Temporal arteritis with scalp ulceration and blindness

Temporal arteritis or giant cell arteritis is a systemic granulomatous vasculitis of medium and large‐sized arteries, most frequently involving the temporal artery. It presents with headache, fever, elevated erythrocyte sedimentation rate and anemia. If untreated, complications like ischemic optic neuritis may even lead to blindness. Although very rare, scalp necrosis and ulcerations are the most common dermatologic findings. An 85‐year‐old woman presented with a two‐month history of bilateral scalp ulcerations, followed by permanent loss of vision in the left eye due to the delay in diagnosing temporal arteritis.     

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.     

巨细胞动脉炎1例

报告1例巨细胞动脉炎。患者男，80岁。因头面部皮下结节伴疼痛1个月余入院。皮肤科检查；前额，双侧额部有条索状皮下结节，有轻微触痛，双眼视物模糊。皮损组织病理检查符合巨细胞动脉炎改变，给予糖皮质激素治疗后病情好转。     

Scalp necrosis in temporal (giant cell) arteritis: implications for the dermatologic surgeon

Temporal arteritis, a variant of giant cell arteritis, is a systemic granulomatous vasculitis of large and medium-sized arteries. Usually the clinical Features arc dominated by ophthalmological and neurological complications. In rare instances, ischaemic necrosis, especially of the scalp, may lead patients to the dermatologist. We report a 76-year-old woman presenting with a unilateral scalp necrosis, accompanied by a dramatic ipsilateral impairment of vision. Immediately after duplex-sonography of the extracranial vessels and after initiation of corticosteroid therapy, the diagnosis of temporal arteritis was confirmed by temporal art en biopsy. One month later, because of insufficient secondary healing of the ulcer, the defect was covered by a mesh graft. The taking of the graft was delayed due to immunosuppressive therapy, but was complete. The patient unfortunately died as a result of complications related to surgical removal of an aspergilloma in the sphenoid cavity secondary to immunosuppressive therapy. We discuss the technique of artery biopsy and the possibility of surgical management of scalp necrosis in temporal arteritis.     

Bilateral scalp necrosis with giant cell arteritis

We present a patient with bilateral scalp necrosis caused by giant cell arteritis (temporal arteritis). A 67-year-old woman, who had been treated with 5 mg of oral prednisolone every other day for polymyalgia rheumatica, developed painful egg-sized regions of necrosis on both of her temples. Doppler pulsemetory revealed bilateral obstruction of the temporal arteries. Biopsy revealed ischemic necrosis of the skin and necrotic angiitis of the temporal arteries with giant cell infiltration. Bilateral stenosis of the internal carotid arteries and moderate retinal bleeding were revealed by angiography. Daily administration of prednisolone (20 mg/day) with intravenous and topical limaprost alphadex markedly improved her condition. The ulcers reepithelized without surgical treatment. There are few reports of bilateral scalp necrosis. Rapid and complete obstruction of the temporal artery may result in this condition. Simultaneous development of two ulcerative lesions in the ventro-parietal cranial regions is thought to correspond to systemic arterial involvement, including involvement of the internal carotid arteries.     

A case of temporal arteritis successfully treated with recombinant interleukin-2

An 80-year-old Japanese woman with temporal arteritis was treated with systemic recombinant human interleukin-2 (IL-2) (1 x 10(6) unit/day for six weeks). The presenting symptoms of headache and skin necrosis and abnormal laboratory findings, such as an elevated erythrocyte sedimentation rate and CRP, promptly improved without any serious side effects. Although the pathogenesis of temporal arteritis and the mechanism(s) of the beneficial effect of IL-2 on it still remain unknown, this preliminary study highly encourages further investigations.     

Macular lymphocytic arteritis: first clinical presentation with ulcers

Macular lymphocytic arteritis describes a recently reported entity, clinically characterized by asymptomatic hyperpigmented macules on the lower limbs, without association of systemic diseases. Histopathologically it is characterized by a lymphocytic arteritis with a hyalinized fibrin ring. We report a new case presenting with ulceration, a finding not previously described. A 25‐year‐old Hispanic woman was evaluated for a 1‐year history of a gradually progressive, asymptomatic eruption that begins at level of both knees and progressively affects both legs and feet. She also referred recently appeared ulcers on inner right ankle without previous traumatism. Physical examination revealed multiple fairly well‐defined light brown and faint pink patches with petechiae on as well as retiform crusts and livedoid lesions on inner right ankle. Both types of lesions were biopsied showing lymphocytic arteritis with fibrinoid necrosis and thrombus. There were no relevant laboratory alterations. The clinical peculiarity of our case is the clinical image of the lesions mimicking a pigmented purpuric dermatosis and the presence of a non‐traumatic ulcer which could be explained because chronic lymphocytic damage may cause ischemic damage. Ulceration in our case supports consideration of macular arteritis as a latent form of cutaneous polyarteritis nodosa.     

