Profil Pemphigus Vulgaris di Instalasi Rawat Inap RSUD Dr. Moewardi Surakarta Periode Januari 2014-Desember 2019
DOI:
https://doi.org/10.56951/medicinus.v34i3.79Kata Kunci:
pemfigus vulgaris, akantolisis, immunosuppressant, corticosteroidAbstrak
Latar belakang: Pemphigus vulgaris adalah penyakit vesikobulosa autoimun yang mengancam jiwa, yang disebabkan oleh adanya akantolisis sel keratinosit akibat gangguan adhesi pada desmoglein 1 (Dsg1) dan desmoglein 3 (Dsg3). Gambaran klinis pada pemphigus vulgaris berupa lepuhan (bullae) kendur yang dapat mengenai seluruh bagian tubuh disertai keterlibatan mukosa. Penelitian ini bertujuan untuk mengetahui profil kasus pemphigus vulgaris di Instalasi Rawat Inap Rumah Sakit Dr. Moewardi Surakarta periode Januari 2014-Desember 2019. Metode: Penelitian dilakukan secara deskriptif retrospektif dengan melihat data rekam medis pasien pemphigus vulgaris di Instalasi Rawat Inap Rumah Sakit Dr. Moewardi Surakarta periode Januari 2014-Desember 2019. Hasil: Didapatkan 25 orang pasien pemphigus vulgaris dalam kurun waktu 6 tahun, dengan kelompok usia terbanyak adalah 51-60 tahun (36%) dan jenis kelamin terbanyak pada wanita (80%). Pemphigus vulgaris melibatkan mukokutan mulut pada 60% pasien dengan komorbiditas terbanyak adalah hiperglikemia (20%) dan kelainan laboratorium tersering adalah hipoalbuminemia (32%). Terapi yang diberikan pada 52% pasien berupa corticosteroid sistemik, sedangkan sisanya (48%) diberikan terapi kombinasi dengan immunosuppressant, antara lain mycophenolate mofetil (20%), cyclosporine (16%) dan azathioprine (12%). Kesimpulan: Pemphigus vulgaris sering dijumpai pada wanita rentang usia 51-60 tahun. Tata laksana kasus pemphigus vulgaris dilakukan dengan menggunakan terapi tunggal corticosteroid sistemik (52%) atau terapi kombinasi menggunakan agen immunosuppressant.
Referensi
Payne A, Stanley J. Pemphigus. Dalam: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, dkk., penyunting. Fitzpatrick’s dermatology. Edisi ke-9. New York: McGraw Hill Companies; 2019. h.909-33.
Kasperkiewicz M. Pemphigus. Nat Rev Dis Primers. 2018;3(1):1-40. DOI: https://doi.org/10.1038/nrdp.2017.26
Shah A, Sheiffert-Sinha K, Sirois D, Werth V, Rengarajan B, Zrnchik W, dkk. Development of disease registry for autoimmune bullous diseases: Initial analysis of the pemphigus vulgaris subset. Acta Derm Venereol. 2015;95(1):86-90. DOI: https://doi.org/10.2340/00015555-1854
Pietkiewicz P, Gornowicz-Porowska J, Bartkiewicz P, Bowszyc-Dmochowska M, Dmochowski M. Reviewing putative industrial triggering in pemphigus: Cluster of pemphigus in the area near the wastewater treatment plant. Postepy Dermatol Allergol. 2017;34(3):185-91. DOI: https://doi.org/10.5114/ada.2017.67840
Huang Y, Kuo C, Chen Y, Yang Y. Incidence, mortality and causes of deathof patients with pemphigus inTaiwan: A nationwide population-based study. J Investig Dermat. 2012;132(1):92-7. DOI: https://doi.org/10.1038/jid.2011.249
Wardhana M, Rusyati L. Prevalence and quality of life of pemphigus patients at Sanglah General Hospital Bali-Indonesia. Bali Med J. 2013;2(1):42-5.
Triana A. Laporan Kasus : Insidensi dan mortalitas pemfigus dan pemfigoid bulosa di Instalasi Rawat Inap RSUD Dr. Moewardi Surakarta periode Januari 2014-Desember 2016.
Aoki V, Fukumori L, Freitas E, Sousa J, Perigo A, Oliveira Z. Direct and indirect immunofluorescence. An Bras Dermatol. 2018;85(4):490-500. DOI: https://doi.org/10.1590/S0365-05962010000400010
Caplan A, Fett N, Werth V. Pemphigus. Dalam: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, dkk., penyunting. Fitpatrick's Dermatology. Edisi ke-9. New York: McGraw Hill; 2019. h.909-33.
Murrell D, Dick S, Ahmed A, Amagai M, Barnadas M, Borradori L, dkk. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. 2008;58(1):1043-6.
Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: Pemphigus and bullous pemphigoid. Arch Dermatol Res. 2014;1(1):1-9.
