ArticleItalian journal of pediatrics2023
Paraneoplastic pemphigus and Castleman's disease: a case report and a revision of the literature.
Article in Italian journal of pediatrics, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 5 citations in OpenAlex.
- Oral Pemphigus in Children and Adolescents: A Narrative Review of Published Case Reports.Reports (MDPI) · 2026Review
- Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report.Oncology letters · 2026Article
- Paraneoplastic pemphigus in a patient with T-cell lymphoma: a case report.Annals of medicine and surgery (2012) · 2024Article
- Case Report: A rare pediatric case of paraneoplastic pemphigus associated with Castleman disease misdiagnosed as Behçets disease.Frontiers in pediatrics · 2024Article
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. CASE PRESENTATION: We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms.
conclusionsReviewing literature, 20 clinical cases have been described in the pediatric age. Diagnosis may be challenging, requiring an average of 3 months (range from 3 weeks to 2 years). In all cases, the initial manifestations were mucocutaneous lesions, especially oral lesions with poor response to conventional treatment. Systemic symptoms may be present as well. Therapeutical approach is still discussed with no consensus. Almost all patients received corticosteroids with poor response. Other drugs including azathioprine, methotrexate, cyclosporine and monoclonal antibodies have been evaluated for the control of the disease. Further studies and experimental trials urge to define the diagnostic criteria and therapy protocol.
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