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Paraneoplastic Pemphigus Occurs Most Commonly in Indolent B Cell Lymphoma

desmoglein 3 were detected by enzyme-linked immunosorbent assay. From these results, the diagnosis of PNP was made. The patient was treated with an R-CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone). Since severe infection developed, we changed the treatment to rituximab monotherapy and then to R-CVP (rituximab, cyclophosphamide, vincristine and prednisolone). After treatment, the paraaortic mass had shrunk and the patient’s conjunctivitis, oral mucosal ulceration and truncal rash had improved. However, 5 months after the diagnosis of PNP, she developed dyspnea with an almost normal chest roentgenogram. A pulmonary function test revealed severe obstructive and restrictive airway disorder. It was proved that she had developed bronchiolitis obliterans, which is a common complication of PNP. Because there is no effective treatment for bronchiolitis obliterans at present [2] , she was transferred to another hospital for palliative therapy. According to a recent study that examined 163 patients with PNP, the percentages of underlying disease were as follows: chronic lymphocytic leukemia (CLL; 18.4%), nonHodgkin’s lymphoma (NHL; 38.6%), Castleman’s disease (18.4%) and benign thymoma (5.5%) [3] . However, to the best of our knowledge, there have been no reports showing the detailed pathology of NHL. Therefore, in order to clarParaneoplastic pemphigus (PNP) was first described by Anhalt et al. in 1990 [1] . It is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphous skin eruptions and underlying neoplasms, most commonly lymphoma. Firstly, we report a typical case of PNP with follicular lymphoma (FL). Then, we show the pathological characteristics of lymphoma associated with this disorder. A 65-year-old Japanese woman was referred to our hospital for evaluation of erosions on the conjunctiva and oral mucosa. She also had red papulae that had arisen on the back and extremities, and erythema palmare similar to erythema multiforme exudativum. Computed tomography revealed a para-aortic mass of around 8 cm in diameter. In the complete laboratory profiles of the serum, lactate dehydrogenase and soluble interleukin 2 receptor were elevated. Biopsy of the abdominal mass was performed and the diagnosis of FL was obtained. The clinical stage was IIIA. Biopsy specimens obtained from the inferior limb skin showed lichen planus-like features. Indirect immunofluorescence testing with a rat bladder section showed the presence of autoantibodies in the patient’s serum. Immunoblot analysis using normal human epidermal extracts detected IgG autoantibodies to the 190-kDa periplakin. Serum autoantibodies reacting with Received: September 10, 2013 Accepted: October 28, 2013 Published online: January 30, 2014