Steroid as a Saviour and Culprit in Pemphigus Vulgaris Therapy: A Rare
Case Series

Deepthi      Avvaru; Raushan   Kumar   Chaudhary; Bhavana      Doshi; Ramesh      Bhandari

doi:10.2174/1574885518666230816095340

Abstract

Introduction: Pemphigus is a group of autoimmune dermatological life-threatening diseases of which Pemphigus Vulgaris (PV) is the most common (1-5 cases/million/year) with a mortality rate of 60-90%. PV is generally characterized by painful oral erosions, along with or without cutaneous lesions, as blisters are often misdiagnosed. The management strategy of PV includes steroids along with adjuvant therapy.

Case Presentation: We report the case of three female patients with a mean age of 30.67 years who were diagnosed with pemphigus vulgaris and was on steroid therapy for a long duration. The longterm steroid therapy in all these three patients resulted in adverse drug reactions such as Cushing syndrome, oral and vaginal candidiasis. Although the steroid was found to cause ADR among these patients, the treatment with steroids and rituximab was continued. The withdrawal of steroids results in flare-up conditions, whereas the addition of rituximab serves as a steroid sparring partner in the management of PV. However, premedication was administered before the infusion of rituximab to prevent potential toxicity associated with rituximab. Further, administration of pulse therapy over oral dosing of steroids lowers the risks of ADR and long-term complications associated with steroids.

Conclusion: As PV requires the management with high doses of steroids along with the immunosuppressant, patients are more prone to developing drug-related problems. Hence, the rigorous monitoring of the therapy is vital to prevent adverse effects of long-term therapy. Clinical pharmacists and pharmacologists are the most suitable pillar of the healthcare system for drug therapy management.

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[1]
Kridin K, Schmidt E. Epidemiology of pemphigus. JID Innovations  2021; 1(1): 100004.
 [http://dx.doi.org/10.1016/j.xjidi.2021.100004] [PMID: 34909708]

[2]
Rehman A, Huang Y, Wan H. Evolving mechanisms in the pathophysiology of pemphigus vulgaris: a review emphasizing the role of desmogle in 3 in regulating p53 and the yes-associated protein. Life  2021; 11(7): 621.
 [http://dx.doi.org/10.3390/life11070621] [PMID: 34206820]

[3]
Porro AM, Seque CA, Ferreira MCC, Enokihara MMSS. Pemphigus vulgaris. An Bras Dermatol  2019; 94(3): 264-78.
 [http://dx.doi.org/10.1590/abd1806-4841.20199011] [PMID: 31365654]

[4]
Kumar Chaudhary R, Bhandari R, Doshi B, Karoli SS, Spoorthi Marripalli S, Ganachari MS. gefitinib induced pemphigus vulgaris with pride complex. J Oncol Pharm Pract  2022; 28(6): 1465-73.
 [http://dx.doi.org/10.1177/10781552221076755] [PMID: 35102778]

[5]
Popescu IA, Statescu L, Vata D, et al. Pemphigus vulgaris: Approach and management. Exp Ther Med  2019; 18(6): 5056-60.
 [PMID: 31819769]

[6]
Petzl-Erler ML. Beyond the HLA polymorphism: A complex pattern of genetic susceptibility to pemphigus. Genet Mol Biol  2020; 43(3): e20190369.
 [http://dx.doi.org/10.1590/1678-4685-gmb-2019-0369] [PMID: 32639508]

[7]
Kridin K, Sagi S, Bergman R. Mortality and cause of death in patients with pemphigus. Acta Derm Venereol  2017; 97(5): 607-11.
 [http://dx.doi.org/10.2340/00015555-2611] [PMID: 28093595]

[8]
Hertl M, Jedlickova H, Karpati S, et al. Pemphigus. s2 guideline for diagnosis and treatment: Guided by the european dermatology forum (edf) in cooperation with the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol  2015; 29(3): 405-14.
 [http://dx.doi.org/10.1111/jdv.12772] [PMID: 25338479]

[9]
Min KH, Rhee CK, Jung JY, Suh MW. Characteristics of adverse effects when using high dose short term steroid regimen. Korean J Audiol  2012; 16(2): 65-70.
 [http://dx.doi.org/10.7874/kja.2012.16.2.65] [PMID: 24653873]

[10]
Paradkar S. Reported adverse drug reactions during the use of corticosteroids in a tertiary care hospital. Ther Innov Regul Sci  2019; 53(1): 128-31.
 [http://dx.doi.org/10.1177/2168479018776262] [PMID: 29759019]

[11]
Chen DM, Odueyungbo A, Csinady E, et al. French study group on autoimmune bullous diseases. Rituximab is an effective treatment in patients with pemphigus vulgaris and demonstrates a steroid‐sparing effect. Br J Dermatol  2020; 182(5): 1111-9.
 [http://dx.doi.org/10.1111/bjd.18482] [PMID: 31487383]

[12]
Joly P, Horvath B, Patsatsi A, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol  2020; 34(9): 1900-13.
 [http://dx.doi.org/10.1111/jdv.16752] [PMID: 32830877]

[13]
Yasir M, Goyal A, Sonthalia S. Corticosteroid Adverse EffectsStatPearls. Treasure Island, FL: StatPearls Publishing 2022.

