Background: Lingual necrosis is a rare complication of giant cell arteritis (GCA). Methods: A 77-year-old woman presented for treatment of a painful and discolored tongue, odynophagia, and dehydration refractory to antimicrobials over 2 weeks. An extensive, well-demarcated necrotic area was visualized on the anterior tongue upon admission. Leukocytosis, thrombocytosis, and elevated erythrocyte sedimentation rate were present. Computed tomography angiogram of the head and neck revealed an undulated-beaded appearance of the distal internal carotid arteries and vertebral arteries bilaterally. Results: High-dose intravenous steroids were initiated for suspected vasculitis. Temporal artery biopsy confirmed the diagnosis of GCA. The patient’s condition improved and the anterior tongue was well healed at 1 month follow-up. Conclusions: An atypical presentation of GCA (eg, isolated lingual necrosis) risks a delay in diagnosis and increased morbidity. Any patient above the age of 50 years presenting with tongue necrosis, in the absence of known cause, should undergo expedited workup for GCA.

Kawasaki disease (KD) is a pediatric systemic vasculitis of unknown etiology wherein genetic influence is suspected. Gene clusters within the HLA region at chromosome 6p21.3 have been linked to KD and other autoimmune disorders. As collagen is a strong autoantigen inducing chronic inflammation in patients with vasculitis, this study tests a hypothesis that single-nucleotide polymorphism (SNP) of a collagen gene, COL11A2, located in this HLA region may affect susceptibility to Kawasaki disease and its arterial sequels. SNP sites rs2294478 (at promoter) and rs2076311 (at intron 19) were genome-typed on 93 KD patients and 680 healthy subjects. Genotypic and allelic frequencies analyses found A allele at rs2076311 as a risk allele for KD. Clinical association study showed protective potential of C/C genotype at rs2294478 and A/A at rs2076311 for developing coronary artery lesions (CALs) in patients. In addition, C-A haplotype of COL11A2 gene associates with KD development and can serve as a genetic marker to differentiate KD patients lacking CALs from those with such lesions. Our findings suggest the involvement of genetic variations of COL11A2 in Kawasaki disease and CAL formation.