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Case Reports
. 2023 Mar 20;49(1):33.
doi: 10.1186/s13052-023-01442-7.

Paraneoplastic pemphigus and Castleman's disease: a case report and a revision of the literature

Mariangela Irrera  1 , Elena Bozzola  2 , Antonello Cardoni  3 , Rita DeVito  3 , Andrea Diociaiuti  4 , Maya El Hachem  4 , Katia Girardi  5 , Alessandra Marchesi  1 , Alberto Villani  1
Affiliations
Case Reports

Paraneoplastic pemphigus and Castleman's disease: a case report and a revision of the literature

Mariangela Irrera et al. Ital J Pediatr. .

Abstract

Background: In 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.

Conclusions: Reviewing 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.

Keywords: Case report; Castelman’s disease; Child; Paraneoplastic pemphigus; Therapy.

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Conflict of interest statement

The Authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Fig. 1
Fig. 1
a Erosive and painful oral mucositis with erosions; b tongue pyogenic granulomas
Fig. 2
Fig. 2
Skin vesicles on the face (a); targetoid crusted and erythematous lesions appeared on the face, neck, chest and shoulder (b, c)
Fig. 3
Fig. 3
Hyperchromic annular macules, sequelae of previous erythematous lesions, on the neck (a) and the left thigh (b)
Fig. 4
Fig. 4
Recurrent erosive mucositis (a), with gingivitis and hemorrhagic crusts on the lips; bilateral conjunctival hyperemia (c)
Fig. 5
Fig. 5
A Lymphoid follicle with atrophic germinal centre and numerous radially oriented penetrating hyalinized blood vessels (“lollipop” appereance). Note the perifollicular areas of sclerosis and the sclerotic blood vessels. B Lymphoid follicle with atrophic germinal centre and expanded mantle zone arranged in concentric rings (“onion skin” appearance). C CD20 immunostain shows many B follicles with atrophic germinal centres. D IgD immunostain shows the follicles mainly composed of mantle B-cells with a concentric arrangement. E CD21 immunostain shows the atrophic germinal centres mainly composed of hyperplastic follicular dendritic cells
Fig. 6
Fig. 6
Flow chart of the selected process
See this image and copyright information in PMC

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