Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2017.008
Autopsy and Case Reports
Article / Autopsy Case Report

Gastrointestinal cytomegalovirus disease in a patient with pemphigus vulgaris treated with corticosteroid and mycophenolate mofetil

Luiza Barbosa Oliveira, Celina Wakisaka Maruta, Denise Miyamoto, Fernanda Aburesi Salvadori, Claudia Giuli Santi, Valeria Aoki, Amaro Nunes Duarte-Neto

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Abstract

Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil. She was hospitalized because of a 1-month history of watery diarrhea and oral ulcers. Unfortunately, the patient died suddenly on the ward. The autopsy revealed a bilateral saddle pulmonary embolism, Gram-positive cocci bronchopneumonia, and gastrointestinal cytomegalovirus infection, causing extensive gastrointestinal mucosal ulcers.

Keywords

Autopsy; Cytomegalovirus; Opportunistic Infections; Pemphigus

References

1. Smeeth L, Cook C, Thomas S, Hall AJ, Hubbard R, Vallance P. Risk of deep vein thrombosis and pulmonary embolism after acute infection in a community setting. Lancet. 2006;367(9516):1075-9. PMid:16581406. http://dx.doi.org/10.1016/S0140-6736(06)68474-2.

2.  Schmidt M, Horvath-Puho E, Thomsen RW, Smeeth L, Sørensen HT. Acute infections and venous thromboembolism. J Intern Med. 2012;271(6):608-18. PMid:22026462. http://dx.doi.org/10.1111/j.1365- 2796. 2011.02473.x.

3.  Alikhan R, Peters F, Wilmott R, Cohen AT. Fatal pulmonary embolism in hospitalized patients: a necropsy review. J Clin Pathol. 2004;57(12):1254-7. PMid:15563663. http://dx.doi.org/10.1136/jcp.2003.013581.

4.  Knudson RM, Kalaaji AN, Bruce AJ. The management of mucous membrane pemphigoid and pemphigus.Dermatol Ther (Heidelb). 2010;23(3):268-80. PMid:20597945. http://dx.doi.org/10.1111/j.1529-8019.2010.01323.x.

5.  Migita K, Arai T, Ishizuka N, et al. Rates of serious intracellular infections in autoimmune disease patients receiving initial glucocorticoid therapy. PLoS One. 2013. 8(11):e78699. PMid:24260127. http://dx.doi.org/10.1371/journal.pone.0078699.

6.  Song ATWS, Abdala E, Bonazzi PR, Bacchella T, Machado MC. Does mycophenolate mofetil increase the risk of cytomegalovirus infection in solid organ transplant recipients?: a mini review. Braz J Infect Dis. 2006. 10(2):132-8. PMid:16878265. http://dx.doi.org/10.1590/S1413-86702006000200011.

7.  Santos JI. Nutrition, infection, and immunocompetence. Infect Dis Clin North Am. 1994;8(1):243-67. PMid:8021446.

8.  Ben Lagha N, Poulesquen V, Roujeau JC, Alantar A, Maman L. Pemphigus vulgaris: a case-based update. J Can Dent Assoc. 2005;71(9):667-72. PMid:16271165.

9.  Mimouni D, Bar H, Gdalevich M, Katzenelson V, David M. Pemphigus: analysis of epidemiological factors in 155 patients. J Eur Acad Dermatol Venereol. 2008. 22(10):1232-5. PMid:18482324. http://dx.doi.org/10.1111/j.1468-3083.2008.02786.x.

10.  Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus: an update. Arch Dermatol. 1996;132(2):203-

12.  PMid:8629830. http://dx.doi.org/10.1001/archderm.1996.03890260105016.

11.  Stanley JR. Therapy of pemphigus vulgaris. Arch Dermatol. 1999. 135(1):76-8. PMid:9923786. http://dx.doi.org/10.1001/archderm.135.1.76.

12.  Leshem YA, Gdalevich M, Ziv M, David M, Hodak E, Mimouni D. Opportunistic infections in patients with pemphigus. J Am Acad Dermatol. 2014;71(2):284-92.  PMid:24815564. http://dx.doi.org/10.1016/j.jaad.2014.03.020.

13.  Chams-Davatchi C, Valikhani M, Daneshpazhooh M, et al. Pemphigus: analysis of 1209 cases. Int J Dermatol. 2005;44(6):470-6. PMid:15941433. http://dx.doi.org/10.1111/j.1365-4632.2004.02501.x.

14.  Keith PJ, Wetter DA, Wilson JW, Lehman JS. Evidencebased guidelines for laboratory screening for infectious diseases before initiation of systemic immunosuppressive agents in patients with autoimmune bullous dermatoses. Br J Dermatol. 2014;171(6):1307-17. PMid:25130049. http://dx.doi.org/10.1111/bjd.13355.

15.  Lehman JS, Khunger M, Lohse CM. Infection in autoimmune bullous diseases: a retrospective comparative study. J Dermatol. 2013;40(8):613-9. PMid:23772962. http://dx.doi.org/10.1111/1346-8138.12175.

