Castleman病に伴う腫瘍随伴性天疱瘡の1例

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Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lymphoproliferative neoplasms. We report a case of PNP associated with Castlemans disease. A 39-year-old man showed white lesions on both edges of the tongue. The lesions were diagnosed as lichen planus, compatible on the basis of clinical features and histopathological examination. The patient was treated with steroid ointment and azulene gargle for 1 year. However, after mild common cold symptoms, he presented with extensive ulceration and erosions throughout the entire oral mucosa, conjunctivitis, and vesicles on the sole of the foot. Serological examination showed elevated immunoglobulin (Ig) G levels, extremely high titers of anti-nuclear antigen, and positive results for anti-dsDNA and anti-ssDNA. Positive results were also obtained for anti-desmoglein 3, anti-envoplakin,and anti-periplakin. Furthermore, direct immunofluorescence testing showed IgG and C3 deposition in the epidermal intercellular spaces. The past history included lymphoproliferative lesions in the mediastinal space. Castlemans disease was therefore diagnosed. The mucocutaneous lesions were thus considered to be PNP associated with Castlemans disease. Treatment was initiated with predonisolone, and the oral lesions, conjunctivitis, and skin lesions all improved markedly. As of the time of this writing, mediastinal lymphoproliferative lesions remain, and oral lesions have not completely disappeared. We are carefully observing the oral lesions while contiuning treatment with predonisolone.
Japanese Society of Oral and Maxillofacial Surgeonsの論文

Castleman病に伴う腫瘍随伴性天疱瘡の1例

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