Bitemporal scalp, lip and tongue necrosis in giant cell arteritis: a rare presentation.

A 74-year-old man was presented with fever, bilateral throbbing temporoparietal headache, jaw claudication, and bilateral loss of vision. On examination, he had bilateral scalp necrosis with impending necrosis of lip and tongue. Temporal artery biopsy was done, and it was compatible with active temporal arteritis. This is one of the rare presentations of giant cell arteritis where there is simultaneous necrosis of scalp, lip, and tongue, and to the best of our knowledge, it is the first case reported from India.

What was known?

1. GCA is a rare systemic granulomatous vasculitis affecting people older than 50 years of age.

2. The scalp, lip, and tongue necrosis has been reported as rare and delayed phenomena and signify worse prognosis.

Introduction

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis affecting primarily large vessels branching out from the aorta. The most commonly involved vessels in GCA are the arteries of the scalp involving the temporal area. Hence, it is called “temporal arteritis.” It is also called Horton's disease after the physician who gave its first description in 1932. Clinically, it manifests as characteristic throbbing headache localized to temporal and parietal region of scalp. In addition, patient may have fever, malaise, jaw claudication, and polymyalgia rheumatica. Finally, as a rare occurrence, patient may have partial or complete blindness.[1] It occurs almost exclusively in patients older than 50 years of age with an incidence of 6.9-32.8 per one lakh population.[2]

Scalp necrosis as a cutaneous sign has been described as a rare presentation in GCA.[34] Others have noticed tongue and lip necrosis as well.[56] There is a direct correlation between a scalp necrosis and visual loss.[7] Scalp necrosis, tongue necrosis, lip necrosis, and visual loss are signs of very active GCA involving multiple vessels and indicate a delay in diagnosis.[8] Polymyalgia rheumatica may be associated with GCA in up to 50% of the cases.[8] Here, we are presenting a rare case in which there is simultaneous occurrence of bilateral scalp, lip, and tongue necrosis with bilateral visual loss without polymyalgia rheumatica. To the best of our knowledge, this is the first case report describing the simultaneous involvement of scalp, lip, and tongue necrosis in case of GCA in an Indian patient.

Case Report

A 74-year-old man was presented with high-grade fever, sudden onset bilateral temporoparietal headache, jaw claudication, and diminished vision in both eyes for last 7 days. On examination, he had bilateral scalp, lip, and tongue necrosis [Figures 1 and 2]. On clinical examination, the temporal arteries were palpable tender and nonpulsatile. In addition, he had exposure keratopathy and diminished corneal reflexes.

Figure 1

Temporal scalp necrosis

Figure 2

Lip and tongue necrosis

Routine hematological and biochemical investigations revealed raised erythrocyte sedimentation rate (ESR: 132 mm at the end of 1 h), anemia (hemoglobin: 8.6 g/dl), leukocytosis (total leukocyte count: 19,700/mm3), thrombocytosis (platelet count: 9, 94,000/mm3), and raised C-reactive protein level (10 mg/L). ANA was negative and the bone marrow examination was normal. The temporal artery biopsy was compatible with giant cell arteritis and showed infiltration of media tunica by lymphocytes and giant cells [Figure 3]. There was a focal destruction of internal elastic lamina and narrowing of the lumen due to fibrin deposition. Ultrasonography and color Doppler was not performed as the patient was bedridden, and we felt it was unnecessary at that given point of time.

Figure 3

Histopathology (H and E, ×4)

We have treated this patient with systemic corticosteroids (oral prednisolone: 1 mg/kg body weight per day). After an initial improvement in headache and scalp necrosis (within 24-36 h of initiation of therapy), there was no improvement for another 2-3 days, neither it was deteriorating. The patient's relatives decided to take him home against the medical advice. Therefore, further follow-up could not be possible.

Discussion

GCA or temporal arteritis is a systemic inflammatory vasculitis affecting medium- to large size vessels. It is more common in female and elderly (above 50 years).[1] Polymyalgia rheumatica may be associated in about 50% of the cases, which is now believed to be a different expression of the same disease.[28] Clinically, the most common symptom is bilateral temporoparietal headache. In addition, the patient may have fever, jaw claudication, loss of vision, and rarely scalp, lip, and tongue necrosis.[23]

Scalp necrosis is a delayed manifestation of GCA and associated with bilateral visual loss and higher mortality.[9] It will be further complicated or not going to respond to therapy if there is simultaneous involvement of lip and tongue. Therefore, scalp necrosis, lip necrosis, tongue necrosis, or loss of vision is sign of active and ongoing GCA involving multiple vessels.[8] Irreversible visual loss is due to arteritic ischemic optic neuropathy.[10] The formation of scalp necrosis may be due to occlusion of four arteries supplying the temporal area of the scalp and depends on the degree of anastomoses between these vessels.[1] With disease progression, more number of vessels are involved which may result in necrosis of the skin of that particular area. The overall mortality rate in GCA with scalp necrosis varies in different studies and ranges from 23.7% to as high as 38%.[19] Tongue infarction may be an early sign of scalp necrosis, but in our case, it was most delayed presentation.[5] Therefore, it is recommended that GCA may be considered a differential diagnosis in case of unusual and sudden headache in elderly.[11]

The diagnosis of GCA can be made by taking biopsy of the affected temporal artery, which shows inflammatory infiltrate in the adventitia and media of the arterial wall with fragmentation of the elastic lamina with or without giant cells. Taking temporal artery biopsy is safe in GCA, rarely a patient or their relative may refuse invasive procedure. Alternatively, color Doppler or duplex ultrasonography of temporal arteries may help in diagnosing and assessing the treatment outcome in GCA. It typically shows a dark hypoechoic circumferential wall thickening around the artery lumen, so-called halo sign, which disappear after 2-3 weeks of therapy.[1213] Once confirmed, treat promptly to avoid these complications especially irreversible visual impairment.[14] The treatment of choice in case of GCA is systemic corticosteroids (prednisolone, 1 mg/kg body weight).[1415] If patient is not responding or to taper the systemic steroids, one can add methotrexate as an adjuvant or maintenance therapy.[15] Recently, cytokines blockade with infliximab, etanercept, and tocilizumab has been tried with conflicting results.[15] Only tocilizumab, a humanized IL-6 receptor antibody, has produced a significant improvement and allowed rapid tapering of the systemic corticosteroids.[16]

To conclude, scalp necrosis, tongue necrosis, lip necrosis, or visual loss is delayed manifestation of GCA involving multiple vessels and could be avoided by early intervention.

What is new?

To the best of our knowledge, it is the first case in the world where there is progressive and simultaneous involvement of bilateral temporal scalp, lower lip and tongue necrosis in case of GCA.