Bilateral Scalp Necrosis in Giant Cell Arteritis

Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Peter Quiros, MD Bilateral Scalp Necrosis in Giant Cell Arteritis Anisha N. Somani, BS, Bayan Al Othman, MD, Tonse Kini, MD, Andrew G. Lee, MD Downloaded from http://journals.lww.com/jneuro-ophthalmology by BhDMf5ePHKav1zEoum1tQfN4a+kJLhEZgbsIHo4XMi0hCywCX1AWnYQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC1y0abggQZXdtwnfKZBYtws= on 05/04/2022 FIG. 1. Tender, excoriated, necrotic-appearing skin lesions over the left temporal artery distribution without rash or vesicles. Abstract: Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most McGovern Medical School (ANS), Houston, Texas; Department of Ophthalmology (BAO, TK, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; Department of Ophthalmology (AGL), University of Texas Medical Branch, Galveston, Texas; Department of Ophthalmology (AGL), University of Texas MD Anderson Cancer Center, Houston, Texas; Department of Ophthalmology (AGL), Texas A and M College of Medicine, Bryan, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Address correspondence to Andrew G. Lee, MD, Blanton Eye Institute, Houston Methodist Hospital, 6560 Fannin Street, Suite 450, Houston, TX 77030; E-mail: [e-mail] Somani et al: J Neuro-Ophthalmol 2021; 41: e125-e127 feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature. Journal of Neuro-Ophthalmology 2021;41:e125–127 doi: 10.1097/WNO.0000000000000862 © 2019 by North American Neuro-Ophthalmology Society A n 85-year-old Caucasian woman presented with a 3month history of episodic headache, weight loss, and jaw claudication. One month before presentation, she devele125 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay oped acute vision loss in the left eye. Three weeks later, she developed a new-onset scalp pain and tenderness and a rightsided scalp lesion, which was erythematous and in the ophthalmic division of the trigeminal nerve. A presumptive diagnosis of herpes zoster ophthalmicus (HZO) was made, and the patient was started on valacyclovir. Her ocular history was significant for the dry form of age-related macular degeneration of both eyes and primary open-angle glaucoma. Her medical history was significant for coronary artery disease with stenting, atrial fibrillation, sick sinus syndrome, hypertension, hyperlipidemia, transient ischemic attack, and hypothyroidism, which were all controlled medically. The remaining past surgical, family, and social histories were noncontributory. One week before presentation, the vision progressed to no light perception in her left eye (left eye). Physical examination revealed a tender, excoriated, necrotic-appearing area over the left temporal artery distribution without rash or vesicles (Fig. 1), several areas of broken-down skin in the right temporal scalp, and a newonset area of tender, linear, erythematous lesions in the right occipital region (Fig. 2). Best-corrected visual acuity was 20/50 in the right eye (right eye) and no light perception in the left eye. Pupillary examination showed an amaurotic pupil and a relative afferent pupillary defect in the left eye. Intraocular pressure measurements, extraocular movements, and anterior segment examination were normal in both eyes. Dilated fundus examination showed pallid edema in the left eye and bilateral macular drusen consistent with age-related macular degeneration in both eyes. The erythrocyte sedimentation rate (ESR) was elevated at 101 mm/hr (normal range 0–20 mm/hour), and C-reactive protein (CRP) was 2.92 mg/dL (normal range 0–0.50 mg/ dL). MRI of the brain and orbit was unremarkable. Biopsy of the left temporal artery showed focal and segmental absence of the elastic lamina, fibromuscular hyperplasia of the intima, multinucleated giant cells, lymphocytic infiltrate, and few vessels in the muscularis and intima consistent with recanalization and consistent with the diagnosis of active giant cell arteritis (GCA). Additional biopsies of the left frontal scalp skin and right occipital skin lesions revealed wedge-shaped necrosis of the epidermis, arteritis of a small-caliber occipital artery with occlusion of the lumen, focal reactive re-epithelialization, infarction of adnexal structures, and fat necrosis of subcutaneous tissue with lipid-laden macrophages. No viral changes were seen on skin pathology. Molecular testing was negative for herpes simplex virus. Within one day of initiation of the high-dose steroids, her headache and jaw pain had improved, and she was tolerating