Rosai-Dorfman Disease With Pure and Multifocal Cutaneous Lesions: A Case Report
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Abstract
A 52‐year‐old woman developed progressive infiltrated purple and hyperpigmented cutaneous lesions in the face, thighs, armpits, chest, and abdomen evolving forone year. Histopathological examination showed large histiocytes exhibiting intact inflammatory cells in their cytoplasm (emperipolesis). Immunohistochemical analyses showed that the histiocyte population was positive for S100 and CD68, but negative for CD1a. Based on the clinical, histopathological, and immunohistochemical findings, we made the diagnosis of Rosai Dorfman disease (RDD). Our patient didn’t manifest any other extra-cutaneous involvement and all the biological and radiological investigations were normal. This form of pure cutaneous RDD (P-CRDD) with multifocal lesions has been rarely reported. RDD is very rare and hardly recognized in the absence of lymphadenopathy. The diagnosis of this entity involves a combination of histology and immunohistochemistry. To date, there is no standard treatment.
1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy: a newly recognized benign clinicopathological entity. Arch Pathol1969;87:63-70.
2. Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy: a pseudolym- phomatous benign disorder. Analysis of 34 cases. Cancer 1972;30:1174-88.
3. Goyal A, Mittal A. Destombes–Rosai Dorfman Disease: A Rare Case Report. J Maxillofac Oral Surg 2011;10:173-5.
4. Galicier L, Boutboul D, Oksenhendler É, Fieschi C, Meignin V. Histiocytosesinusale de Rosai-Dorfman. Presse Med 2016;46:107-16.
5. Galicier L, Fieschi C, Clauvel J. Histiocytosesinusale de Rosai-Dorfman. Press Medicale2007;36:1669-75.
6. Khoo JJ, Rahmat BO. Cutaneous Rosai-Dorfman disease. Malays J Pathol2007;29:49-52.
7. Nicolae-iustin B, Mihaela B, Daniel C. Purely cutaneous Rosai-Dorfman disease: a true clinical diagnosis challenge. ARS Med Tomitana 2018;4:168-73.
8. Brenn T, Calonje E, GranterSR,Leonard N, Grayson W, Fletcher CD, et al. Cutaneous Rosai-Dorfman disease is a distinct clinical entity. Am J Dermatopathol2002;24:385-91.
9. Wang K, Chen W, Liu H, Huang C, Lee W, Hu C. Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum and evolution of 21 lesions in six patients. Br J Dermatol 2006;154:277-86.
10. Ahmed A, Crowson N, Magro CM. A comprehensive assessment of cutaneous Rosai-Dorfman disease. Ann DiagnPathol2019;40:166-73.
11. Kong YY, Kong JC, Shi DR, Lu HF, Zhu XZ, Wang J. Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol2007;31:341-50.
12. Scheel MM, Rady PL, Tyring SK, Pandya AG. Sinus histiocytosis with massive lymphadenopathy: presentation as giant granuloma annulare and detection of human herpesvirus 6. J Am Acad Dermatol 1997;37:643-6.
13. Luppi M, Barozzi P, Garber R, Maiorana A, Bonacorsi G, Artusi T, et al. Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. Am J Pathol1998;153:815-23.
14. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 1992;166:291-5.
15. Fayne R, González I, Rengifo SS, Vega F, Cho-vega JH. Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant. Ann DiagnPathol 2019;151446.
16. Radzun HJ, Kreipe H, Zavazava N, Hansmann ML, Parwaresch MR. Diversity of the human monocyte/macrophage system as detected by monoclonal antibodies. J Leukoc Biol 1988;43:41-50.
17. Morgan NV, Morris MR, Cangul H, Gleeson D, Straatman-Iwanowska A, Davies N, et al. Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease. PLoS Genet 2010;6:e1000833.
18. Maric I, Pittaluga S, Dale JK, Niemela JE, Delsol G, Diment J, et al. Histologic features of sinus histiocytosis with massive lymphadenopathy in patients with autoimmune lymphoproliferative syndrome. Am J Surg Pathol2005;29:903-11.
19. Han M, Yue Z, Guoxing Z, Jie W, Chun L, Wei L. Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back. Ann Dermatol 2015;27:71-5.
20. Li X, Hong Y, An Q, Chen J, Wei H, Chen HD, et al. Successful Treatment of Rosai-Dorfman Disease With Low-Dose Oral Thalidomide. JAMA Dermatol2013;149:992-3.
21. Lu CI, Kuo TT, Wong WR, Hong HS. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am Acad Dermatol 2004;51:931-9.
22. Gebhardt C, Averbeck M, Paasch U, Ugurel S, Kurzen H, Stumpp P, et al. A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol 2009;145:571-4.
23. Chang HS, Son SJ, Cho KH, Lee JH. Therapeutic challenge of dapsone in the treatment of purely cutaneous Rosai–Dorfman disease. Clin Exp Dermatol. 2011;36:420-2.
24. Nasseri E, Belisle A, Funaro D. Rosai-Dorfman disease treated with methotrexate and low-dose prednisone: case report and review of the literature. J Cutan Med Surg. 2012;16:281-5.
