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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 165
A case of sarcoidosis in a patient with systemic sclerosis


Department of Dermatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan

Date of Web Publication5-Mar-2013

Correspondence Address:
Noriaki Nakai
Department of Dermatology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto 602-8566
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.108119

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How to cite this article:
Isohisa T, Nakai N, Hanada K, Takenaka H, Katoh N. A case of sarcoidosis in a patient with systemic sclerosis. Indian J Dermatol 2013;58:165

How to cite this URL:
Isohisa T, Nakai N, Hanada K, Takenaka H, Katoh N. A case of sarcoidosis in a patient with systemic sclerosis. Indian J Dermatol [serial online] 2013 [cited 2019 Sep 22];58:165. Available from: http://www.e-ijd.org/text.asp?2013/58/2/165/108119


Sir,

Sarcoidosis is a multisystem granulomatous disease, including skin and lung, of unknown etiology. [1] Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by diffuse fibrosis of the skin and internal organs. [2] Co-existence of these two diseases has been reported, but is rare. [3],[4],[5] Here, we describe the case of a 68-year-old Japanese woman who developed sarcoidosis 15 years after diagnosis of limited cutaneous systemic sclerosis (lcSSc).

The patient consulted with a doctor about erythematous plaques that had appeared one year earlier on her face [Figure 1] and left knee. Fifteen years previously, at age 53, she was diagnosed with lcSSc based on proximal scleroderma from the fingers to the forearms, swelling of the fingers, Raynaud phenomenon symptoms of the hands, a positive anticentromere antibody, and an increase of collagen fiber with degenerative changes of the skin. She had taken oral tocopherol acetate for 15 years and the SSc-related skin symptoms had remitted. Laboratory data were normal at the time of consultation, including a complete blood count and tests for liver and kidney function. Serum angiotensin-converting enzyme, immunoglobulin (Ig) A, IgG, IgM, complement component 3 and 4, and total hemolytic complement (CH50) levels were also normal. Autoantibody screening was positive for antinuclear antibody (titer, 1:1280; normal range, <1:40) with a speckled pattern, and was also positive for anticentromere antibody (139; normal range, <10.0 index). Anti-topoisomerase I antibody was negative. The serum level of Krebs von den Lungen-6 was slightly elevated (580 U/ml). Chest radiography findings were normal. The lcSSc-associated pulmonary fibrosis in both lower lobes and a slight dysfunction of peristalsis of the esophagus was detected by chest computed tomography and esophagography with barium contrast medium, respectively, but did not require treatment. A gallium-67 citrate scintigram showed increased uptake in the parotid glands and a tuberculin skin test was negative. Biopsy specimens in plaques collected from the face and left knee showed multiple and well-circumscribed non-caseating epithelioid granulomas in the superficial and middle dermis [Figure 2]a and b. We diagnosed the case as sarcoidosis in a patient with lcSSc based on the findings in skin lesions, the negative tuberculin skin test, and the scintigraphic finding.
Figure 1: Erythematous plaques on the face consistent with sarcoidosis

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Figure 2: A hematoxylin-eosin (HE)-stained section of specimen from the face (a) Granulomas in the dermis and a comparatively dense inflammatory cell infiltration in the superficial and middle dermis (HE, original magnification ×40) (b) A well-circumscribed non-caseating epithelioid granuloma surrounded by a comparatively large number of lymphocytes in the dermis (HE, original magnification ×200)

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The prevalence of sarcoidosis and SSc is estimated to be 10.9 [6] and 10.0 [7] per 100,000, respectively. The mechanism underlying the coexistent occurrence is unclear. However, the reason may be the difference of immune responses closely related to sarcoidosis and SSc. Sarcoidosis is believed to be caused by Th1 immune responses. [8] In contrast, SSc is closely related to/Th2 immune responses in its early stage, but to/Th1 immune responses in its later stage. [8],[9] In the present case, the skin sclerosis had remitted and the active lung inflammation was also not detected, and the sarcoidosis occurred 15 years after the onset of SSc, suggesting that the shift from/Th2 to/Th1 might have contributed to the development of sarcoidosis. However, the number of reported cases of coexistence of these two diseases is insufficient to establish a clear mechanism and further accumulation of data is needed.

 
   References Top

1.Hoang DQ, Nguyen ET. Sarcoidosis. Semin Roentgenol 2010;45:36-42.  Back to cited text no. 1
[PUBMED]    
2.Dghoughi S, El Wady W, Taleb B. Systemic sclerosis. Case report and review of literature. N Y State Dent J 2010;76:30-5.  Back to cited text no. 2
[PUBMED]    
3.Cox D, Conant E, Earle L, Newman J, Kahaleh B, Jimenez S, et al. Sarcoidosis in systemic sclerosis: Report of 7 cases. J Rheumatol 1995;22:881-5.  Back to cited text no. 3
[PUBMED]    
4.De Bandt M, Perrot S, Masson C, Meyer O. Systemic sclerosis and sarcoidosis: A report of five cases. Br J Rheumatol 1997;36:117-9.  Back to cited text no. 4
[PUBMED]    
5.Takahashi T, Munakata M, Homma Y, Kawakami Y. Association of progressive systemic sclerosis with pulmonary sarcoidosis. Just a chance occurrence? Intern Med 1997;36:435-8.  Back to cited text no. 5
[PUBMED]    
6.Marchell RM, Thiers B, Judson MA. Sarcoidosis. In: Wolff K, Goldsmith LA, Katz SI, editors. Dermatology in General Medicine. 7th ed. New York: McGraw-Hill; 2008. p. 1484-93.  Back to cited text no. 6
    
7.Denton CP, Black CM. Scleroderma. In: Wolff K, Goldsmith LA, Katz SI, editors. Dermatology in General Medicine. 7th ed. New York: McGraw-Hill; 2008. p. 1553-62.  Back to cited text no. 7
    
8.Meloni F, Solari N, Cavagna L, Morosini M, Montecucco CM, Fietta AM. Frequency of Th1, Th2 and/Th17 producing T lymphocytes in bronchoalveolar lavage of patients with systemic sclerosis. Clin Exp Rheumatol 2009;27:765-72.  Back to cited text no. 8
[PUBMED]    
9.Matsushita T, Hasegawa M, Hamaguchi Y, Takehara K, Sato S. Longitudinal analysis of serum cytokine concentrations in systemic sclerosis: Association of interleukin 12 elevation with spontaneous regression of skin sclerosis. J Rheumatol 2006;33:275-84.  Back to cited text no. 9
[PUBMED]    


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1 A case of sarcoidosis with systemic sclerosis
Authors of Document Ren, X.-X., Hou, S.-W., Li, Z.-F., (...), Liu, L., Xu, L.-M.
Source of the Document Chinese Medical Journal. 2013;
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