A case of non-Hodgkin's lymphoma-associated pemphigus foliaceus and use of rituximab for treatment.

BACKGROUND: Pemphigus foliaceus (PF), an autoimmune skin disorder resulting in the formation of superficial blisters, is a rarely reported skin manifestation of non-Hodgkin's lymphoma (NHL). Typical therapies for PF in non-malignant cases include immunosuppressants such as corticosteroids. We report the case of a patient undergoing therapy for NHL who subsequently developed PF which resolved following treatment with rituximab, an anti-CD20 monoclonal antibody.

CASE REPORT: A 73-year-old Caucasian male who received a course of rituximab for NHL 3 years prior, presented with atypical urticarial lesions that developed into blisters. Pathological determination identified the lesions as PF. Traditional corticosteroid therapy failed to resolve the PF lesions. However, in this clinical situation, the patient responded to a repeat rituximab administration with complete resolution of the PF lesions.

DISCUSSION: Paraneoplastic pemphigus, the most serious form of pemphigus, is more commonly associated with malignancies, such as NHL, whereas PF is seen in non-malignant cases. Although our patient was undergoing therapy for a malignancy, the less severe pemphigus emerged and responded to a non-traditional therapy. As such, we recommend that consideration is given to alternative therapies, including monoclonal antibodies, in cases of PF with atypical presentations and pathology associated with malignancy.