As we and others have found signalling and cell death defects in OTULIN‐deficient non‐haematopoietic cells (Figs 3 and 4; Heger et al, 2018), complete remission after HSCT strikingly shows that OTULIN deficiency in haematopoietic cells is necessary for clinical manifestation of ORAS including the organ‐specific symptoms panniculitis and diarrhoea (Fig 7D). Here, OTULIN is linked to autoinflammation, panniculitis, and dermatosis syndrome, autosomal recessive.