ACP5 and pulmonary arterial hypertension: This is an autosomal recessive inherited disorder and the genetic variants of this patient were p.S267Lfs*20 and p.G239D of ACP5. For the PRAAS/CANDLE patient, the most prominent characteristic was lypodystrophy, other clinical features included fever, rash, PAH, arthritis, intracranial calcification, uveitis, loss of hearing, and growth retardation.