Castleman's disease associated with pemphigus vulgaris

Castleman's disease is a rare, benign, lymphoproliferative disorder of unknown cause. The hyaline-vascular type is frequently associated with a localized mediastinal mass. The plasma-cell type is associated with constitutional symptoms, multicentric lymph node involvement, lymphoma development, and autoimmune disease-like laboratory abnormalities such as elevated erythrocyte sedimentation rate, anemia, and thrombocytopenia. We report a case of hyaline-vascular Castleman's disease associated with a cutaneous autoimmune disease, pemphigus vulgaris. We also reviewed the clinicopathologic features of four similar cases. Among these five reports of Castleman's disease, five patients had severe erosive stomatitis diagnosed as oral pemphigus, three had keratoconjunctivitis, and three had circulating pemphigus antibodies. All were young, ranging in age from 15 to 21 years, and four of the five were women. Two had hyaline-vascular Castleman's disease, whereas three had plasma-cell Castleman's disease. All five had surgical resection of the Castleman's disease mass. After surgery, remission of pemphigus vulgaris could be achieved with reduced dosages of steroids in all cases. In at least two cases steroid treatment could be completely discontinued. We postulate that an underlying immune dysfunction in Castleman's disease facilitates the expression of pemphigus.