Journal of Dermatology 2015; 42: 315–317
doi: 10.1111/1346-8138.12752
CONCISE COMMUNICATION
Case of Behc ß et’s disease complicated by oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm Toshifumi YAMAOKA, Hiroyuki MUROTA, Ichiro KATAYAMA Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan
ABSTRACT Behc ß et’s disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology involving systemic vasculitis. Vasculitis in BD results from the involvement of arteries, veins and blood vessels of all sizes, which leads to the three major manifestations of this condition: venous occlusion, arterial occlusion and aneurysm formation. Therefore, whole-body vascular involvement should always be considered in BD patients. Here, we describe the first appearance of an internal carotid-posterior communicating artery aneurysm, resulting in complete oculomotor nerve palsy in a BD patient. A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. His condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Ten years after his initial clinical presentation, he had manifestations of right-sided ptosis and cyclic dull pain in his right temporal region. Magnetic resonance imaging and angiography revealed a right internal carotid artery-posterior communicating artery aneurysm. Although oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm in a BD patient has not been reported previously, our report highlights the fact that this abnormal manifestation should be considered in those with vasculo-BD.
Key words: Behcßet’s disease, internal carotid artery-posterior communicating artery aneurysm, oculomotor nerve palsy, various neutrophilic dermatosis, vasculo-Behcßet’s disease.
INTRODUCTION Behc ß et’s disease (BD) is an inflammatory disorder of unknown etiology, for which an immunological pathogenesis has been proposed. It is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis and various neutrophilic dermatoses. Vasculitis is thought to be a pivotal factor underlying most clinical manifestations of BD. This disease is common in the Middle East and Japan but rare in Northern Europe and the USA, and although it typically affects young adults, it can also occur in children.1 BD is divided into three subtypes, comprising neuro-, entero- and vasculo-BD.2 The reported incidence of vascular complications in BD patients is variable, ranging 3.6– 24%, with large vessel involvement in 1.3–2.2% of all BD patients.3 The involvement of intracranial vessels is extremely rare, with an overall incidence of 0.3–1.5%.4 Vasculo-BD occurs when BD affects the arteries, veins and blood vessels, and it can present with three major manifestations: venous occlusion, arterial occlusion and aneurysm formation. Aorta and pulmonary artery aneurysm formations in vasculo-BD are the most frequently seen manifestations and are among the most life-threatening complications.5 The
prognosis depends upon accurate diagnosis and proper therapeutic strategy. Here, we report on a vasculo-BD patient presenting with oculomotor nerve palsy associated with an internal carotid artery-posterior communicating artery aneurysm. This is a lesion that arises around the origin of the posterior communicating artery from the internal carotid artery, and is a potentially lethal condition that, to our knowledge, has not been previously described in vasculo-BD.
CASE REPORT A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. He was admitted to our hospital, and his condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Human leukocyte antigen typing was A2, A24, B39 and B52. Ophthalmological examinations associated with BD were performed, and uveitis was not diagnosed. Oral prednisolone (up to 0.3 mg/kg) was administrated under controlled conditions with loxoprofen sodium and colchicines;
Correspondence: Toshifumi Yamaoka, M.D., Ph.D., Department of Dermatology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan. Email: [email protected] Received 14 August 2014; accepted 14 November 2014.
© 2015 Japanese Dermatological Association
315
T. Yamaoka et al.
however, during tapering of prednisolone to 0.25 mg/kg daily, the patient’s arthritis flared up. The addition of oral cyclosporin (2.5 mg/kg) improved his arthritis, and his C-reactive protein (CRP) levels fluctuated less than 2 mg/dL over an 8-year period. Ten years after his initial clinical manifestations, the patient presented with a cyclic dull pain in his right temporal region, which he had experienced for the past month and that had expanded to his temple. Additionally, the patient had manifestations of right-sided ptosis for the past 10 days and presented with folliculitis on the face (Fig. 1a). Results of laboratory tests were as follows: white blood cell count, 7.95 9 109 cells/L (neutrophils, 52%); hemoglobin, 133 g/L; and platelet count, 2.68 9 1011/L. Serum creatinine and blood urea nitrogen were 141 lmol/L and 8.57 mmol/L, respectively. Liver function, electrolytes and CRP were within normal ranges. Immunoglobulin (Ig)G IgA, and IgM were 8.46, 5.16 and 1.78 g/L, respectively. Magnetic resonance imaging and angiography showed a right internal carotid artery-posterior communicating artery aneurysm (Fig. 1b). Based on these findings, a diagnosis of vasculo-BD was made. Coil embolization was performed immediately after diagnosis and resulted in gradual improvement of the right-sided ptosis over a 2-year period (Fig. 1c).
