Profil Pemphigus Vulgaris di Instalasi Rawat Inap RSUD Dr. Moewardi Surakarta Periode Januari 2014-Desember 2019

Penulis

  • Eka Devinta Novi Diana Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Wibisono Nugraha Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Alfina Rahma Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Frieda Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Anindya Oktafiani Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Rieska Widyaswari Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta
  • Muhammad Eko Irawanto Bagian Ilmu Kesehatan Kulit dan Kelamin, Fakultas Kedokteran, Universitas Sebelas Maret Surakarta/RSUD Dr. Moewardi, Surakarta

DOI:

https://doi.org/10.56951/medicinus.v34i3.79

Kata Kunci:

pemfigus vulgaris, akantolisis, immunosuppressant, corticosteroid

Abstrak

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

Diterbitkan

01-12-2021

Unduhan

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Cara Mengutip

[1]
Profil Pemphigus Vulgaris di Instalasi Rawat Inap RSUD Dr. Moewardi Surakarta Periode Januari 2014-Desember 2019. MEDICINUS 2021;34:27-34. https://doi.org/10.56951/medicinus.v34i3.79.