oral corticosteroid intake. She was referred to rheumatology to consider initiation of tocilizumab, a humanized monoclonal antibody against the interleukin-6 receptor (IL-6R). e126 FIG. 2. Tender, linear, erythematous lesions in the right occipital region (after hair shaving). Images courtesy Imtiaz Chaudhry, MD, Houston Eye Associates. GCA is commonly associated with new-onset headache, scalp tenderness, and jaw claudication in an elderly patient (1-5). Although scalp necrosis in GCA is well known, our case is unique, in that it initially mimicked HZO, and later, the skin biopsies on the contralateral occipital region were also positive for GCA. The scalp is known to have a rich anastomotic arterial blood supply; therefore, necrosis in GCA is believed to result from occlusion of branches of the external carotid arteries supplying the scalp region (1,6). There is a positive correlation in GCA between the amount of arterial elastic tissue and extent of vessel involvement, with the most severely involved arteries in GCA being the superficial temporal, vertebral, ophthalmic, and posterior ciliary arteries (7). To the best of our knowledge, there have been only 5 previous reports in the English literature of scalp necrosis involving the occipital region in patients with GCA, suggesting an uncommon, severe involvement of the occipital artery in our patient. About one-third of patients with biopsy-proven GCA have small-vessel involvement (8), the presence of which has been correlated with more severe clinical symptoms (9). To the best of our knowledge, this is an interesting and unique case of GCA mimicking HZO and confirmed as arteritic scalp necrosis by biopsies of the involved temporal artery, the scalp, and the contralateral occipital skin including small-vessel arteritis. Somani et al: J Neuro-Ophthalmol 2021; 41: e125-e127 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay Interestingly, patients with GCA and scalp necrosis have a higher frequency of vision loss (31.5%) or other ophthalmic findings (38.4%) compared with patients without scalp necrosis (maximum of 20%) (1). GCA with scalp necrosis is also associated with an increased mortality compared with GCA without scalp necrosis. The fatal outcomes in these cases stem mostly from arteritic cardiovascular events and secondary infections (1). The scalp lesions may not be recognized initially as GCA and can mimic HZO. Patients with scalp necrosis experience delay in diagnosis on average of 1 month later than GCA patients without scalp necrosis, suggesting that the presence of scalp lesions may complicate the diagnosis (1). The differential diagnosis of scalp necrosis in older patients includes arteritic (i.e., GCA) and nonarteritic etiologies (e.g., herpes zoster, irritant contact dermatitis, postirradiation ulcers, microbial infections, ulcerated skin tumors, and pyoderma gangrenosum) (1,2). The scalp lesions are commonly initially misdiagnosed as “shingles” because of herpes-zoster virus (HZV) (3), especially when they are in the distribution of the ophthalmic division of the trigeminal nerve (as with our patient). However, it is important to note that HZV lesions are more painful, obey a dermatomal distribution, and are rarely bilateral, whereas scalp necrosis due to GCA often crosses the midline and is bilateral (3). Unfortunately, patients with HZO-like GCA can have ipsilateral scalp pain and tenderness and an elevated serum ESR and CRP. Clinicians should be aware that scalp lesions can occur as an early or late sign of GCA. Somani et al: J Neuro-Ophthalmol 2021; 41: e125-e127 STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: B. Al Othman; b. Acquisition of data: A. Lee; c. Analysis and interpretation of data: A. Somani. Category 2: a. Drafting the manuscript: B. Al Othman; b. Revising it for intellectual content: A. Kini. Category 3: a. Final approval of the completed manuscript: A. Lee. REFERENCES 1. Tsianakas A, Ehrchen JM, Presser D, Fischer T, KruseLoesler B, Luger TA, Sunderkoetter C. Scalp necrosis in giant cell arteritis: case report and review of the relevance of this cutaneous sign of large-vessel vasculitis. J Am Acad Dermatol. 2009;61:701–706. 2. Akram Q, Knight S, Saravanan R. Clin Rheumatol. 2015;34:185. Available at: https://doi.org/10.1007/s10067014-2792-y. Accessed April 15, 2019. 3. Dudenhoefer EJ, Cornblath WT, Schatz MP. Scalp necrosis with giant cell arteritis. Ophthalmology. 1998;105:1875–1878. 4. Rudd JC, Fineman MS, Sergott RC. 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