16.  Aki HE, Sotto MN, Sampaio SA. Post-mortem evaluation in endemic pemphigus foliaceus and pemphigus vulgaris. Med Cutan Ibero Lat Am. 1984;12(2):151-7. PMid:6374324.

17.  Saito K, Morita M, Enomoto K. Bronchiolitis obliterans with pemphigus vulgaris and Castleman’s disease of hyaline-vascular type: an autopsy case analyzed by computer-aided 3-D reconstruction of the airway lesions. Hum Pathol. 1997;28(11):1310-2. PMid:9385940. http://dx.doi.org/10.1016/S0046-8177(97)90208-2.

18.  Jauregui HO. Lymphomatoid granulomatosis after immunosuppression for pemphigus. Arch Dermatol. 1978. 114(7):1052-5. PMid:686725. http://dx.doi.org/10.1001/archderm.1978.01640190040014.

19.  Jong MD, Galasso GJ, Gazzard B, et al. Summary of the II International Symposium on Cytomegalovirus. Antiviral Res. 1998;39(3):141-62. PMid:9833956. http://dx.doi.org/10.1016/S0166-3542(98)00044-8.

20.  Almeida LNB, Azevedo RS, Amaku M, Massad E. Cytomegalovirus seroepidemiology in an urban community of São Paulo, Brazil. Rev Saude Publica. 2001;35(2):124-9.  PMid:11359197. http://dx.doi.org/10.1590/S0034-89102001000200004.

21.  Goodgame RW. Gastrointestinal cytomegalovirus disease. Ann Intern Med. 1993;119(9):924-35. PMid:8215005. http://dx.doi.org/10.7326/0003-4819-119. 9-199311010-00010.

22.  Orton DI, Orteu CH, Rustin MHA. Cytomegalovirusassociated gastric ulcer in an immunosuppressed patient with pemphigus vulgaris. Clin Exp Dermatol. 2001. 26(2):170-2. PMid:11298108. http://dx.doi.org/10.1046/j.1365-2230.2001.00787.x.

23.  Chiu H-Y, Chang C-Y, Hsiao C-H, Wang LF. Concurrent cytomegalovirus and herpes simplex virus infection in pemphigus vulgaris treated with rituximab and prednisolone. Acta Derm Venereol. 2013;93(2):200-1. PMid:22948546. http://dx.doi.org/10.2340/00015555-1429.

24.  Buckner FS, Pomeroy C. Cytomegalovirus disease of the gastrointestinal tract in patients without AIDS. Clin Infect Dis. 1993;17(4):644-56. PMid:8268345. http://dx.doi.org/10.1093/clinids/17.4.644.

25.  Hokama A, Taira K, Yamamoto Y, et al. Cytomegalovirus gastritis. World J Gastrointest Endosc. 2010;2(11):379-80.  PMid:21173917. http://dx.doi.org/10.4253/wjge.v2.i11.379.

26.  Oliveira-Baptista D, Janini MER, Fernandes NC, Santos N. Laboratory diagnosis of herpesvirus infections in patients with pemphigus vulgaris lesions. Intervirology. 2013. 56(4):231-6. PMid:23735576. http://dx.doi.org/10.1159/000349889.

27.  Feldmeyer L, Trüeb RM, French LE, Hafner J. Pitfall: pemphigus herpeticatus should not be confounded with resistant pemphigus vulgaris. J Dermatolog Treat. 2010. 21(5):311-3. PMid:19895326. http://dx.doi.org/10.3109/09546630903287452.

28.  Marzano AV, Tourlaki A, Merlo V, Spinelli D, Venegoni L, Crosti C. Herpes simplex virus infection and pemphigus. Int J Immunopathol Pharmacol. 2009;22(3):781-6. PMid:19822095. http://dx.doi.org/10.1177/039463200902200324.

29.  Nikkels AF, Delvenne P, Herfs M, Pierard GE. Occult herpes simplex virus colonization of bullous dermatitides. Am J Clin Dermatol. 2008;9(3):163-8. PMid:18429645. http://dx.doi.org/10.2165/00128071-200809030-00004.

30.  Brandão ML, Fernandes NC, Batista DP, Santos N. Refractory pemphigus vulgaris associated with herpes infection: case report and review. Rev Inst Med Trop Sao Paulo. 2011;53(2):113-7. PMid:21537760. http://dx.doi.org/10.1590/S0036-46652011000200010.

31.  Avalos-Peralta P, Herrera A, Ríos-Martín JJ, Pérez-Bernal AM, Moreno-Ramírez D, Camacho F. Localized Kaposi’s sarcoma in a patient with pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2006;20(1):79-83. PMid:16405614. http://dx.doi.org/10.1111/j.1468-3083.2005.01348.x.

32.  Halpern SM, Parslew R, Cerio R, Kirby JT, Sharpe GR. Kaposi’s sarcoma associated with immunosuppression for bullous pemphigoid. Br J Dermatol. 1997. 137(1):140-3. PMid:9274643. http://dx.doi.org/10.1111/j.1365-2133.1997.tb03718.x.


Publication date:
03/31/2017

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