DISCUSSION Migratory or recurrent superficial venous thrombosis should raise the suspicion for BD and is associated with complicated deep venous thrombosis.6 These conditions are accompanied by arterial disease, as well as caval thrombosis. In patients with vasculo-BD, arterial lesions are frequently localized in the
(a)
(b)
aorta, cerebral, carotid, subclavian, brachial, ulnar, renal and popliteal arteries,7 and vasculo-BD is the only vasculitis known to lead to pulmonary artery aneurysms.3 Conversely, cerebral arterial aneurysm is reported to be rare. The majority of patients present with ruptured aneurysm, resulting in subarachnoid hemorrhage.4 Furthermore, some have multiple aneurysms predominately involving the anterior circulation, particularly the middle cerebral artery.4 Male sex and a positive pathergy test may be risk factors for pulmonary arterial aneurysms8 and may lead to other arterial aneurysms, including cerebral aneurysm in patients with vasculo-BD. Vasculo-BD is frequently experienced in BD patients with disease lasting over 10 years,9 and the first vascular lesion increases the risk for other vascular lesions and progressive multifocal vessel-related complications.10 Therefore, we should pay close attention to the possibility of the disease affecting arteries throughout the whole body in male BD patients who experience long disease duration and have a positive pathergy test. Oculomotor nerve palsy is a well-known clinical manifestation associated with posterior communicating artery aneurysms.11 Direct compression of the oculomotor nerve by the aneurysm is considered to be the mechanism underlying this condition. Patients who present with an acutely ruptured cerebral aneurysm have a 50% mortality rate.12 Therefore, proper diagnostic examinations, including magnetic resonance imaging and angiography, are critical for accurate diagnoses and prevention of cerebral aneurysm rupture. Surgical clipping has been considered the mainstay of treatment strategy. Recent advances in microcatheter technology, endovascular techniques and embolization material have increased the neurointerventional therapy in cerebral aneurysms patients.13,14 In this
(c)
Figure 1. Clinical and angiographic manifestations present in the patient. (a) Before coil embolization, the patient had right-sided ptosis and folliculitis on the face. (b) Angiography of the right carotid artery indicated an internal carotid artery/posterior communicating artery aneurysm. (c) After coil embolization, the right-sided ptosis gradually improved.
316
© 2015 Japanese Dermatological Association
Vasculo-Behcßet’s disease
patient, the clinical manifestations were compatible with oculomotor nerve palsy, associated with unruptured internal carotid artery-posterior communicating artery aneurysm, and magnetic resonance imaging and angiography were useful for the detection of aneurysm, leading to a diagnosis of vasculo-BD. An early treatment strategy, namely, coil embolization, greatly improved his oculomotor nerve palsy by alleviating the compression on the oculomotor nerve. In conclusion, our findings indicate that abnormal manifestations, such as oculomotor nerve palsy, may be important indicators of complicated vasculo-BD.
