Spinal dermoid cysts
Spinal dermoid cysts are benign ectopic growths thought to be a consequence of embryology errors during neural tube closure. Their reported incidence is extremely rare, accounting for less than 1% of intramedullary spinal cord tumours. It has been proposed that a possible 180 cases of spinal dermoid tumours have been identified over the past century in the literature.Dermoid cysts more often involve the lumbosacral region than the thoracic vertebrae and are extramedullary presenting in the first decade of life. Various hypotheses have been advanced to explain the pathogenesis of spinal dermoids, the origin of which may be acquired or congenital. Acquired or iatrogenic dermoids may arise from the implantation of epidermal tissue into the subdural space i.e. spinal cutaneous inclusion, during needle puncture (e.g. lumbar puncture) or during surgical procedures on closure of a dysraphic malformation. Congenital dermoids, however, are thought to arise from cells whose position is correct but which fail to differentiate into the correct cell-type. The long-time held belief was that the inclusion of cutaneous ectodermal cells occurred early in embryonic life, and the displaced pluripotent cells developed into a dermoid lesion.Spinal abnormalities, e.g. intramedullary dermoid cysts may arise more frequently in the lumbosacral region (quite often at the level of the conus medullaris) and may be seen with other congenital anomalies of the spine including posterior spina bifida occulta as identified by the neuroradiological analysis. Diagnosis
Differential diagnosis
A small dermoid cyst on the  coccyx can be difficult to distinguish from a pilonidal cyst.
The mutation in the TNFSF5 gene causes there to be no recognition of CD40 by CD40 ligand, and thus the T cells do not induce Ig class switching in B cells, so there are markedly reduced levels of IgG, IgA, and IgE, but have normal or elevated levels of IgM. CD40 ligand is also required in the functional maturation of T lymphocytes and macrophages, so patients with this disorder have a variable defect in T-lymphocyte and macrophage effector function, as well as hyper IgM. Type 2
Immunodeficiency with hyper IgM type 2 is caused by a mutation in the Activation-Induced Cytidine Deaminase (AICDA) gene, which is located on the short arm of chromosome 12. The protein that is encoded by this gene is called Activation-Induced Cytidine Deaminase (AICDA) and functions as a DNA-editing deaminase that induces somatic hypermutation, class switch recombination, and immunoglobulin gene conversion in B cells. When a person is homozygous for the mutation in the AICDA gene, the protein fails to function, and thus somatic hypermutation, class switch recombination, and immunoglobulin gene conversion cannot occur, which creates an excess of IgM. Type 3
Immunodeficiency with hyper IgM type 3 is caused by a mutation in the gene that codes for CD40. As mentioned above, CD40 is expressed on the surface of B cells, and its binding to CD40 ligand on activated T cells induces Ig class switching.