Multicentric Castleman Disease in a DOCK8 Deficient Patient with Orf Virus Infection

Abstract

Castleman disease is a rare, heterogeneous disorder that driven by\nproinflammatory responses. Human herpes virus-8 has a major role in\npathogenesis of multicentric Castleman disease. There is also a subgroup\nof cases, human herpes virus-8 negative, idiopathic multicentric\nCastleman disease. The role of primary immunodeficiencies in idiopathic\nCastleman disease are poorly described. DOCK8 deficiency is a combined\nprimary immunodeficiency. It has a broad clinic spectrum including\natopy, autoimmunity and cancer. We present a 10-year-old, DOCK8\ndeficient patient. He had giant lobular capillary hemangiomas on his\nneck, iliac and gluteal regions and multiple lymphadenopathies.\nAbdominal lymph node pathology revealed hyaline vascular type Castleman\ndisease and human herpes virus-8 staining was negative. His lesions were\nshown to be infected with orf virus. Our case is the first case to\nrelate idiopathic multicentric Castleman disease and DOCK8 deficiency;\nalso, very unusual presentation of orf virus infection in humans.