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January 2013

Paraneoplastic Pemphigus With Anti–Laminin-332 Autoantibodies in a Patient With Follicular Dendritic Cell Sarcoma

Author Affiliations

Author Affiliations: Departments of Dermatology, Nagoya University Graduate School of Medicine, Nagoya, Japan (Drs Sugiura, Matsumoto, Matsubara, Hagiwara, Muro, and Akiyama), Kurume University School of Medicine and Kurume University Institute of Cutaneous Cell Biology, Kurume, Fukuoka, Japan (Drs Koga and Hashimoto), and Gifu Prefectural Tajimi Hospital, Tajimi, Gifu, Japan (Dr Ishikawa).

JAMA Dermatol. 2013;149(1):111-113. doi:10.1001/2013.jamadermatol.512

To our knowledge, only 4 cases of paraneoplastic pemphigus (PNP) associated with follicular dendritic cell sarcoma (FDCS) have been reported.1-4 We describe herein a patient with PNP associated with FDCS with anti–laminin-332 autoantibodies.

A 28-year-old Japanese man presented with a 6-month history of painful erosions on the oral mucosa as well as polymorphic cutaneous lesions including erythemas and erosions (Figure 1A). Skin biopsy specimens taken from an erythematous area on the trunk revealed scattered necrotic keratinocytes in the epidermis, vacuolization in the basal layer, and a bandlike lymphocytic infiltration throughout the papillary dermis (Figure 1C). Under the diagnosis of autoimmune blistering disease, the patient had been treated at another institution with immunosuppressive therapies including steroid pulse therapy for 5 months, with no remarkable improvement.