Giant Cell Arteritis with Scalp and Tongue Necrosis: A Rare Case of Extensive Mucocutaneous Ischemia

Background Giant Cell Arteritis (GCA) is a primary large-vessel vasculitis that typically affects individuals over the age of 50.[1] It is characterized by granulomatous inflammation involving all 3 layers of the vessel wall, primarily affecting the major branches of the aorta, including the extracranial branches of the carotid arteries.[1-3] Typical disease presentations involve polymyalgia rheumatica (PMR), weight loss, jaw claudication, headaches, temporal tenderness, and vision loss (partial or complete).[3]

Case Report We reviewed the clinical course, diagnostic workup, and management of a 76-year-old Caucasian man with a 60-pack-year smoking history and comorbid hypertension, who developed severe scalp and tongue necrosis from GCA. Clinical data were mainly obtained from dental and rheumatology office visits in addition to pathology reports. Pertinent investigations included CT brain and angiography, laboratory testing (ESR, CRP), oral biopsy, and temporal artery biopsy. Relevant imaging and histopathology findings were incorporated. The patient provided informed consent for publication. The patient experienced several weeks of evolving symptoms, including jaw claudication, bilateral headaches, transient diplopia, and eventually developed scalp tenderness with necrosis, and a painful tongue ulcer (Figure 1A,B). Initial dental and neurologic evaluations were unrevealing, contributing to a delayed diagnosis. Laboratory studies showed mildly elevated inflammatory markers (ESR 24 mm/hr, CRP 29 mg/L). Temporal artery biopsy confirmed severe active arteritis with multinucleated giant cells and partial luminal occlusion. High-dose prednisone (50 mg daily) was initiated, followed by tocilizumab 162 mg subcutaneously weekly as a steroid-sparing agent. The patient demonstrated rapid clinical improvement, with near-complete resolution of scalp and tongue necrosis within 2 months (Figure 1C,D) and no recurrence of visual symptoms during follow-up.

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Conclusion This case highlights the importance of promptly recognizing atypical GCA presentations, such as scalp and tongue necrosis, which are linked to delayed diagnosis and increased morbidity. Early initiation of corticosteroids with adjunctive tocilizumab led to rapid recovery and prevention of complications in our patient. Notably, his 60-pack-year smoking history raises the possibility that heavy, long-term smoking may predispose to more severe or necrotizing forms of GCA. Future studies are needed to clarify the role of smoking as a risk factor and potential disease modifier in GCA, which may help guide earlier diagnosis and tailored treatment strategies.

References [1.] Samec MJ. J Rheumatol 2023;50:1310-7. [2.] Stamatis P. Front Med (Lausanne) 2024;11:1453462. [3.] Chehem Daoud Chehem F. Semin Arthritis Rheum 2024;64:152348.