Management of pemphigus vulgaris: challenges and solutions

Gregoriou, Stamatis; Efthymiou, Ourania; Stefanaki, Christina; Rigopoulos, Dimitrios

doi:10.2147/ccid.s75908

Cited by 62 publications

(71 citation statements)

References 62 publications

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“…Our study confirms the efficacy of rituximab in patients with a difficult-to-treat pemphigus: 96.6% of our patients experienced partial or complete remission, similarly to the literature data. However, a complete clinical remission rate, documented by declining levels of anti-Dsg 1 and anti-Dsg 3, was lower than in other reported series (48.3% vs. 65-78%;Gregoriou, 2015;Wang, 2015).…”

contrasting

confidence: 71%

“…However, a complete clinical remission rate, documented by declining levels of anti-Dsg 1 and anti-Dsg 3, was lower than in other reported series (48.3% vs. 65-78%;Gregoriou, 2015;Wang, 2015).The debate regarding which protocol is optimal is still open. However, a complete clinical remission rate, documented by declining levels of anti-Dsg 1 and anti-Dsg 3, was lower than in other reported series (48.3% vs. 65-78%;Gregoriou, 2015;Wang, 2015).…”

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confidence: 72%

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confidence: 72%

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Rituximab therapy in pemphigus: A long-term follow-up

Loi

Magnano

Ravaioli

et al. 2018

Dermatologic Therapy

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group after a mean period of 18.2 months (p = .0588), managed with systemic immunosuppressants or rituximab re-infusion (500 mg).Our study confirms the efficacy of rituximab in patients with a difficult-to-treat pemphigus: 96.6% of our patients experienced partial or complete remission, similarly to the literature data. However, a complete clinical remission rate, documented by declining levels of anti-Dsg 1 and anti-Dsg 3, was lower than in other reported series (48.3% vs. 65-78%;Gregoriou, 2015;Wang, 2015).The debate regarding which protocol is optimal is still open. Apparently the rheumatologic protocol is the most appropriate, as pemphigus is an autoimmune disease in which B-cell clone eradication is not necessary and unsafe. Our study is the first to document a significantly greater efficacy of the oncologic protocol: despite similar remission rates among the two groups, the oncologic protocol showed better results in terms of complete clinical remission and shorter relapse-free score, without enhanced adverse effects. Remission depends on number of parameters. In our series, no significant differences were observed among disease severity. Actually, the patients treated with the oncologic protocol presented unfavorable prognostic factors, as more past treatments (mean 5.4 vs. 2.5, p < .0001) and longer disease duration (mean 60.8 vs. 39.7 months, nonstatistically significant).Our preliminary data, although needing to be confirmed by further studies, suggest that oncologic dosing protocol may be preferred to rheumatologic protocol in pemphigus patients requiring a secondline therapy.

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confidence: 72%

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Rituximab therapy in pemphigus: A long-term follow-up

Loi

Magnano

Ravaioli

et al. 2018

Dermatologic Therapy

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“…1,2 There seems to be racial predilection in this disease towards Ashkenazi Jews, individuals of Mediterranean and South Asian origins. [1][2][3] Also, rare familial cases had been reported in literature. 3 Genetic predilection is present in this disease which is related to Human Leukocyte antigen (HLA) class 2 phenotypes like HLA DR4, DRw14 and DQB.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3] Also, rare familial cases had been reported in literature. 3 Genetic predilection is present in this disease which is related to Human Leukocyte antigen (HLA) class 2 phenotypes like HLA DR4, DRw14 and DQB. 4 This phenotype seems to be present in other autoimmune diseases as well.…”

Section: Introductionmentioning

confidence: 99%

Juvenile Pemphigus Vulgaris as a Differential Diagnosis for Chronic Generalized Oral Ulceration in an Adolescent: A Case Report and a Review of Literature

Hettiarachchi¹,

Jayasinghe²,

Gunasena³

et al. 2018

International Journal of Experimental Dental Science

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Pemphigus vulgaris (PV) is an autoimmune disorder involving the mucocutaneous tissues. Pathogenesis of this disease causes auto-antibodies against desmogleins in desmosomes which leads to intraepithelial blister formation. It is a rare but potentially life-threatening disease with a prevalence of 1 to 9 per 1 x 10 9 . The disease has an equal sex predilection and commonly occurs in the 5th and 6th decade of life. Clinically, oral lesions are more evident than skin lesions. Diagnosis of the disease is by clinicopathological correlation with the definitive diagnosis requiring immunofluorescent investigations. The mainstay of treatment involves immunosuppression through the use of corticosteroids and other steroid-sparing agents like dapsone, azathioprine, and methotrexate. Presentation of this entity in an adolescent is a rare occurrence, and this may lead to misdiagnosis of the condition. Quality of life of these patients can be improved by controlling the disease through early diagnosis and necessary management. In this article, we report a case of a 15-year-old male patient diagnosed with Juvenile PV (JPV) and discuss the possibility of pemphigusvulgaris as a differential diagnosis for chronic generalized oral ulceration in an adolescent with a brief review of the literature.

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Failure of double filtration plasmapheresis to treat severe pemphigus vulgaris: A case report

Liu

Wang

2022

J of Clinical Apheresis

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Pemphigus vulgaris (PV) is a chronic, mucocutaneous, autoimmune bullous disease. Double filtration plasmapheresis (DFPP) may be effective when PV fails to be controlled by conventional corticosteroid treatment. The patient was a 64-year-old man with erythema, blisters, and erosions on his head, face, mouth, trunk, limbs, and scrotum for over a month. He was diagnosed with severe PV, and the original rash area continued to expand after treatment with systemic corticosteroids, immunosuppressants, and intravenous immunoglobulin, with massive exudate and ≥5 new blisters and macules still occurring daily. Subsequently, the patient completed three sessions of DFPP. After the first DFPP, the original erosion surface exudate was significantly reduced and gradually healed. After the second DFPP, the erosion area and exudate increased compared with the previous one. After the third DFPP, the rash did not improve further and had a tendency to continue to progress. During the entire three sessions of DFPP, the patient had new blisters and bullae on his limbs every day. The Nikolsky's sign of the limbs turned negative at the initial stage, and then the trunk and limbs Nikolsky's sign became positive again.The titer of autoantibodies did not decrease significantly after the plasmapheresis. The patient eventually died of secondary lung infection and septic shock.

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Management of pemphigus vulgaris: challenges and solutions

Cited by 62 publications

References 62 publications

Rituximab therapy in pemphigus: A long-term follow-up

Rituximab therapy in pemphigus: A long-term follow-up

Juvenile Pemphigus Vulgaris as a Differential Diagnosis for Chronic Generalized Oral Ulceration in an Adolescent: A Case Report and a Review of Literature

Failure of double filtration plasmapheresis to treat severe pemphigus vulgaris: A case report

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