Nishioka T, Shimizu J, Iida R, Yamazaki S, Sakaguchi S. CD4+CD25+Foxp3+ T cells and CD4+CD25SFoxp3+ T cells in aged mice. J Immunol. 2006;176(1):6586-93. DOI: https://doi.org/10.4049/jimmunol.176.11.6586
Weiskopf D, Weinberger B, Grubeck-Lobenstein B. The aging of the immune system. Transpl Int. 22(1):1041-50. DOI: https://doi.org/10.1111/j.1432-2277.2009.00927.x
Haynes L, Maue A. Effects of aging on T cell function. Curr Opin Allergy Clin Immunol. 2009;21(1):414-7. DOI: https://doi.org/10.1016/j.coi.2009.05.009
Feliciani C, Toto P, Amerio P, Pour S, Coscione G, Shivji G, dkk. In vitro and in vivo expression of interleukin-1alpha and tumor necrosis factor-alpha mRNA in Pemphigus vulgaris: Interleukin-1alpha and tumor necrosis factor-alpha are Involved in acantholysis. J Invest Dermatol. 2000;114(1):71-7. DOI: https://doi.org/10.1046/j.1523-1747.2000.00835.x
Nanda A, Dvorak R, Al-Saeed K, Al-Sabah H, Alsaleh Q. Spectrum of autoimmune bullous diseases in Kuwait. Int J Dermatol. 2004;43(1):876-81. DOI: https://doi.org/10.1111/j.1365-4632.2004.02292.x
Olbrich M, Kunstner A, Witte M, Witte H, Busch H, fahnrich A. Genetics and omics analysis of autoimmune skin blistering diseases. Front immunol. 2019;10(1):1-14. DOI: https://doi.org/10.3389/fimmu.2019.02327
Javidi Z, Meibodi N, Nahidi Y. Epidemiology of pemphigus in Northeast Iran: A 10-year retrospective study. Indian J Dermatol. 2007;52(4):188-91. DOI: https://doi.org/10.4103/0019-5154.37723
Vodo D, Sarig O, Sprecher E. The genetics of pemphigus vulgaris. Front Med. 2018;5(226):1-7. DOI: https://doi.org/10.3389/fmed.2018.00226
Reis V, Toledo R, Lopez A, Diaz L, Martin J. UVB induced acantolysis inendemic pemphigus foliaceus (fogo selfagem) and pemphigus vulgaris. J Am Acad Dermatol. 2000;42(4):571-6. DOI: https://doi.org/10.1067/mjd.2000.104891
Kername M. A psychological study on stress among employed women and housewives and its management through progressive relaxation technique (PMRT) and mindfulness breathing. J Psychol Psychother. 2016;6(1):1-5.
Klarreich S. Work without stress: A practical guide to emotional and physical well-being on the job. New York: Brunner/Mazel Int;1990. h.1-168.
Bashir S, Harris G, Denman M, Blake D, Winyard P. Oxidative DNA damage and cellular sensitivity to oxidative stress in human autoimmune diseases. Ann Rheum Dis. 2008;52(9):659-66. DOI: https://doi.org/10.1136/ard.52.9.659
Lee S, Hong W, Kim S. Analysis of serum cytokine profile in pemphigus. Ann Dermatol. 2017;29(4):438-45. DOI: https://doi.org/10.5021/ad.2017.29.4.438
Shah A, Sinha A. Oxidative stress and autoimmune skin disease. Eur J Dermatol 2013;23(1):5-13. DOI: https://doi.org/10.1684/ejd.2012.1884
Zhu X, Pan J, Yu Z, Wang Y, Cai L, Zheng S. Epidemiologyof pemphigus vulgaris in the Northeast China: A 10-year retrospective study. J Dermatol. 2014;41(1):70-5. DOI: https://doi.org/10.1111/1346-8138.12286
Stanley J, Amagai M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med. 2006;355(17):1800-10. DOI: https://doi.org/10.1056/NEJMra061111
Nagao K, Udey M. Basic principles of immunologic diseases in skin (Pathophysiology of immunologic/inflammatory skin diseases). Dalam: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, dkk., penyunting. Fitzpatrick’s dermatology. Edisi ke-9. New York: McGraw Hill Companies; 2019. h.201-3.
Heelan K, Mahar A, Walsh S, Shear N. Pemphigus and associated comorbidities: A cros-sectional study. Clin Exp Dermatol. 2015;40(1): 593-9. DOI: https://doi.org/10.1111/ced.12634
Alavi A, Lowe J, Walsh S, Juurlink D, Mortaz-Hedjri S, Shear N. Corticosteroid-induced hyperglycemia is increased 10-fold in patients with pemphigus. Int J Dermatol. 2012;51(10):1248-52. DOI: https://doi.org/10.1111/j.1365-4632.2012.05470.x
Darjani A, Nickhah N, Emami M, Alizadeh N, Rafiei R, Eftekhari H, dkk. Assesment of the prevalence and risk factors associated with glucocorticoid-induced diabetes mellitus in Pemphigus vulgaris patients. Acta Med Iran. 2017;55(6):375-80.