The development of Kaposi's sarcoma during immunosuppressive therapy for temporal arteritis

Background The appearance of Kaposi's sarcoma during iatrogenic immnunosuppression for renal transplantation is well known. A growing number of patients undergoing immnunosuppressive therapy for conditions other than organ transplanation have also developed this neoplasm, and temporal arteritis is emerging as one of the more common of those conditions. Case: We report a case of Kaposi's sarcoma complicating corticosteroid therapy for temporal arteritis, summarize the previously reported cases and consider the possible association between these two disorders. Conclusion: Although inconclusive from the available data, patients with temporal arteritis may be predisposed to iatrogenic Kaposi's sarcoma due to a shared antigen specific immune response.     

Giant cell (temporal) arteritis involving both external and internal carotid arteries.

A 76-year-old woman with giant cell (temporal) arteritis was described; she presented with a one year history of headache and tinnitus. Histopathological findings from a superficial temporal artery showed arteritis with granulomatous changes. Bilateral carotid arteriograms demonstrated the stenoses of both internal carotid arteries as well as the narrowing of the superficial temporal arteries. Although we dermatologists rarely encounter the disease in daily clinical practice, it is of clinical importance to perform cerebral angiography in patients suspected of temporal arteritis.     

Generalized granuloma annulare in a patient with temporal arteritis—are these conditions associated?

A case of generalized granuloma annulare associated with temporal arteritis is described. The patient, a 79-year-old man, noticed numerous asymptomatic lesions on his trunk and extremities for 3 months. Four months later, he suffered from headache and loss of vision. Both were successfully treated by oral administration of prednisolone.     

Significant relationship between temporal hair loss and other scalp areas in female pattern hair loss

Female pattern hair loss affects the central scalp, sparing the frontal hairline. The temporal area can also be affected by hair loss. We investigated the degree of temporal hair loss and correlation of other sites of scalp hair loss in Korean female pattern hair loss patients. A total of 109 women with female pattern hair loss were enrolled in this retrospective analysis. We measured hair density and thickness in five scalp sites including the frontal, vertex, occipital and bilateral temporal areas by phototrichogram. Frontal and vertex area hair loss were classified according to the Basic and Specific (BASP) classification, and temporal scalp and occiput areas were also assessed. Eighty-nine patients showed temporal hair loss. The mean of the hair density was lowest in the temporal area among all scalp areas. Total and thick hair densities of the frontal scalp were correlated with those of the vertex, temporal scalp and occiput in descending order, and hair thickness of the frontal scalp was more related with that of the temporal scalp than the vertex. In this study, temporal involvement is evident in female pattern hair loss. We suggest that temporal involvement should be added to pattern hair loss classification, especially BASP classification.     

Reticulated lesion as an early diagnostic sign for giant cell arteritis

We report a case of giant cell arteritis (GCA) in an elderly woman. The patient presented to her dermatologist with a reticulated, purpuric patch on her frontal-temporal scalp and forehead that was associated with unilateral head pain. A punch biopsy of the lesion was suspicious for GCA, which was later confirmed by temporal artery biopsy. This case highlights a potential early cutaneous finding of GCA that may aid the clinician in the diagnosis before more severe complications occur.     

伴结节性红斑的大动脉炎2例临床及组织病理分析并文献复习

报告2例伴结节性红斑的大动脉炎。例1.患者女,43岁。例2.患者女,21岁。二例患者均因双下肢反复出现结节性红斑就诊,其中例2有间断发热。皮肤科检查：二例患者双下肢均可见散在结节性红斑。二例患者皮损组织病理检查均提示血管周围少许单核细胞或淋巴细胞浸润,皮下脂肪间隔增宽,胶原增生,部分脂肪细胞变性坏死,呈混合性脂膜炎改变。计算机断层摄影血管造影术（CTA）提示2例患者血管管腔均有不同程度的狭窄,例1患者狭窄部位位于头臂干、双侧颈动脉及锁骨下动脉,例2患者位于左锁骨下动脉。诊断：大动脉炎。     

Giant-cell (Temporal) Arteritis Following a Bypass Operation for Cerebral Infarction

A 61-year-old man was referred to us with linear and indurated erythemas and severe tenderness on the scalp, neck, tongue, and scrotum which had gradually spread over the previous six months. He had a history of an intracranial bypass operation for cerebral infarction. Histologic and ultrastructural examinations revealed giant-cell arteries in the skin as well as inflammatory cells infiltrating into nerves. Carotid angiograms indicated stenosis of cranial arteries. It is suggested that the bypass operation may be related to the occurrence of the giant-cell arteritis and that the inflammatory cell infiltrate into nerves may have caused the severe tenderness of this disease.     

