Unicentric Castleman disease with paraneoplastic pemphigus in a young woman: a case report

TL;DR

A young woman with a rare combination of unicentric Castleman disease and paraneoplastic pemphigus was successfully treated with surgery and steroid therapy.

Contribution

This case report highlights the successful surgical treatment and steroid therapy for a rare UCD-PNP combination in a young patient.

Findings

Complete surgical resection of the UCD mass led to resolution of PNP symptoms.

Two years post-surgery, there was no recurrence of lesions or the abdominal mass.

Combining surgery with a tapering steroid regimen improved patient prognosis.

Abstract

The case reports a rare case of a patient diagnosed with unicentric Castleman disease (UCD) with paraneoplastic pemphigus (PNP). According to the literature, surgery is considered the first-line therapy for such patient. Therefore, we collected and analyzed preoperative and postoperative data to evaluate the surgical outcome and prognosis.

A 28-year-old female patient had manifested erosions on oral mucosa and fingers for several months. Contrast-enhanced CT imaging revealed an abdominal mass. Histopathology of the lesions revealed lichenoid interface dermatitis. Direct immunofluorescence examination of the skin revealed a reticular deposition of IgG and C3 in the intercellular spaces of the epidermis. According to the latest diagnostic criteria, she was diagnosed with PNP. She underwent surgery at our hospital, during which the mass was completely resected. Postoperative pathology confirmed the mass as UCD, specifically of the hyaline vascular type. Following the surgery, the patient was concurrently treated with a tapering regimen of oral glucocorticoids. After 2 years of follow-up, there was no recurrence of the oral or hand erosions, and a repeat abdominal CT scan showed no evidence of mass recurrence.

Complete resection is the first-line therapy for UCD with PNP. Early and correct diagnosis is crucial for patient prognosis.

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