Erdem O, Polat AK, Leblebici C, K Aksu AE. Erasmus syndrome: A rare occupational disease. Indian J Dermatol [serial online] 2022 [cited 2022 Sep 29];67:290-2. Available from:

   Introduction Top

Erasmus syndrome is a rare entity in which silicosis may accompany systemic scleroderma was defined in 1957 by Erasmus.[1] Systemic scleroderma, on the other hand, is an autoimmune disease with diffuse fibrosis in skin, gastrointestinal system, respiratory system, heart, and kidney. Although its pathogenesis is not fully understood, silica exposure has been associated with the disease.[2] People working in mines, quarries, ceramics manufacturing, and denim grinding are at risk of developing silicosis caused by silica dust inhalation. Silicosis is characterized by irreversible and progressive fibrosis that develops in the lung tissue due to exposure to silica crystals through inhalation.[3] In addition to silicosis, silica exposure can trigger autoimmune diseases such as scleroderma and rheumatoid arthritis. Herein, a 41-year-old male patient, who worked in denim grinding for 3 months and was diagnosed with Erasmus syndrome, will be presented.

   Case Report Top

A 41-year-old man presented with the complaint of tightening and discoloration of the skin, which has been observed for 2 years on the palmar and dorsal face of the hands, arms, trunk. In dermatological examination, depigmented and sclerotic plaques with peripheral salt-and-pepper appearance from shoulders to the distal end of the sternum in anterior trunk, from neck and shoulders to interscapular area in posterior trunk were observed. In addition, lesions compatible with serpinous supravenous hypermelanosis were observed in the bilateral forearm, in which venous trace was preserved and observed in dark color [Figure 1]. Telangiectasia on the patient's face and loss of skin lines on the forehead, sclerodactyly on the fingers were also determined [Figure 2]. There was no Raynaud phenomenon. In laboratory tests, hemogram and routine biochemical parameters were within normal limits. Fluorescent antinuclear antibody was positive with a fluorescent intensity of 4+, in the fine speckled pattern and a titer of 1/2560, anti-centromere (++++), anti-Scl-70 antibody (−) and quantiferon test were negative. The patient did not have dysphagia but dyspnea with effort. High-resolution computerized tomography (HRCT) of the lungs showed thickening of interlobular septa compatible with interstitial involvement and diffuse calcified lymph nodes more intensely in the hilar area [Figure 3]. There was bronchus in the bilateral lower zones. Respiratory function test resulted in FEV1/FVC: 98% FVC: 98%. On echocardiography, pulmonary artery pressure was increased to 38 mm Hg. Calcinosis cutis was not detected on direct radiographs.
Figure 1: (a) Salt-and-pepper appearance (b and c) Flexural surface of the right and left forearm: serpentine supravenous hyperpigmentation

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Figure 2: (a) Telangiectasia on face (b) Sclerodactyly on left hand

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Figure 3: (a and b) Thickening of interlobuler septa compatible with interstitial involvement. Common calcified lymph nodes, more intensely in the hiler area (HRCT)

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Skin biopsy revealed that hair follicle structures were not observed and the sweat glands' fat pad was lost. In addition, the sweat gland structures were pushed up in the dermis caused by the prominent sclerotic collagen production in the deep dermis [Figure 4].
Figure 4: (a) Loss in the hair follicle structures and sweat glands fat pads, the appearance of sweat gland, structures pushed up the dermis due to prominent sclerotic collagen production in the deep dermis (HE × 20)

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The patient was diagnosed with Erasmus syndrome as a result of anamnesis, current clinical, laboratory, and radiologic findings.

   Discussion Top

The history of occupational exposure to silica and silicosis concurrently with scleroderma called Erasmus syndrome (1). Inhaled silica particles are phagocyted by alveolar macrophages. as a result of macrophage destruction, interleukins are released. IL-2 plays a major role in chronic inflammation and fibrosis. Because of increased inflammatory response excessive accumulation of extracellular matrix proteins; clustering of fibroblasts, epithelioid inflammatory cells, and cell fragments are observed. Because immune-mediated tissue damage is triggered in the host with fibrogenic stimulation, there is an increased risk of developing interstitial lung disease, lung tuberculosis, lung cancer, and autoimmune diseases (systemic sclerosis, rheumatoid arthritis, and systemic lupus erythematosus).[4]

In Rustin et al.'s[5] study, systemic scleroderma caused by silica exposure in miners and stove-fitter workers developed in an average of 16.2 years (range 4–36) after silica exposure. However, in intensive exposure, clinical symptoms may occur between 5 and 10 years. Our case is the shortest duration of silica exposure with a 3-months of history.

