Model of the cellular effects of OTULIN deficiency in myeloid cells and fibroblasts in ORAS. Hyper-signalling and TNF secretion in myeloid cells as well as TNF-induced cell death of both haematopoietic cells and cell types with LUBAC downregulation, e.g. dermal fibroblasts, may contribute to the inflammation, pathogenesis, and clinical manifestation of ORAS. 