6
7
8
9 10
CONFLICT OF INTEREST:
None declared. 11
REFERENCES 1 Yurdakul S, Yazici H. Behcet’s syndrome. Best Pract Res Clin Rheumatol 2008; 22: 793–809. 2 Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 1990; 335: 1078–1080. 3 Al-Basheer M, Hadadin F. Aneurysm formation type of vasculo-Behcet’s disease. Heart Lung Circ 2007; 16: 407–409. 4 Kurdi M, Baeesa S, Bin-Mahfoodh M, Kurdi K. Onyx embolization of ruptured intracranial aneurysm associated with Behcet’s disease. Case Rep Vasc Med Published online: 2013; 2013: 797045. 5 Pay S, Abbasov T, Erdem H et al. Serum MMP-2 and MMP-9 in patients with Behcet’s disease: do their higher levels correlate to
© 2015 Japanese Dermatological Association
12
13
14
vasculo-Behcet’s disease associated with aneurysm formation? Clin Exp Rheumatol 2007; 25: S70–S75. Tunc R, Keyman E, Melikoglu M, Fresko I, Yazici H. Target organ associations in Turkish patients with Behcet’s disease: a cross sectional study by exploratory factor analysis. J Rheumatol 2002; 29: 2393–2396. Alpagut U, Ugurlucan M, Dayioglu E. Major arterial involvement and review of Behcet’s disease. Ann Vasc Surg 2007; 21: 232–239. Hirohata S, Kikuchi H. Histopathology of the ruptured pulmonary artery aneurysm in a patient with Behcet’s disease. Clin Exp Rheumatol 2009; 27: S91–S95. Koc Y, Gullu I, Akpek G et al. Vascular involvement in Behcet’s disease. J Rheumatol 1992; 19: 402–410. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behcet’s disease: an update. Curr Opin Rheumatol 2011; 23: 24–31. Chen PR, Amin-Hanjani S, Albuquerque FC, McDougall C, Zabramski JM, Spetzler RF. Outcome of oculomotor nerve palsy from posterior communicating artery aneurysms: comparison of clipping and coiling. Neurosurgery 2006; 58: 1040–1046. Higashida RT, Smith W, Gress D et al. Intravascular stent and endovascular coil placement for a ruptured fusiform aneurysm of the basilar artery. Case report and review of the literature. J Neurosurg 1997; 87: 944–949. Brilstra EH, Rinkel GJ, van der Graaf Y, van Rooij WJ, Algra A. Treatment of intracranial aneurysms by embolization with coils: a systematic review. Stroke 1999; 30: 470–476. Bhatti MT, Peters KR, Firment C, Mericle RA. Delayed exacerbation of third nerve palsy due to aneurysmal regrowth after endovascular coil embolization. J Neuroophthalmol 2004; 24: 3–10.
317
doi: 10.1111/1346-8138.12752
CONCISE COMMUNICATION
Case of Behc ß et’s disease complicated by oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm Toshifumi YAMAOKA, Hiroyuki MUROTA, Ichiro KATAYAMA Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan
ABSTRACT Behc ß et’s disease (BD) is a relapsing systemic inflammatory disorder of unknown etiology involving systemic vasculitis. Vasculitis in BD results from the involvement of arteries, veins and blood vessels of all sizes, which leads to the three major manifestations of this condition: venous occlusion, arterial occlusion and aneurysm formation. Therefore, whole-body vascular involvement should always be considered in BD patients. Here, we describe the first appearance of an internal carotid-posterior communicating artery aneurysm, resulting in complete oculomotor nerve palsy in a BD patient. A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. His condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Ten years after his initial clinical presentation, he had manifestations of right-sided ptosis and cyclic dull pain in his right temporal region. Magnetic resonance imaging and angiography revealed a right internal carotid artery-posterior communicating artery aneurysm. Although oculomotor nerve palsy associated with internal carotid artery-posterior communicating artery aneurysm in a BD patient has not been reported previously, our report highlights the fact that this abnormal manifestation should be considered in those with vasculo-BD.
Key words: Behcßet’s disease, internal carotid artery-posterior communicating artery aneurysm, oculomotor nerve palsy, various neutrophilic dermatosis, vasculo-Behcßet’s disease.
INTRODUCTION Behc ß et’s disease (BD) is an inflammatory disorder of unknown etiology, for which an immunological pathogenesis has been proposed. It is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis and various neutrophilic dermatoses. Vasculitis is thought to be a pivotal factor underlying most clinical manifestations of BD. This disease is common in the Middle East and Japan but rare in Northern Europe and the USA, and although it typically affects young adults, it can also occur in children.1 BD is divided into three subtypes, comprising neuro-, entero- and vasculo-BD.2 The reported incidence of vascular complications in BD patients is variable, ranging 3.6– 24%, with large vessel involvement in 1.3–2.2% of all BD patients.3 The involvement of intracranial vessels is extremely rare, with an overall incidence of 0.3–1.5%.4 Vasculo-BD occurs when BD affects the arteries, veins and blood vessels, and it can present with three major manifestations: venous occlusion, arterial occlusion and aneurysm formation. Aorta and pulmonary artery aneurysm formations in vasculo-BD are the most frequently seen manifestations and are among the most life-threatening complications.5 The
prognosis depends upon accurate diagnosis and proper therapeutic strategy. Here, we report on a vasculo-BD patient presenting with oculomotor nerve palsy associated with an internal carotid artery-posterior communicating artery aneurysm. This is a lesion that arises around the origin of the posterior communicating artery from the internal carotid artery, and is a potentially lethal condition that, to our knowledge, has not been previously described in vasculo-BD.
