Paraneoplastic Pemphigus (PNP) As a Presenting Finding in a Patient with Hodgkin's Lymphoma

We report a case of PNP in a patient who was subsequently diagnosed with classical Hodgkins Lymphoma mixed cellularity type. PNP is an autoimmune mediated blistering disorder that presents with skin lesions demonstrating characteristics of erythema multiforme and pemphigus vulgaris.

A 76 year-old Hispanic male presented to the Emergency department with intermittent low fevers of 4 months duration associated with progressive weakness and fatigue; he now had mouth ulcers of 1-week duration along with anorexia. Physical exam was significant for a temperature of 102.4F, bilateral conjunctivitis with mild white discharge. Labs included WBC 5,000/mm3, hemoglobin 9.5 gm/dL, platelets 99,000/mm3; differential showed lymphocytes 22%, monocytes 11%, eosinophils 3%, and myelocytes 9%. Oral examination revealed dry mucosa with bleeding perioral ulcers. An ulcer biopsy was obtained and demonstrated necrotic keratinocytes and necrotic neutrophilic inflammation consistent with PNP. Chest CT showed a multinodular goiter, enlarged mediastinal and hilar nodes with calcified granulomas bilaterally as well as a new noncalcified nodule in the right lung. PET scan revealed FDG avid lymph nodes in the left supraclavicular, hilar and left axilla areas as well as multiple regions in the spleen and retroperitoneal nodes. A left axillary node biopsy showed classical Hodgkins lymphoma of mixed cellularity type. The patient was started on prednisone 75 mg per day in divided doses and his oral ulcerations and clinical status improved after 2 days of therapy. Subsequently the patient was started on gemcitabine, vinorelbine and liposomal doxorubicin (GVD) chemotherapy for his malignancy.

Although PNP has been described as being associated with hematologic malignancies such as Non-Hodgkins Lymphoma, Chronic Lymphocytic Leukemia and Castleman's disease, it has rarely been demonstrated in association with classical Hodgkins disease. This case report highlights the need for a higher index of suspicion of underlying malignancy including classical Hodgkins disease when confronted with a patient presenting PNP.

No relevant conflicts of interest to declare.