Paraneoplastic pemphigus in a patient with follicular non-Hodgkin lymphoma

Paraneoplastic pemphigus is described as an autoimmune disease, which presents with polymorphous blistering eruption and mucocutaneous ulcerations in a patient with an underlying neoplasm, either malignant or benign. The cutaneous lesions of these patients are quite variable and consist of a mixture of blisters, erosions, and target lesions. The most frequently observed clinical feature of this disease is an intractable stomatitis, which is usually the earliest presenting sign, and is extremely resistant to therapy.

Herein, we report a 30-year-old woman with follicular non- Hodgkin's lymphoma who presented with severe erosions and ulcerations involving oropharyngeal and vaginal mucosa and lichenoid papules over both palms with typical histopathological and direct immunofluorescence findings of paraneoplastic pemphigus. The lesions were recalcitrant to conventional therapy with oral corticosteroids but significantly improved with plasmapheresis followed by dexamethasone and cyclophosphamide pulse therapy as adjuvant treatment.