Mucocutaneous Paraneoplastic Syndromes Associated With Hematologic Malignancies

Cutaneous paraneoplastic syndromes are a group of dermatoses that demonstrate a range of morphological and pathological findings. These syndromes may precede, be concurrent with, or follow the diagnosis of an underlying malignancy. Treatment of the malignancy is often associated with improvement in or resolution of the mucosal and cutaneous disease; however, this is not the case with paraneoplastic pemphigus (PNP). PNP is a rare syndrome that was first described in 1990, and it occurs almost exclusively in patients with lymphocytic neoplasms. Pulmonary manifestations occur in 30% to 40% of cases, and it is the only form of pemphigus that attacks epithelium other than squamous epithelium in an antibody-mediated fashion. The mortality rate for PNP associated with malignancy is greater than 90%. Treatment guidelines are not available, but case series point to the use of rituximab (Rituxan) as well as corticosteroids and various other immunomodulating agents. Here we present a diagnostic and treatment dilemma in a 39-year-old active-duty male who developed PNP in the setting of treatment with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) for grade 3 follicular lymphoma. This case report is followed by a review of the diagnosis and treatment of other cutaneous paraneoplastic syndromes that are associated with hematologic malignancies.