A resistant case of pemphigus gestationis successfully treated with cyclosporine

Özdemir, Özhan; Atalay, Cemal; Asgarova, Vusala; Ilgin, Bunyamin Ugur

doi:10.1556/1646.8.2016.1.3

Cited by 7 publications

(6 citation statements)

References 8 publications

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“…Azathioprine and dapsone were the second and third most commonly prescribed steroid-sparing medications, always given after delivery/abortion. On the other hand, cyclosporine, which was the fourth most frequently administered steroid-sparing drug, was successfully used also during pregnancy (14,15). Interestingly, rituximab was used in two persistent cases either during pregnancy to prevent disease flare (16) and after delivery (17).…”

Section: Evidence About Pemphigoid Gestationis Treatment Emerged Frommentioning

confidence: 99%

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

et al. 2020

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Background: Treatment regimens for pemphigoid gestationis (PG) are non-standardized, with most evidence derived from individual case reports or small series.Objectives: To systematically review current literature on treatments and clinical outcomes of PG and to establish recommendations on its therapeutic management.Methods: An a priori protocol was designed based on PRISMA guidelines. PubMed, Scopus, and Web of Science databases were searched for English-language articles detailing PG treatments and clinical outcomes, published between 1970 and March 2020.Results: In total, 109 articles including 140 PG patients were analyzed. No randomized controlled trials or robust observational studies detailing PG treatment were found. Systemic corticosteroids ± topical corticosteroids and/or antihistamines were the most frequently prescribed treatment modality (n = 74/137; 54%). Complete remission was achieved by 114/136 (83.8%) patients. Sixty-four patients (45.7%) were given more than one treatment modality due to side effects or ineffectiveness. Leaving aside topical corticosteroids as monotherapy ± antihistamines in patients with mild disease, systemic corticosteroids ± topical corticosteroids and/or antihistamines led to complete remission in the highest proportion of patients (83%), while steroid-sparing treatments ± topical corticosteroids and/or antihistamines were associated with the lowest proportion of flares (55.5%).Limitations: The review has been drafted based on a limited number of single case reports and small case series. Underreporting/underdiagnosis of patients with mild-to-moderate PG, partial/absent follow-up, absence of precise description of neonatal outcomes and lack of validated objective scores for measuring disease severity are other limitations of our study. Our systematic review was affected by publication bias.Conclusion: Systemic corticosteroids are the most frequently used treatment for PG. Whilst most patients achieve complete remission, many of them have refractory/persistent disease requiring multiple lines of therapy. Therefore, we provided an algorithm for PG treatment integrating the results of this systematic review with current knowledge available for bullous pemphigoid. High-quality studies will further help assess the effectiveness of different treatment options for PG.

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Section: Evidence About Pemphigoid Gestationis Treatment Emerged Frommentioning

confidence: 99%

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

et al. 2020

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“…Ozhan et al, treated 4 unresponsive cases of PV during pregnancy with topical and systemic glucocorticoids drugs, using cyclosporine (300mg/day) combined with prednisolone, had a good response on the treatment. In one of these cases, with persistent postnatal PV cyclosporine was used after intravenous immunoglobulin administration [7].…”

Section: Discussionmentioning

confidence: 99%

“…A recent review demonstrates the reliability of association between azathioprine and steroids.The use of immunosuppressive treatment should be carefully evaluated, because of the risk of having low weight babies, infections, adrenal insufficiency, and preterm delivery. Also, the literature presents some reports which recommend rituximab, cyclosporine intravenous immunoglobulin, dapsone and plasmapheresis as safety as well [6,7].…”

Section: Introductionmentioning

confidence: 99%

Management of Pemphigus Vulgaris in Pregnancy - Case Report

2019

JGRM

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Pemphigus vulgaris is a rare autoimmune bullous disease caused by producing autoantibodies directed against transmembrane glycoproteins of desmosomes that can lead to steric hindrance to homophilic adhesion of desmogleins. The development of pemphigus during the pregnancy is rare. The disruption of the Th1:Th2 balance in the autoimmune disease during the pregnancy plays a crucial role and explains why some studies have reported the exacerbation of disease, while others observe improvements during the pregnancy. We present aclinical case of a 31-year-oldprimigravida, with dichorionic - diamniotic twin pregnancyassociatedwith pemphigus vulgaris, being discovered 2 years ago, who went under treatment with Medrol 64mg/day and Imuran 50mg/day. She was admitted to the hospital at 31 weeks of gestation with non-systemic contractions of moderate intensity. The clinical findings are multiple erosions and blisters which have been accentuated during the second semester and have been remitted after delivery. Nowadays, the therapeutic management of the pemphigus during the pregnancy is not yet established and all of these cases are individual evaluated with the primary target to control the disease with the safety of the fetus.

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“…Continued monitoring of this patient population remains a key component to understanding the risk factors associated with cyclosporin exposure during pregnancy 15 . Cyclosporine treatment can be consid-ered antenatally when treating severe and persistent PG unresponsive to topical and systemic glucocorticoids 16 .…”

Section: Treatmentmentioning

confidence: 99%

A severe case of pemphigoid gestationis persisting after labour - case report and review of the literature

Herman¹,

Křepelka²,

Faridova³

et al. 2024

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub

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Background and Aim. Pemphigoid gestationis (PG) is a rare skin disease of pregnancy. Given its incidence in pregnant women, physicians and especially obstetricians may not encounter this diagnosis in their entire career. We find this to be a major problem and there is an obligation to report it in as much detail as possible along with recommended treatments with proven efficacy. Case report. We describe the case of a 27 year old patient who was referred to the dermatology department with severe dissemination of blisters in the 9th week of pregnancy. She was diagnosed with pemphigoid gestationis in her first pregnancy. High doses of corticosteroids were initiated but due to inadequate effect cyclosporine was added. The pregnancy was complicated with gestational diabetes. The patient gave birth in her 33rd week by caesarian section due to premature rupture of the membrane. Vesicles were seen on the newborn immediately after birth which diminished spontaneously over 2 weeks. Blisters were still seen on the patient 1 month after labor even with the combination of systemic corticosteroids with cyclosporine. Conclusion. PG is a rare dermatosis of pregnancy. The course of the disease can be severe, necessitating systemic therapy. As described in this patient, systemic corticosteroids may not be sufficient and adding another immunosuppressive treatment may be needed. If pemphigoid gestationis has occurred during a previous pregnancy it is advised to reconsider another pregnancy.

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A resistant case of pemphigus gestationis successfully treated with cyclosporine

Cited by 7 publications

References 8 publications

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis

Management of Pemphigus Vulgaris in Pregnancy - Case Report

A severe case of pemphigoid gestationis persisting after labour - case report and review of the literature

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