Blau syndrome, or early-onset sarcoidosis, is a juvenile-onset systemic granulomatosis associated with a mutation in nucleotide-binding oligomerization domain 2 (NOD2).
CARD15 mutations in familial granulomatosis syndromes: a study of the original Blau syndrome kindred and other families with large-vessel arteritis and cranial neuropathy.
A number of case reports have emerged concerning sarcoid-like granulomatosis secondary to TNF-α therapy, an adverse effect that typically affects the pulmonary and cutaneous systems.
The image highlights the necessity of including granulomatosis as a differential from malignancy in interpretation of FDG PET/CT showing diffuse mucosal/submucosal oropharyngeal and/or laryngeal lesions but without established diagnosis.