Multivariate Cox regression analysis confirmed PTX3 levels in plasma (hazard ratio [HR] 3.87, 95%CI [1.87–8.00], P<0.001) and BALF (HR 2.40 [1.19–4.84], P = 0.014) were independent prognostic factors for IPA mortality. The gene discussed is PTX3; the disease is Ito hypomelanosis.