Serpentine supravenous hypermelanosis (SSH) occurs after intravenous administration of chemotherapeutics. The most common drugs that cause this entity are 5-FU, doxtaxel, broxuridine, fetomustine, vinorelbin, actinomycin, methotrexate, daunorobicin, mercaptopurine, cyclophosphamide, bleomycin, vinblastine, decarbazine, vincristine, and prednisone.[6] Intravenous administration of chemotherapeutic drugs may cause the melanosomes in the keratinocyte to break down contributing to the formation of SSH.[6] Except for chemotherapeutics, it is defined depending on minocycline. In addition, SSH is rarely described in drug-free systemic lupus erythematosus, rheumatoid arthritis, and scleroderma.[7]

It was first described in 1984 as drug-unrelated SHH in 3 scleroderma patients in the study of Jawitz et al.'s.[8] In addition, SHH occurred drug-unrelated in scleroderma patients in our case. To the best of our knowledge, that is the first case in the literature that SHH is associated with Erasmus syndrome.

Differential diagnoses of SHH may include thrombophlebitis, Kurtis marmorata telangiectasia, erythema ab igne, livedo reticularis, and lichen planus following superficial venous trace.[9] The diagnosis is made by anamnesis, clinical examination, and radiographic methods.

Anti-Scl-70 positivity with the severity of interstitial lung disease in scleroderma; anti-centromer is associated with increased pulmonary artery pressure.[5] In our case, anti scl 70 was found to be negative and anti-centromer positive, unlike the literature.

Treatment is based on the severity and progression of interstitial lung disease. In the treatment, methylprednisolone, methotrexate, endothelin receptor antagonists, acetylsalicylic acid, and calcium channel blocker are frequently used. Parenteral cyclophosphamide, mycophenolate mofetil, and azathioprine and the combination of these two agents with systemic steroids are among the most frequently used agents in interstitial lung disease.[9],[10]

   Conclusion Top

Erasmus syndrome is an occupational disease. Because of the infrequency of this new entity awareness of this diagnosis is important. In order not to miss silicosis, especially in male patients diagnosed with scleroderma, silica exposure should be questioned. As stated in our case, Erasmus syndrome can develop even with short-term silica exposure. Measurements of silica concentration level and use of personal protective equipment against silica particle inhalation are important. And also HRCT is important for diagnosis in patients with interstitial lung disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Erasmus L. Scleroderma in gold-miners on the witwatersrand with particular reference to pulmonary manifestations. S Afr J Lab Clin Med 1957;3:209-31.  Back to cited text no. 1
Stern EP, Denton CP. The pathogenesis of systemic sclerosis. Rheum Dis Clin North Am 2015;41:367-82.  Back to cited text no. 2
Leung CC, Yu IT, Chen W. Silicosis. Lancet (London, England) 2012;379:2008-18.  Back to cited text no. 3
Hoy RF, Chambers DC. Silica-related diseases in the modern world. Allergy 2020;75:2805-17.  Back to cited text no. 4
Rustin MH, Bull HA, Ziegler V, Mehlhorn J, Haustein UF, Maddison PJ, et al. Silica-associated systemic sclerosis is clinically, serologically and immunologically indistinguishable from idiopathic systemic sclerosis. Br J Dermatol 1990;123:725-34.  Back to cited text no. 5
Sibaud V, Fricain JC, Baran R, Robert C. [Pigmentary disorders induced by anticancer agents. part I: Chemotherapy]. Ann Dermatol Venereol 2013;140:183-96.  Back to cited text no. 6
Makol A, Reilly MJ, Rosenman KD. Prevalence of connective tissue disease in silicosis (1985-2006)-a report from the state of Michigan surveillance system for silicosis. Am J Ind Med 2011;54:255-62.  Back to cited text no. 7
Jawitz JC, Albert MK, Nigra TP, Bunning RD. A new skin manifestation of progressive systemic sclerosis. J Am Acad Dermatol 1984;11:265-8.  Back to cited text no. 8
Ghosh SK, Bandyopadhyay D, Ghoshal L, Basu S. Letter: Docetaxel-induced supravenous serpentine dermatitis. Dermatol Online J 2011;17:16.  Back to cited text no. 9
Sari Surmel IZ, Orucoglu N. Erasmus syndrome: Systemic sclerosis and silicosis co-occurrence. Int J Rheum Dis 2018;21:1326-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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