Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series

Area of Science:

• Autoimmune diseases
• Pediatric oncology
• Rare diseases

Background:

• Paraneoplastic pemphigus (PNP) is a rare, severe autoimmune blistering disease.
• In pediatric cases, PNP frequently co-occurs with Castleman disease (CD).

Purpose of the Study:

• To describe clinical characteristics and outcomes of pediatric patients with PNP associated with CD.
• To identify prognostic factors for survival in this patient cohort.

Main Methods:

• Retrospective review of medical records for patients aged ≤22 years diagnosed with PNP and CD.
• Inclusion criteria: clinical and immunopathologic evidence of PNP; histopathologic diagnosis of CD.

Main Results:

• Five patients (2 children, 2 adolescents, 1 young adult) were included, with a mean age of 11.8 years.
• Common manifestations included oral and anogenital mucositis; four patients had unicentric CD (UCD) and one had multicentric CD (MCD).
• Survival was associated with UCD and complete CD resection; mortality was linked to MCD, incomplete resection, and bronchiolitis obliterans (BO).

Conclusions:

• Unicentric CD, particularly with complete resection, is associated with better outcomes in pediatric PNP.
• PNP should be considered in pediatric mucositis cases, as it can mimic other conditions.
• Prognosis is significantly impacted by the type of CD, surgical completeness, and presence of BO.