Unicentric Castleman disease-associated paraneoplastic pemphigus successfully managed with surgical resection and rituximab

We report a case of a woman in her early 20s who presented with chronic and refractory oro-oesophageal ulcers and significant weight loss. The common causes of refractory ulcers were ruled out. Biopsy from the oesophageal ulcers was suggestive of paraneoplastic pemphigus (PNP). The patient underwent a PET scan that revealed an incidental mass. PET-guided biopsy from the infrarenal mass was suggestive of unicentric Castleman disease (UCD). She was managed with local surgical resection, steroids and rituximab. The current case highlights that PNP can be the presenting manifestation of an underlying UCD. In addition, it also highlights the adjunctive role of rituximab in the management of UCD-associated PNP in patients with suboptimal response to steroids and surgical resection.