Effective Management of Pemphigus Vulgaris: A Case Report
--------------Case Report-----------
DOI:
https://doi.org/10.31580/pjmls.v5i2.2741Keywords:
Corticosteroids, Erosions, Immunosuppressant, Lesions, Pemphigus vulgaris, TreatmentAbstract
Pemphigus vulgaris is a unique chronic condition of bullous autoimmune dermatosis whose development and diagnosis is mostly unidentifiable. It is classified as a specific hypersensitivity type II reaction that initiates the antibodies against the desmosomes, which are the maculae adherentes of the skin also known as the adhering spots in common term. These adhering spots keep the certain layers and compartments of the skin intact. When the desmosomes are invaded by the antibodies, the intact layers of the skin separate out and the clinical picture resembles a blister. With the passage of time the condition becomes worse and without treatment and monitoring the blister type lesions increase in size and surface area of the body similar to the condition of severe burn. The prime focus in the treatment of pemphigus vulgaris is to control the infirmity, prevent relapses, avoid adverse events and complications associated with the broad use of the steroids and immunosuppressive agents. Systemic corticosteroids evidenced as the golden standard in the treatment regimens of the pemphigus vulgaris. Azathioprine and mycophenolate mofetil are considered as the choice of drug in the treatment of the pemphigus vulgaris as a steroid sparing agent. Rituximab is believed to be immensely efficacious in unmanageable and refracted types of pemphigus vulgaris when other remedies fail to control the disease. One of the most noticeable complications of the pemphigus vulgaris is the compromised quality of life due to the blister forming all over the body which aggravates the soreness and pain on the affected area leading to ulceration and formation of erosive lesions. If there is a recurrence of the disease then the steroids doses will be checked properly and if needed then the doses could also be increased according to the weight in safe limit thus augmenting the immunosuppressants in the body and find an alternate safe drug in concomitant therapy
References
1. Sivapathasundharam B. Shafer's Textbook of Oral Pathology-E Book. Elsevier Health Sciences; 2016 Jul 25.
Korman N. Pemphigus J. Am Acad dermat. 1998;18:1219-38.
Ahmed AR. Lymphocyte studies in Pemphigus. Arch Dermatol Res. 1998;271:111-5.
Ahmed A R , Moy R. Death in pemphigus. J. Am Acad Dermatol. 1982;7(2): 221-8.
Tsunoda K, Ota T, Saito M, Hata T, Shimizu A, Ishiko A, Yamada T, Nakagawa T, Kowalczyk AP, Amagai M. Pathogenic relevance of IgG and IgM antibodies against desmoglein 3 in blister formation in pemphigus vulgaris. The American journal of pathology. 2011;179(2):795-806.
Hammers CM, Stanley JR. Mechanisms of disease: pemphigus and bullous pemphigoid. Annual Review of Pathology: Mechanisms of Disease. 2016;11:175-97.
Kasperkiewicz M, Schmidt E, Zillikens D. Current therapy of the pemphigus group. Clinics in dermatology. 2012;30(1):84-94.
Ljubojević S, Lipozenčić J, Brenner S, Budimčić D. Pemphigus vulgaris: a review of treatment over a 19‐year period. Journal of the European Academy of Dermatology and Venereology. 2002;16(6):599-603.
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clinics in dermatology. 2011;29(4):432-6.
Baum S, Astman N, Berco E, Solomon M, Trau H, Barzilai A. Epidemiological data of 290 pemphigus vulgaris patients: a 29-year retrospective study. European Journal of Dermatology. 2016;26:382-7.
Kershenovich R, Hodak E, Mimouni D. Diagnosis and classification of pemphigus and bullous pemphigoid. Autoimmunity reviews. 2014;13(4-5):477-81.
Korman NJ. New and emerging therapies in the treatment of blistering diseases. Dermatologic clinics. 2000;18(1):127-37.
Carson PJ, Hameed A, Ahmed AR. Influence of treatment on the clinical course of pemphigus vulgaris. Journal of the American Academy of Dermatology. 1996 Apr 1;34(4):645-52.
Golchai J, Shams G. A familial case of pemphigus vulgaris. Medical journal of islamic Republic of Iran. 1993;6(4)297-8.
Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus: an update. Archives of dermatology. 1996 Feb 1;132(2):203-12.
Chams-Davatchi C, Daneshpazhooh M. Prednisolone dosage in pemphigus vulgaris. J Am Acad Dermatol. 2005;53(3):547.
Tabrizi MN, Chams-Davatchi C, Esmaeeli N, et al. Accelerating effects of epidermal growth factor on skin lesions of pemphigus vulgaris: a double-blind, randomized, controlled trial. J Eur Acad Dermatol Venereol. 2007;21(1):79-84.
Harman KE, Albert S, Black MM. Guidelines for the management of pemphigus vulgaris. British Journal of Dermatology. 2003;149(5):926-37.
El Tal AK, Posner MR, Spigelman Z, Ahmed AR. Rituximab: a monoclonal antibody to CD20 used in the treatment of pemphigus vulgaris. J Am Acad Dermatol. 2006;55(3):449-59.
Joly P, Maho-Vaillant M, Prost-Squarcioni C. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017;389 (10083):2031-2040.
El-Darouti M, Marzouk S, Abdel Hay R. The use of sulfasalazine and pentoxifylline (low-cost antitumour necrosis factor drugs) as adjuvant therapy for the treatment of pemphigus vulgaris: a comparative study. Br J Dermatol. 2009;161(2):313-9.
Sinha AA, Hoffman MB, Janicke EC. Pemphigus vulgaris: approach to treatment. European Journal of Dermatology. 2015;25:103-13.
Sharma VK, Khandpur S. Evaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgaris. Clin Exp Dermatol. 2013;38 (6):659-64.
Downloads
Published
Issue
Section
License
Copyright (c) 2022 Pak-Euro Journal of Medical and Life Sciences

This work is licensed under a Creative Commons Attribution 4.0 International License.




