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Massive cutaneous follicular lymphoid hyperplasia in a patient with the Sjögren syndrome: 7-year follow-up and immunohistochemical study

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The Sjögren syndrome often gives rise to complications such as pseudolymphomas and/or mucosa-/skin-associated lymphocytic tumors (MALT/SALT lymphomas). This paper presents a 74-year-old female patient with the Sjögren syndrome complicated by cutaneous plaques/tumors as cutaneous lymphoid hyperplasia over a 7-year follow-up study period. Immunohistochemical examination disclosed B-cell rich lymphoid follicle formation in the skin with the presence of T and B cells and a varied assortment of features to include abundant plasma cell infiltration. Based on the clinical course and histopathological findings, patient condition was considered benign and arises from reaction toward certain unknown microbes.

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  1. Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjogren syndrome. Arch Intern Med 164:1275–1284

    Article  PubMed  Google Scholar 

  2. Masaki Y, Sugai S (2004) Lymphoproliferative disorders in Sjogren syndrome. Autoimmun Rev 3:175–182

    Article  PubMed  Google Scholar 

  3. Giannotti B, Santucci M (1993) Skin-associated lymphoid tissue (SALT)-related B-cell lymphoma (primary cutaneous B-cell lymphoma). A concept and a clinicopathologic entity. Arch Dermatol 129:353–355

    Article  PubMed  CAS  Google Scholar 

  4. Fox RI, Adamson TC III, Fong S, Robinson CA, Morgan EL, Robb JA, Howell FV (1983) Lymphocyte phenotype and function in pseudolymphoma associated with Sjogren’s syndrome. J Clin Invest 72:52–62

    Article  PubMed  CAS  Google Scholar 

  5. Bunim JJ (1965) The frequent occurrence of hypergammaglobulinemia and multiple tissue antibodies in Sjogren’s syndrome. Ann N Y Acad Sci 124:852–859

    Article  PubMed  CAS  Google Scholar 

  6. Whittingham S, McNeilage J, Mackay IR (1985) Primary Sjogren’s syndrome after infectious mononucleosis. Ann Intern Med 102:490–493

    PubMed  CAS  Google Scholar 

  7. Gilliam AC, Wood GS (2000) Cutaneous lymphoid hyperplasia. Semin Cutan Med Surg 19:133–141

    Article  PubMed  CAS  Google Scholar 

  8. Zandbelt MM, van den Hoogen FHJ (2002) Sjogren’s syndrome criteria. Ann Rheum Dis 61:948

    Article  PubMed  CAS  Google Scholar 

  9. Hansmann ML, Kikuchi M, Wacker HH, Radzun HJ, Nathwani BN, Hesse K, Parwaresch MR (1992) Immunohisto-chemical monitoring of plasmacytoid cells in lymph node sections of Kikuchi-Fujimoto disease by a new pan-macrophage antibody Ki-M1P. Hum Pathol 23:676–680

    Article  PubMed  CAS  Google Scholar 

  10. Skelton HG, Smith KL (1998) Extranodal multicentric Castleman’s disease with cutaneous involvement. Mod Pathol 11:93–98

    PubMed  CAS  Google Scholar 

  11. Yoshida H, Hara Y, Sato T, Inuo G, Arai J, Yumura W, Nihei H, Sugino N (1989) A case of idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmuno-globulinemia presenting plasmacytic infiltration and IgA deposition in the kidney (in Japanese). Nippon Naika Gakkai Zasshi 78:1324–1328

    PubMed  CAS  Google Scholar 

  12. Sallah S, Gagnon GA (1998) Angioimmunoblastic lymphadenopathy with dysproteinemia: emphasis on pathogenesis and treatment. Acta Haematol 99:57–64

    Article  PubMed  CAS  Google Scholar 

  13. Rijlaarsdam JU, Nieboer C, de Vries E, Willemze R (1990) Characterization of the dermal infiltrates in Jessner’s lymphocytic infiltrate of the skin, polymorphous light eruption and cutaneous lupus erythematosus: differential diagnostic and pathogenetic aspects. J Cutan Pathol 17:2–8

    Article  PubMed  CAS  Google Scholar 

  14. Schmid U, Eckert F, Griesser H, Steinke C, Cogliatti SB, Kaudewitz P, Lennert K (1995) Cutaneous follicular lymphoid hyperplasia with monotypic plasma cells. Clinicopathologic study of 18 patients. Am J Surg Pathol 19:12–20

    Article  PubMed  CAS  Google Scholar 

  15. Ceballos KM, Gascoyne RD, Martinka M, Trotter MJ (2002) Heavy multinodular cutaneous lymphoid infiltrates: clinicopathologic features and B-cell clonality. J Cut Pathol 29:159–167

    Article  CAS  Google Scholar 

  16. McCurley TL, Collins RD, Ball E, Collins RD (1990) Nodal and extranodal lymphoproliferative disorders in Sjogren’s syndrome: a clinico-pathological study. Human Pathol 21:482–492

    Article  CAS  Google Scholar 

  17. Yokoyama T, Miyazawa K, Otawa M, Kawakubo K, Kuriyama Y, Serizawa H, Mukai K, Ohyashiki K (2003) Spontaneous regression of MALT lymphoma of the hard palate accompanied by Sjogren syndrome (in Japanese). Rinsho Ketsueki 44:468–470

    PubMed  Google Scholar 

  18. Imai Y, Yamakawa M (1996) Morphorogy, function and pathology of follicular dendritic cells. Pathol Int 46:807–833

    Google Scholar 

  19. Maitland N, Flint S, Scully C, Crean SJ (1995) Detection of cytomegalovirus and Epstein-Barr virus in labial salivary glands in Sjogren’s syndrome and non-specific sialadenitis. J Oral Pathol Med 24:293–298

    Article  PubMed  CAS  Google Scholar 

  20. Jarvis MA, Nelson JA (2002) Human cytomegalovirus persistence and latency in endothelial cells and macrophages. Curr Opin Microbiol 5:403–407

    Article  PubMed  CAS  Google Scholar 

  21. Han K, Kim Y, Kahng J, Lee J, Moon Y, Kang C, Shim S (1996) Studies of cytomegalovirus and Epstein-Barr virus in reactive histiocytic hyperplasia with hemophagocytosis. Acta Haematol 96:140–145

    Article  PubMed  CAS  Google Scholar 

  22. Hunziker L, Recher M, Macpherson AJ, Ciurea A, Freigang S, Hengartner H, Zinkernagel RM (2003) Hypergamma-globulinemia and autoantibody induction mechanisms in viral infection. Nat Immunol 4:343–349

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Yasuhiro Horiuchi.

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Horiuchi, Y., Hakugawa, J., Shimizu, K. et al. Massive cutaneous follicular lymphoid hyperplasia in a patient with the Sjögren syndrome: 7-year follow-up and immunohistochemical study. Rheumatol Int 26, 1044–1049 (2006).

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