<b>Results</b>: PTX3 levels in both BALF and plasma were significantly higher in IPA patients than in non-IPA controls (BALF: 8.98 [3.47-22.53] ng/ml vs. 1.12 [0.46-3.35] ng/ml, <i>P</i> < 0.0001; plasma: 8.29 [4.68-21.41] ng/ml vs. 2.60 [1.35-5.02] ng/ml, <i>P</i> < 0.0001). Here, PTX3 is linked to Ito hypomelanosis.