The first syndrome associated with CARD9 deficiency affects the skin and subcutaneous tissues, and patients in this category present with dramatic persistent fungal infections of the skin, nails and/or scalp (Lanternier et al., 2013; Wang et al., 2014), with occasional contiguous dissemination to the subcutaneous layers, lymph nodes and bone (Lanternier et al., 2013). Here, CARD9 is linked to fungal infectious disease.