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.