Case of juvenile pemphigus vulgaris which responded to i.v. immunoglobulin therapy

Matsuda, Haruna; Okamoto, Osamu; Kohno, T.; Takahashi, Akio; Takeo, Naoko; Fujiwara, Sakuhei

doi:10.1111/j.1346-8138.2011.01354.x

Cited by 3 publications

(3 citation statements)

References 8 publications

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“…IVIg therapy has been reported to be effective in children with juvenile PV . It can be used as monotherapy or in combination with other systemic agents .…”

Section: Pemphigus Vulgaris In Children (Strength Of Recommendation Dmentioning

confidence: 99%

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British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017

et al. 2017

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“…IVIg therapy has been reported to be effective in children with juvenile PV . It can be used as monotherapy or in combination with other systemic agents .…”

Section: Pemphigus Vulgaris In Children (Strength Of Recommendation Dmentioning

confidence: 99%

“…It can be used as monotherapy or in combination with other systemic agents . IVIg is an attractive second‐line option for juvenile PV as the risks of thromboembolic events and renal failure are considered to be much less compared with adults …”

Section: Pemphigus Vulgaris In Children (Strength Of Recommendation Dmentioning

confidence: 99%

British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017

et al. 2017

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“…By contrast, the high cost of IVIG and possibility of cardiovascular events due to hyperviscosity syndrome make IVIG a choice for patients who have corticosteroid-refractory PV. 11 Based on the rationale that pemphigus is primarily an autoantibodydriven autoimmune disorder, therapies that deplete autoreactive B-cell clones have been investigated for the treatment of pemphigus. Rituximab, a monoclonal antibody directed against the CD20 antigen on B lymphocytes, has demonstrated efficacy for PV and pemphigus foliaceus.…”

Section: Discussionmentioning

confidence: 99%

Oral Ulcers as an Initial Presentation of Juvenile Pemphigus: A Case Report

Chen¹,

Mu²,

Tsai³

et al. 2016

Pediatrics & Neonatology

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Pemphigus vulgaris (PV) is an autoimmune disease in which the autoantibody, immunoglobulin G, is directed against the keratinocytes in the epidermis. The classic presentations of PV are flaccid vesicles or bullae over the oral mucosa, trunk, groin, and extremities. The age of onset is usually between 40 and 60 years, and cases of PV in children or adolescent patients are rare. Here, we present a 17-year-old boy who had painful oral ulcers for 3 months initially and bullae spreading to the whole body in the following days. Paraneoplastic pemphigus was another differential diagnosis due to the atypical appearance of the skin lesion. However, PV was confirmed by hematoxylin and eosin staining and immunofluorescence examination of the skin biopsy specimens. The patient had a good response to corticosteroid treatment and the immunosuppressive agent, rituximab.

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Consensus Based Indian Guidelines for the Management of Pemphigus Vulgaris and Pemphigus Foliaceous

De,

Mehta,

Shah

et al. 2024

Indian Dermatology Online Journal

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Pemphigus is an autoimmune blistering disorder characterized by the presence of intraepidermal blisters and erosions, primarily affecting the mucosa and/or skin. There are no established Indian guidelines for the management of pemphigus, and Western guidelines cannot be directly applied due to differences in clinicodemographic profiles, comorbidities, and resource limitations. These guidelines aim to provide Indian dermatologists with evidence-based and consensus-driven recommendations for the management of pemphigus vulgaris (PV) and pemphigus foliaceous (PF), taking into account the unique challenges posed by the Indian healthcare setting. The guidelines focus on the comprehensive management of PV and PF, addressing diagnosis, treatment, monitoring, and follow-up. It is intended for dermatologists working in both outpatient and inpatient settings across India. The first draft of the guidelines was prepared by the writing group and then reviewed by 19 national experts in pemphigus management, including inputs from allied specialties. Areas with limited evidence or anticipated variation in recommendations were subjected to rounds of voting, with responses categorized as “strongly agree,” “agree,” “neutral,” “disagree,” or “strongly disagree.” Suggestions were incorporated, and statements were revised until a mean agreement score of 4 or higher was reached across 16 key areas after four voting rounds. These guidelines offer a structured approach to managing pemphigus in India, addressing the need for region-specific recommendations that account for unique challenges such as resource constraints and specific comorbidities in Indian patients. They serve as a valuable resource for dermatologists treating pemphigus in a range of clinical settings.

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Case of juvenile pemphigus vulgaris which responded to i.v. immunoglobulin therapy

Cited by 3 publications

References 8 publications

British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017

British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017

Oral Ulcers as an Initial Presentation of Juvenile Pemphigus: A Case Report

Consensus Based Indian Guidelines for the Management of Pemphigus Vulgaris and Pemphigus Foliaceous

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