CASE REPORT A 44-year-old Japanese man suffered from recurrent episodes of erythema nodosum that had presented on the lower extremities for the past 2 years. He was admitted to our hospital, and his condition was diagnosed as an incomplete type of BD based on relapsing oral and genital ulcers, skin eruptions, such as erythema nodosum and folliculitis, a positive pathergy test and systemic arthralgia. Human leukocyte antigen typing was A2, A24, B39 and B52. Ophthalmological examinations associated with BD were performed, and uveitis was not diagnosed. Oral prednisolone (up to 0.3 mg/kg) was administrated under controlled conditions with loxoprofen sodium and colchicines;
Correspondence: Toshifumi Yamaoka, M.D., Ph.D., Department of Dermatology, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan. Email: [email protected] Received 14 August 2014; accepted 14 November 2014.
© 2015 Japanese Dermatological Association
315
T. Yamaoka et al.
however, during tapering of prednisolone to 0.25 mg/kg daily, the patient’s arthritis flared up. The addition of oral cyclosporin (2.5 mg/kg) improved his arthritis, and his C-reactive protein (CRP) levels fluctuated less than 2 mg/dL over an 8-year period. Ten years after his initial clinical manifestations, the patient presented with a cyclic dull pain in his right temporal region, which he had experienced for the past month and that had expanded to his temple. Additionally, the patient had manifestations of right-sided ptosis for the past 10 days and presented with folliculitis on the face (Fig. 1a). Results of laboratory tests were as follows: white blood cell count, 7.95 9 109 cells/L (neutrophils, 52%); hemoglobin, 133 g/L; and platelet count, 2.68 9 1011/L. Serum creatinine and blood urea nitrogen were 141 lmol/L and 8.57 mmol/L, respectively. Liver function, electrolytes and CRP were within normal ranges. Immunoglobulin (Ig)G IgA, and IgM were 8.46, 5.16 and 1.78 g/L, respectively. Magnetic resonance imaging and angiography showed a right internal carotid artery-posterior communicating artery aneurysm (Fig. 1b). Based on these findings, a diagnosis of vasculo-BD was made. Coil embolization was performed immediately after diagnosis and resulted in gradual improvement of the right-sided ptosis over a 2-year period (Fig. 1c).
DISCUSSION Migratory or recurrent superficial venous thrombosis should raise the suspicion for BD and is associated with complicated deep venous thrombosis.6 These conditions are accompanied by arterial disease, as well as caval thrombosis. In patients with vasculo-BD, arterial lesions are frequently localized in the
(a)
(b)
aorta, cerebral, carotid, subclavian, brachial, ulnar, renal and popliteal arteries,7 and vasculo-BD is the only vasculitis known to lead to pulmonary artery aneurysms.3 Conversely, cerebral arterial aneurysm is reported to be rare. The majority of patients present with ruptured aneurysm, resulting in subarachnoid hemorrhage.4 Furthermore, some have multiple aneurysms predominately involving the anterior circulation, particularly the middle cerebral artery.4 Male sex and a positive pathergy test may be risk factors for pulmonary arterial aneurysms8 and may lead to other arterial aneurysms, including cerebral aneurysm in patients with vasculo-BD. Vasculo-BD is frequently experienced in BD patients with disease lasting over 10 years,9 and the first vascular lesion increases the risk for other vascular lesions and progressive multifocal vessel-related complications.10 Therefore, we should pay close attention to the possibility of the disease affecting arteries throughout the whole body in male BD patients who experience long disease duration and have a positive pathergy test. Oculomotor nerve palsy is a well-known clinical manifestation associated with posterior communicating artery aneurysms.11 Direct compression of the oculomotor nerve by the aneurysm is considered to be the mechanism underlying this condition. Patients who present with an acutely ruptured cerebral aneurysm have a 50% mortality rate.12 Therefore, proper diagnostic examinations, including magnetic resonance imaging and angiography, are critical for accurate diagnoses and prevention of cerebral aneurysm rupture. Surgical clipping has been considered the mainstay of treatment strategy. Recent advances in microcatheter technology, endovascular techniques and embolization material have increased the neurointerventional therapy in cerebral aneurysms patients.13,14 In this
(c)
Figure 1. Clinical and angiographic manifestations present in the patient. (a) Before coil embolization, the patient had right-sided ptosis and folliculitis on the face. (b) Angiography of the right carotid artery indicated an internal carotid artery/posterior communicating artery aneurysm. (c) After coil embolization, the right-sided ptosis gradually improved.