[14]
Farhan MA, Moharram AM, Salah T, Shaaban OM. Types of yeasts that cause vulvovaginal candidiasis in chronic users of corticosteroids. Med Mycol  2019; 57(6): 681-7.
 [http://dx.doi.org/10.1093/mmy/myy117] [PMID: 30544194]

[15]
Jelti L, Cordel N, Gillibert A, et al. French study group on autoimmune blistering diseases. Incidence and mortality of pemphigus in france. J Invest Dermatol  2019; 139(2): 469-73.
 [http://dx.doi.org/10.1016/j.jid.2018.07.042] [PMID: 30236611]

[16]
Murrell DF, Peña S, Joly P, et al. Diagnosis and management of pemphigus: Recommendations of an international panel of experts. J Am Acad Dermatol  2020; 82(3): 575-585.e1.
 [http://dx.doi.org/10.1016/j.jaad.2018.02.021] [PMID: 29438767]

[17]
Aryanian Z, Balighi K, Daneshpazhooh M, et al. Rituximab exhibits a better safety profile when used as a first line of treatment for pemphigus vulgaris: A retrospective study. Int Immunopharmacol  2021; 96: 107755.
 [http://dx.doi.org/10.1016/j.intimp.2021.107755] [PMID: 34162136]

[18]
Pappachan JM, Hariman C, Edavalath M, Waldron J, Hanna FW. Cushing’s syndrome: A practical approach to diagnosis and differential diagnoses. J Clin Pathol  2017; 70(4): 350-9.
 [http://dx.doi.org/10.1136/jclinpath-2016-203933] [PMID: 28069628]

[19]
Nieman LK. Recent updates on the diagnosis and management of cushing’s syndrome. Endocrinol Metab  2018; 33(2): 139-46.
 [http://dx.doi.org/10.3803/EnM.2018.33.2.139] [PMID: 29947171]

[20]
Barbot M, Zilio M, Scaroni C. Cushing’s syndrome: Overview of clinical presentation, diagnostic tools and complications. Best Pract Res Clin Endocrinol Metab  2020; 34(2): 101380.
 [http://dx.doi.org/10.1016/j.beem.2020.101380] [PMID: 32165101]

[21]
Xiao J, Xu G, de Hoog S, Qiao J, Fang H, Li Y. Oral prevalence of Candida species in patients undergoing systemic glucocorticoid therapy and the antifungal sensitivity of the isolates. Infect Drug Resist  2020; 13: 2601-7.
 [http://dx.doi.org/10.2147/IDR.S262311] [PMID: 32801797]

[22]
Coleman JJ, Pontefract SK. Adverse drug reactions. Clin Med  2016; 16(5): 481-5.
 [http://dx.doi.org/10.7861/clinmedicine.16-5-481] [PMID: 27697815]

[23]
Hopkins RL, Leinung MC. Exogenous Cushing’s syndrome and glucocorticoid withdrawal. Endocrinol Metab Clin North Am  2005; 34(2): 371-84. ix
 [http://dx.doi.org/10.1016/j.ecl.2005.01.013] [PMID: 15850848]

[24]
Kridin K. Emerging treatment options for the management of pemphigus vulgaris. Ther Clin Risk Manag  2018; 14: 757-78.
 [http://dx.doi.org/10.2147/TCRM.S142471] [PMID: 29740210]

[25]
Esmaili N, Mortazavi H, Noormohammadpour P, et al. Pemphigus vulgaris and infections: A retrospective study on 155 patients. Autoimmune Dis  2013; 2013: 1-5.
 [http://dx.doi.org/10.1155/2013/834295] [PMID: 23844280]

[26]
Gheisari M, Faraji Z, Dadras MS, et al. Methylprednisolone pulse therapy plus adjuvant therapy for pemphigus vulgaris: an analysis of 10 years’ experience on 312 patients. Dermatol Ther  2019; 32(5): e13057.
 [http://dx.doi.org/10.1111/dth.13057] [PMID: 31400243]

[27]
Mustafi S, Sinha R, Hore S, Sen S, Maity S, Ghosh P. Pulse therapy: Opening new vistas in treatment of pemphigus. J Family Med Prim Care  2019; 8(3): 793-8.
 [http://dx.doi.org/10.4103/jfmpc.jfmpc_114_19] [PMID: 31041203]

[28]
Hassan I, Sameem F, Mannan Masood Q, Majid M, Abdullah Z. Masood Ahmad q. Non comparative study on various pulse regimens (DCP, DAP and DMP) in pemphigus: Our experience. Indian J Dermatol  2014; 59(1): 30-4.
 [http://dx.doi.org/10.4103/0019-5154.123487] [PMID: 24470657]

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DOI https://dx.doi.org/10.2174/1574885518666230816095340	Print ISSN 1574-8855
Publisher Name Bentham Science Publisher	Online ISSN 2212-3903

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Steroid as a Saviour and Culprit in Pemphigus Vulgaris Therapy: A Rare Case Series

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