2. Rosai J, Dorfman R. Sinus histiocytosis with massive lymphadenopathy: a pseudolym- phomatous benign disorder. Analysis of 34 cases. Cancer 1972;30:1174-88.
3. Goyal A, Mittal A. Destombes–Rosai Dorfman Disease: A Rare Case Report. J Maxillofac Oral Surg 2011;10:173-5.
4. Galicier L, Boutboul D, Oksenhendler É, Fieschi C, Meignin V. Histiocytosesinusale de Rosai-Dorfman. Presse Med 2016;46:107-16.
5. Galicier L, Fieschi C, Clauvel J. Histiocytosesinusale de Rosai-Dorfman. Press Medicale2007;36:1669-75.
6. Khoo JJ, Rahmat BO. Cutaneous Rosai-Dorfman disease. Malays J Pathol2007;29:49-52.
7. Nicolae-iustin B, Mihaela B, Daniel C. Purely cutaneous Rosai-Dorfman disease: a true clinical diagnosis challenge. ARS Med Tomitana 2018;4:168-73.
8. Brenn T, Calonje E, GranterSR,Leonard N, Grayson W, Fletcher CD, et al. Cutaneous Rosai-Dorfman disease is a distinct clinical entity. Am J Dermatopathol2002;24:385-91.
9. Wang K, Chen W, Liu H, Huang C, Lee W, Hu C. Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum and evolution of 21 lesions in six patients. Br J Dermatol 2006;154:277-86.
10. Ahmed A, Crowson N, Magro CM. A comprehensive assessment of cutaneous Rosai-Dorfman disease. Ann DiagnPathol2019;40:166-73.
11. Kong YY, Kong JC, Shi DR, Lu HF, Zhu XZ, Wang J. Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol2007;31:341-50.
12. Scheel MM, Rady PL, Tyring SK, Pandya AG. Sinus histiocytosis with massive lymphadenopathy: presentation as giant granuloma annulare and detection of human herpesvirus 6. J Am Acad Dermatol 1997;37:643-6.
13. Luppi M, Barozzi P, Garber R, Maiorana A, Bonacorsi G, Artusi T, et al. Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. Am J Pathol1998;153:815-23.
14. Levine PH, Jahan N, Murari P, Manak M, Jaffe ES. Detection of human herpesvirus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 1992;166:291-5.
15. Fayne R, González I, Rengifo SS, Vega F, Cho-vega JH. Primary cutaneous Rosai-Dorfman disease; a case-based review of a diagnostically and therapeutically challenging rare variant. Ann DiagnPathol 2019;151446.
16. Radzun HJ, Kreipe H, Zavazava N, Hansmann ML, Parwaresch MR. Diversity of the human monocyte/macrophage system as detected by monoclonal antibodies. J Leukoc Biol 1988;43:41-50.
17. Morgan NV, Morris MR, Cangul H, Gleeson D, Straatman-Iwanowska A, Davies N, et al. Mutations in SLC29A3, encoding an equilibrative nucleoside transporter ENT3, cause a familial histiocytosis syndrome (Faisalabad histiocytosis) and familial Rosai-Dorfman disease. PLoS Genet 2010;6:e1000833.
18. Maric I, Pittaluga S, Dale JK, Niemela JE, Delsol G, Diment J, et al. Histologic features of sinus histiocytosis with massive lymphadenopathy in patients with autoimmune lymphoproliferative syndrome. Am J Surg Pathol2005;29:903-11.
19. Han M, Yue Z, Guoxing Z, Jie W, Chun L, Wei L. Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back. Ann Dermatol 2015;27:71-5.
20. Li X, Hong Y, An Q, Chen J, Wei H, Chen HD, et al. Successful Treatment of Rosai-Dorfman Disease With Low-Dose Oral Thalidomide. JAMA Dermatol2013;149:992-3.
21. Lu CI, Kuo TT, Wong WR, Hong HS. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am Acad Dermatol 2004;51:931-9.
22. Gebhardt C, Averbeck M, Paasch U, Ugurel S, Kurzen H, Stumpp P, et al. A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy. Arch Dermatol 2009;145:571-4.
23. Chang HS, Son SJ, Cho KH, Lee JH. Therapeutic challenge of dapsone in the treatment of purely cutaneous Rosai–Dorfman disease. Clin Exp Dermatol. 2011;36:420-2.
24. Nasseri E, Belisle A, Funaro D. Rosai-Dorfman disease treated with methotrexate and low-dose prednisone: case report and review of the literature. J Cutan Med Surg. 2012;16:281-5.
| Files | ||
| Issue | Vol 61 No 4 (2023) | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/acta.v61i4.13177 | |
| Keywords | ||
| Rosai-Dorfman disease emperipolesis corticosterois methotrexate | ||
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How to Cite
1.
Chebbi D, Marzouk S, Ben Salah R, Chabchoub I, Snoussi M, Bahloul Z. Rosai-Dorfman Disease With Pure and Multifocal Cutaneous Lesions: A Case Report. Acta Med Iran. 2023;61(4):247-250.