Donichi A, Raval D, Saul M, Korytkowski M, DeVita M. Prevalence and predictors of corticosteroid-related hyperglycemia in hospitalized patients. Endocr Pract. 2006;12(1):358-62. DOI: https://doi.org/10.4158/EP.12.4.358
Kandhari K, Pasricha J. Study of proteins and electrolytes of serum and blister fluid in pemphigus. J Invest Dermatol. 1965;44(4):246-51. DOI: https://doi.org/10.1038/jid.1965.44
Porro A, Seque C, Ferreira M, Enokihara M. Pemphigus vulgaris. An Bras Dermatol. 2019;94(3):264-78. DOI: https://doi.org/10.1590/abd1806-4841.20199011
Falodun O, Ogunbiyi A. Dermatological Emergencies: Current Trends in Management. Ann Ib Postgrad Med. 2006;4(2):15-21. DOI: https://doi.org/10.4314/aipm.v4i2.55216
Shen A, Chowdhury S, Bandyopadhyay D. Inpatient dermatology: Characteristics of patients and admissions in a tertiary level hospital in Eastern India. Indian J Dermatol. 2016;61(5):561-4. DOI: https://doi.org/10.4103/0019-5154.190104
Pires C, Viana V, Araujo F, Muller S, Oliveira M, Carneiro F. Evaluation of cases of pemphigus vulgaris and pemphigus foliaceus from a reference service in Parastate, Brazil. An Bras Dermatol. 2014;89(4):556-61. DOI: https://doi.org/10.1590/abd1806-4841.20142679
Murrell D, Dick S, Ahmed A, Amagai M, Barnadas M, Borradori L, dkk. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. 2008;58(6):1043-6. DOI: https://doi.org/10.1016/j.jaad.2008.01.012
Gregoriou S, Efthymiou O, Stefanaki C, Rigopoulos D. Management of pemphigus vulgaris: Challenges and solutions. Clin Cosmet Investig Dermatol. 2015;8(1):521-7. DOI: https://doi.org/10.2147/CCID.S75908
Chams-Davatchi C, Esmaili N, Daneshpazhooh M, Valikhani M, Balighi K, Hallaji Z, dkk. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol. 2007;57(4):622-8. DOI: https://doi.org/10.1016/j.jaad.2007.05.024
Gheisari M, Faraji Z, Dadras M, Nasiri S, Robati R, Moravvej H, dkk. Methylprednisolone pulse therapy plus adjuvant therapy for pemphigus vulgaris: An analysis of 10 years' experience on 312 patients. Dermatol Ther. 2019;32(1):1-6. DOI: https://doi.org/10.1111/dth.13057
Shahidi-Dadras M, Karami A, Toosy P, Shafiyan A. Pulse versus oral methylprednisolone therapy in Pemphigus vulgaris. Arch Iranian Med. 2007;10(1):1-6.
Cholera M, Wu N. Management of pemphigus vulgaris. Adv Ther. 2016;33(1):910-58. DOI: https://doi.org/10.1007/s12325-016-0343-4
Caplan A, Fett N, Werth V. Glucocorticoids. Dalam: Kang S, Amagai M, Bruckner A, Enk A, Margolis D, McMichael A, dkk., penyunting. Fitpatrick's Dermatology. Edisi ke-9. New York: McGraw Hill; 2019. h.3382-94.
Akhtar S, Hasan M. Treatment of pemphigus: Local experience. J Pak Med Assoc. 1998;48(10):300-4.
Sukanjanapong S, Thongtan D, Kanokrungsee S, Suchonwanit P, Chanprapaph K. A Comparison of azathioprine and mycophenolate mofetil as adjuvant drugs in patients with Pemphigus: A retrospective cohort study. Dermatol Ther. 2019;10(1):179-89. DOI: https://doi.org/10.1007/s13555-019-00346-x
Ioannides D, Chrysomallis F, Bystryn J. Ineffectiveness of cyclosporine as an adjuvant to corticosteroid in the treatment of pemphigus. Arch Dermatol. 2000;136(1):868-72. DOI: https://doi.org/10.1001/archderm.136.7.868
Harman K, Brown D, Exton L, Groves R, Hampton P, Mustapa M, et al. British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017. Br Med J. 2017;177(1):1170-201. DOI: https://doi.org/10.1111/bjd.15930
Almugairen N, Hospital V, Bedane C, Duvert-Lehembre S, Picard D, Tronquoy A, dkk. Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium-and high-dose corticosteroids. J Am Acad Dermatol. 2013;69(1):583-8. DOI: https://doi.org/10.1016/j.jaad.2013.05.016
Lyakhovitsky A, Baum S, Scope A, Amichai B, Barzilai A, Rimer J, dkk. The impact of stratifying initial dose of corticosteroids by severity of pemphigus vulgaris on long-term disease severity. Int J Dermatol. 2011;50(1):1014-9. DOI: https://doi.org/10.1111/j.1365-4632.2010.04828.x
Unduhan
Terbitan
Bagian
Diterbitkan
Cara Mengutip
Lisensi
Hak Cipta (c) 2021 Eka Devinta Novi Diana, Wibisono Nugraha, Alfina Rahma, Frieda, Anindya Oktafiani, Rieska Widyaswari, Muhammad Eko Irawanto

Artikel ini berlisensi Creative Commons Attribution-NonCommercial 4.0 International License.