Hyper IgM syndrome with tuberculous osteomyelitis and scrofuloderma.

INDIAN PEDIATRICS 952 VOLUME 42__SEPTEMBER 17, 2005 Hyper IgM syndrome (HIGM) is a rare primary immunodeficiency characterized by low levels of serum IgA and IgG and a normal or increased levels of IgM due to a block in the B cell immunoglobulin switch from IgM to IgG(1). The estimated incidence is approximately 1 in 10,30,000 live births(2). Two types are described X linked hyper IgM (XHIM) and autosomal recessive hyper IgM (ARHIM). XHIM is more common and affects males. Respiratory tract infections, pneumocystis carinii pneumonia (PCP), chronic diarrhea, cryptosporidium infection, sclerosing cholangitis and chronic neutropenia are the common clinical manifestations. ARHIM is much rarer and affects both sexes and generally has a more benign course.