Sarcoidosis is a multisystem granulomatous disease characterized by hilar lymphadenopathy, involvement of internal organs, and diverse skin lesions. Systemic sclerosis is an autoimmune disease characterized by skin hardening and different internal organ fibrosis, including vascular abnormality. Immune response associated with Th-2 has been shown in the early and active stage of the disease. In this paper, we report coexistence of systemic sclerosis with sarcoidosis in a female patient presenting with granulomatous dermatitis, interstitial lung disease, and Raynaud’s phenomenon complaints. 1. Introduction Sarcoidosis is a systemic disease characterized by the involvement of multiple tissues and organs and a noncaseating granulomatous reaction, which is not well understood [1]. Although its pathogenesis is not clear, there appears to be a cellular immune system activation and a nonspecific inflammatory response against some genetic and environmental factors [2]. Th1 and macrophages caused by proinflammatory cytokines induce the inflammatory cascade; the formations of granulomas occur as a result of tissue permeability, cellular influx, and local cell proliferation [3]. The key pathological finding of sarcoidosis are noncaseating epitheloid cellular granulomas [4]. Epitheloid cells are transformed bone marrow monocytes with significant secretory activity. Increased active CD4 T lymphocytes have been shown in tissues with sarcoidosis. These lymphocytes visibly increase in the granulomatous lesion, while small numbers of CD8 T-Ly, B-cells, plasma, and mast cells are identified on the outer part of the granuloma [5]. A difference in prevalence, clinical findings, and the course of disease in varied races and ethnic groups suggests that sarcoidosis is a heterogeneous disease [6]. The disease is more prevalent in women and tends to develop after 40 years of age. The incidence of sarcoidosis in the USA is 10.9 per 100.000 in the white race and increases to 35.5 per 100.000 in African-Americans, with a more severe course [7]. Sarcoidosis is a chronic granulomatous disease that may display different clinical findings. The disease most frequently presents with bilateral hilar lymphadenopathy, infiltrations in the lungs and skin, and eye lesions. It may mimic a number of primary rheumatic diseases and/or develop associated to these [8]. The incidence of sarcoidosis together with different connective tissue diseases (RA, SLE, and Sj？gren’s syndrome) has increased. Similar immunological abnormalities observed between sarcoidosis and autoimmune diseases suggest a
References
[1]
L. S. Newman, C. S. Rose, and L. A. Maier, “Sarcoidosis,” The New England Journal of Medicine, vol. 336, no. 17, pp. 1224–1234, 1997.
[2]
S. Hofmann, A. Franke, A. Fischer et al., “Genome-wide association study identifies ANXA11 as a new susceptibility locus for sarcoidosis,” Nature Genetics, vol. 40, no. 9, pp. 1103–1106, 2008.
[3]
E. S. Chen and D. R. Moller, “Etiology of sarcoidosis,” Clinics in Chest Medicine, vol. 29, no. 3, pp. 365–377, 2008.
[4]
A. Smith, I. Brownell, M. Sanchez, and S. Prystowsky, “Advances in the genetics of sarcoidosis,” Clinical Genetics, vol. 73, no. 5, pp. 401–412, 2008.
[5]
Y. P. Kataria and J. F. Holter, “Immunology of sarcoidosis,” Clinics in Chest Medicine, vol. 18, no. 4, pp. 719–739, 1997.
[6]
B. A. Rybicki, M. Major, J. Popovich Jr., M. J. Maliarik, and M. C. Iannuzzi, “Racial differences in sarcoidosis incidence: a 5-year study in a health maintenance organization,” The American Journal of Epidemiology, vol. 145, no. 3, pp. 234–241, 1997.
[7]
N. Milman and O. Selroos, “Pulmonary sarcoidosis in the Nordic countries 1950–1982. Epidemiology and clinical picture,” Sarcoidosis, vol. 7, no. 1, pp. 50–57, 1990.
[8]
T. Pettersson, “Rheumatic features of sarcoidosis,” Current Opinion in Rheumatology, vol. 10, no. 1, pp. 73–78, 1998.
[9]
R. J. Enzenauer and S. G. West, “Sarcoidosis in autoimmune disease,” Seminars in Arthritis and Rheumatism, vol. 22, no. 1, pp. 1–17, 1992.
[10]
M. de Bandt, S. Perrot, C. H. Masson, and O. Meyer, “Systemic sclerosis and sarcoidosis, a report of five cases,” The British Journal of Rheumatology, vol. 36, no. 1, pp. 117–119, 1997.
[11]
A. C. H. Ho, N. Hasson, and D. Singh-Grewal, “Sarcoidosis or scleroderma? An unusual case of sarcoidosis in a 3-year-old Caucasian girl,” Rheumatology, vol. 48, no. 9, pp. 1172–1173, 2009.
[12]
N. Sakamoto, Y. Ishimatsu, T. Kakugawa et al., “Sarcoidosis in a patient with systemic sclerosis and primary biliary cirrhosis,” Internal Medicine, vol. 49, no. 15, pp. 1609–1611, 2010.
[13]
H. Suga, Y. Asano, Z. Tamaki et al., “A case of systemic sclerosis with sarcoidosis,” Acta Dermato-Venereologica, vol. 91, no. 6, pp. 718–720, 2011.
[14]
H. Groen, D. S. Postma, and C. G. M. Kallenberg, “Interstitial lung disease and myositis in a patient with simultaneously occurring sarcoidosis and scleroderma,” Chest, vol. 104, no. 4, pp. 1298–1300, 1993.
[15]
M. D. Mayes, J. V. Lacey Jr., J. Beebe-Dimmer et al., “Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population,” Arthritis and Rheumatism, vol. 48, no. 8, pp. 2246–2255, 2003.
[16]
S. Romagnani, “T-cell subsets (Th1 versus Th2),” Annals of Allergy, Asthma and Immunology, vol. 85, no. 1, pp. 9–18, 2000.
