The genetic background of PASH syndrome is very heterogeneous; the absence of a PSTPIP1 gene mutation was first reported, however, researchers recently found a PSTPIP1 gene mutation in a PASH patient (Calderon-Castrat et al., 2016). This evidence concerns the gene PSTPIP1 and Pyoderma gangrenosum-acne-suppurative hidradenitis syndrome.