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Mariko Seishima, MD; Makiko Oda, MD; Zuiei Oyama, MD; Tomoaki Yoshimura, PhD; Futoshi Yamazaki, PhD; Takahiko Aoki, MD; Mariko Nei, MD; Takashi Hashimoto, MD

Arch Dermatol. 2004;140:1500-1503.

Background  Most paraneoplastic pemphigus (PNP) cases reported to date have been associated with lymphoproliferative neoplasms. Patients with PNP have autoantibodies against the plakin family (eg, envoplakin and periplakin). Antibodies against desmoglein 3 (Dsg3) and Dsg1, antigens for classic types of pemphigus, have also been reported to play an important role in the initial stage of PNP.

Observations  We describe a patient with PNP associated with follicular dendritic cell sarcoma. Antibodies to envoplakin and periplakin were detected. When only mucosal lesions were observed at the early stage, the antibody to Dsg3 but not to Dsg1 was detected by enzyme-linked immunosorbent assay. After skin lesions appeared, antibodies to Dsg1 and Dsg3 were detected. These titers were elevated, with exacerbation of skin lesions. Although the patient received corticosteroid therapy, double-filtration plasmapheresis, and intravenous human immunoglobulin therapy after surgical resection of follicular dendritic cell sarcoma, she died of fungal infective lung embolisms. A direct immunofluorescence study of autopsy samples showed IgG deposition in the epidermis of the skin and oral mucosal membrane, but not in the lungs and kidneys and follicular dendritic cell sarcoma of the para-aortic area.

Conclusion  In this patient with PNP and follicular dendritic cell sarcoma, there was an association between the clinical phenotype and the anti-Dsg antibody profile, as seen in pemphigus vulgaris.

Author Affiliations: Departments of Dermatology (Drs Seishima, Oda, and Oyama), Pharmacy (Drs Yoshimura and Yamazaki), and Internal Medicine (Dr Aoki), Ogaki Municipal Hospital, Ogaki, Japan; and Department of Dermatology, Kurume University School of Medicine, Kurume, Japan (Drs Nei and Hashimoto).

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