Lymphocytic thrombophilic arteritis complicated by systemic involvement

Lymphocytic thrombophilic arteritis (LTA) is a recently described entity defined by primary lymphocytic vasculitis; it typically has a chronic indolent course. We describe a patient who presented with clinical and histological findings consistent with LTA and later developed bilateral focal testicular infarcts as well as an acute median nerve neuropathy.     

Hutchinson-Horton ulcero-necrotizing cranial arteritis

Arteritis cranialis ulcero-necroticans Hutchinson-Horton is the necrotizing variant of giant-cell temporal arteritis, which was first described by Hutchinson in 1890 and Horton in 1932. Although the histomorphological findings (occluding proliferation of the intima, destruction of the elastica interna, giant-cell infiltration) suggest reactive pathomechanisms, the aetiology of the disease remains rather uncertain. The hypothesis currently favoured is that auto-immunological processes originate from vascular alterations and subsequently lead to the recognition of antigenic determinants by the host immune system being followed by immunological response mechanisms clinically manifest as vasculitis. Arteritis cranialis is most frequently found in the temporal artery; however, the reasons for this preferential location are not clearly established, and giant-cell arteritis can also be found elsewhere. Ulceration of the skin can be encountered and may affect large areas of the scalp, depending on the vascular perfusion pattern involved. The case report described in this paper comprises anamnestic data, clinical and serological findings, and the histological picture typical for giant-cell arteritis. Stress is laid on the importance of early diagnosis to prevent irreversible complications with possibly fatal outcome.     

Granulomatous vasculitides and the skin

Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss granulomatosis may all have cutaneous involvement. The morphology of cutaneous lesions in these disorders varies from macular erythema to frank gangrenous ulceration. Most often lesions are located on the extremities; however, truncal or facial involvement has been reported, the latter especially in Wegener's granulomatosis. A common histologic finding in these cutaneous lesions is necrotizing vasculitis. However, it is also possible to see Churg-Strauss extravascular granulomas and even periarteritis. Cutaneous involvement with these three forms of systemic granulomatosis generally parallels the systemic course. The treatment for the cutaneous lesions is dictated by the treatment for the systemic vasculitis. It is important to recognize that the cutaneous extravascular granuloma and cutaneous granulomatous vasculitis can be associated with other disorders in addition to systemic granulomatosis. These disorders include most importantly lymphoproliferative diseases, inflammatory disorders such as arthritis, autoimmune diseases, and other inflammatory disorders such as sarcoidosis. Cutaneous involvement with giant cell or temporal arteritis is not common, but ulcerative temporal-parietal scalp lesions are distinctive. Although not common in the United States, Takayasu's arteritis may have several cutaneous manifestations, including erythema nodosum-like lesions. Granulomatous vasculitides have a myriad of cutaneous manifestations. Knowledge of these manifestations may allow for prompt diagnosis in many cases and increased surveillance in other cases for associated systemic illnesses.     

Survey of Japanese dermatological vasculitis specialists on cases of cutaneous arteritis (cutaneous polyarteritis nodosa)

We developed a questionnaire to examine the findings of cutaneous arteritis among dermatological specialists experienced in vasculitis as certified by the Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. We sent a questionnaire to 12 dermatological facilities identified through the revised Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. Retrospective data obtained from 84 patients at the 12 dermatological facilities between 2012 January 2016 December were evaluated. The 84 patients were categorized into two groups, a systemic steroid treatment group (group 1, n = 52) and a no systemic steroid treatment group (group 2, n = 32). C-reactive protein in group 1 patients was significantly higher than that in group 2 patients. Frequency of fever, arthritis, myalgia- and peripheral neuropathy in group 1 was significantly higher than that in group 2. We propose that these symptoms could serve as early markers for the transfer from cutaneous arteritis to systemic polyarteritis nodosa. We further suggest that patients who are subsequently associated with cerebral hemorrhage and infarction, who are originally diagnosed as having cutaneous arteritis, could progress to systemic polyarteritis nodosa. The study demonstrated that it is important for dermatologists to detect these findings early in order to establish an accurate diagnosis and a timely treatment.     

Three cases of polyarteritis nodosa cutanea and a review of the literature

We describe three cases of polyarteritis nodosa cutanea (PNC) showing necrotizing arteritis and only cutaneous lesions without systemic symptoms or visceral involvement for eleven, six, and three years after the onset of the disease. Since it was first described, there has been continuous controversy as to whether PNC progresses to systemic PN. Some cases have been described which had begun with a cutaneous lesion and progressed to the systemic form 19 and 18 years after the onset of the disease, so we believe that long term follow-up of this disease is essential.     

Scalp necrosis in temporal arteritis

Giant cell arteritis is a systemic disease of the elderly which affects large and medium-sized arteries and which may occur in association with polymyalgia rheumatica. Scalp necrosis is a rare cutaneous complication of giant cell arteritis, and this dermatological presentation is not commonly reported.1-4 Giant cell arteritis should be considered in all elderly patients with scalp ulceration. We describe two patients who presented in this manner.