X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency caused by mutations in the CD40Ligand (CD40L) gene, leading to defective immunoglobulin class-switch recombination and impaired T-cell activation. Well-known manifestations of the syndrome include recurrent sinopulmonary infections, gastrointestinal complications, and neuroendocrine tumors. Cutaneous warts have rarely been reported in XHIGM.
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