Multicentric Castleman Disease in a DOCK8 Deficient Patient with Orf
Castleman disease is a rare, heterogeneous disorder that driven by
proinflammatory responses. Human herpes virus-8 has a major role in
pathogenesis of multicentric Castleman disease. There is also a subgroup
of cases, human herpes virus-8 negative, idiopathic multicentric
Castleman disease. The role of primary immunodeficiencies in idiopathic
Castleman disease are poorly described. DOCK8 deficiency is a combined
primary immunodeficiency. It has a broad clinic spectrum including
atopy, autoimmunity and cancer. We present a 10-year-old, DOCK8
deficient patient. He had giant lobular capillary hemangiomas on his
neck, iliac and gluteal regions and multiple lymphadenopathies.
Abdominal lymph node pathology revealed hyaline vascular type Castleman
disease and human herpes virus-8 staining was negative. His lesions were
shown to be infected with orf virus. Our case is the first case to
relate idiopathic multicentric Castleman disease and DOCK8 deficiency;
also, very unusual presentation of orf virus infection in humans.