316
© 2015 Japanese Dermatological Association
Vasculo-Behcßet’s disease
patient, the clinical manifestations were compatible with oculomotor nerve palsy, associated with unruptured internal carotid artery-posterior communicating artery aneurysm, and magnetic resonance imaging and angiography were useful for the detection of aneurysm, leading to a diagnosis of vasculo-BD. An early treatment strategy, namely, coil embolization, greatly improved his oculomotor nerve palsy by alleviating the compression on the oculomotor nerve. In conclusion, our findings indicate that abnormal manifestations, such as oculomotor nerve palsy, may be important indicators of complicated vasculo-BD.
6
7
8
9 10
CONFLICT OF INTEREST:
None declared. 11
REFERENCES 1 Yurdakul S, Yazici H. Behcet’s syndrome. Best Pract Res Clin Rheumatol 2008; 22: 793–809. 2 Criteria for diagnosis of Behcet’s disease. International Study Group for Behcet’s Disease. Lancet 1990; 335: 1078–1080. 3 Al-Basheer M, Hadadin F. Aneurysm formation type of vasculo-Behcet’s disease. Heart Lung Circ 2007; 16: 407–409. 4 Kurdi M, Baeesa S, Bin-Mahfoodh M, Kurdi K. Onyx embolization of ruptured intracranial aneurysm associated with Behcet’s disease. Case Rep Vasc Med Published online: 2013; 2013: 797045. 5 Pay S, Abbasov T, Erdem H et al. Serum MMP-2 and MMP-9 in patients with Behcet’s disease: do their higher levels correlate to
© 2015 Japanese Dermatological Association
12
13
14
vasculo-Behcet’s disease associated with aneurysm formation? Clin Exp Rheumatol 2007; 25: S70–S75. Tunc R, Keyman E, Melikoglu M, Fresko I, Yazici H. Target organ associations in Turkish patients with Behcet’s disease: a cross sectional study by exploratory factor analysis. J Rheumatol 2002; 29: 2393–2396. Alpagut U, Ugurlucan M, Dayioglu E. Major arterial involvement and review of Behcet’s disease. Ann Vasc Surg 2007; 21: 232–239. Hirohata S, Kikuchi H. Histopathology of the ruptured pulmonary artery aneurysm in a patient with Behcet’s disease. Clin Exp Rheumatol 2009; 27: S91–S95. Koc Y, Gullu I, Akpek G et al. Vascular involvement in Behcet’s disease. J Rheumatol 1992; 19: 402–410. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behcet’s disease: an update. Curr Opin Rheumatol 2011; 23: 24–31. Chen PR, Amin-Hanjani S, Albuquerque FC, McDougall C, Zabramski JM, Spetzler RF. Outcome of oculomotor nerve palsy from posterior communicating artery aneurysms: comparison of clipping and coiling. Neurosurgery 2006; 58: 1040–1046. Higashida RT, Smith W, Gress D et al. Intravascular stent and endovascular coil placement for a ruptured fusiform aneurysm of the basilar artery. Case report and review of the literature. J Neurosurg 1997; 87: 944–949. Brilstra EH, Rinkel GJ, van der Graaf Y, van Rooij WJ, Algra A. Treatment of intracranial aneurysms by embolization with coils: a systematic review. Stroke 1999; 30: 470–476. Bhatti MT, Peters KR, Firment C, Mericle RA. Delayed exacerbation of third nerve palsy due to aneurysmal regrowth after endovascular coil embolization. J Neuroophthalmol 2004; 24: 3–10.
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