å¡ CASE REPORT å¡
Unilateral Facial Swelling and Exophthalmos in a Patient with Polyarteritis Nodosa Kazuyuki Ishida, Tsukasa Yokota, Yoshiaki Wada, Masahito Yamada and Hiroshi Tsukagoshi A patient with polyarteritis nodosa (PN) presenting with exophthalmos and facial swelling, which are rarely found in PN, is reported. The patient, a 27-year-old male, complained of painful facial swelling and diplopia. Physical examinations showed facial swelling around the right orbit and exophthalmos. After admission, he experienced myalgia in both calves. Laboratory studies disclosed leukocytosis and liver dysfunction. Celiac and renal angiograms showed aneurysms. A biopsy specimen of his left calf showed an arterial inflammatory process. The patient was diagnosed as having PN. He had an excellent response to corticosteroids, with prompt improvement. (Internal Medicine 31: 500-503, 1992) Key words: ocular proptosis, systemic vasculitis, MRI scan, muscle biopsy, steroid therapy
Introduction Polyarteritis nodosa (PN) is a systemic disease in volving small- and medium-sized arteries and veins of many organ systems, including the kidney, heart, gastrointestinal tract, muscle and nervous system, with widely varied manifestations (1). Morbidity and mor tality are related to the type and degree of organ system involvement The eye, orbit,(1). and skin are affected in about 10% of patients with PN, as types of systemic vasculitis or as local, atypical forms (2, 3). Common ophthalmologic compli cations of PN, are episcleritis, hypertensive retinopathy, central retinal artery occlusion, choroidal insufficiency, extraocular muscle dysfunction and ischemic optic neuropathy (2, 4, 5). Cutaneous involvement of PN is also common, which includes ulcers, livedo, nodules and gangrene (6). However exophthalmos and facial swelling secondary to PN are very rare (5, 7-ll). Such symptoms are commonly found in otherwith connective Here, we report a case of PN presenting exoph tissue diseases like Wegener's granulomatosis (12-14). thalmos and facial swelling.
referred from an oral surgeon to our neurologic clinic. He complained of painful swelling of the right face and double vision. The patient had been in good health until one month prior to admission to our hospital, when he noticed painful unilateral facial swelling around the right eye, followed by a lowatgrade fever and diplopia. He underwent examination a dental hospital, was
diagnosed as having Quincke's edema, and was treated with oral prednisolone (30mg daily), resulting in im provement of his symptoms. However, three weeks later the same symptoms recurred, with additional symptoms of impaired vision of the right eye, lower abdominal pain, myalgia in both At ourand initial evaluation in calves. January, he had a remittent fever up to 40°C, his blood pressure was 134/90mmHg, and pulse rate was 88/min. There was an obvious swelling on the right side of the face around the orbit with a puffy eyelid, a narrow palpebral fissure, slight proptosis, and an injected conjunctiva on the sideor(Fig. left). There were no abnormalities inright the oral nasal1,cavity. Lymphadenopathy was absent. The chest was clear to percussion and auscultation, and palpation of the abdomen was negative. Nodules, skin rash, and edema were not found. Neurological examination revealed Case Report diplopia in all directions. The ocular movements were within normal limits. Visual acuity was 0.2 on the right In January 1990, a 27-year-old man, an engineer, was and 0.5 on the left. Visual fields were intact. Funduscopic From the Department of Neurology, Faculty of Medicine, Tokyo Medical and Dental University, Tokyo Received for publication May 9, 1991; Accepted for publication October 25, 1991 Reprint requests should be addressed to Dr. Kazuyuki Ishida, the Kakeyu Hospital, 1308 Nishiuchi, Maruko-machi, Chiisagata-gun, Naga 386-03, Japan 500
Internal
Medicine
Vol. 31, No. 4 (April
1992)
PN with Facial
Swelling
and Exophthalmos
total bilirubin 0.9 mg/dl, lactic dehydrogenase 364 U/l, glutamic oxaloacetic transaminase 38U/l, glutamic pyruvic transaminase (GPT) 122U/l, alkaline phos phatase 582U/l, and gamma-glutamyl transpeptidase 237U/l. The creatine kinase level was normal and the amylase value was slightly high. The erythrocyte sedi mentation rate was 78mm/h, C-reactive protein (CRP) was 6+. Tests for rheumatoid factor, antinuclear antibody, and circulating immune complexes were negative. A quantitative analysis of immunoglobulins disclosed that IgG was 616mg/dl, IgM below 46mg/dl, and IgA 167mg/dl. The serum C3 level was 154mg/dl, C4 51mg/ dl, and CH50 49.3 U/ml. A test for HBs antigen was nega tive. Thyroid function tests were normal. Although Fig. 1. Appearance of the patient before treatment, showing Aurinary roentgenogram of thenormal, chest showed no abnor findings were stools were positive for swelling of the right face and proptosis (left), and after treatmentmalities. An MRI scan of the orbit revealed thickening occult blood. (right). of the right lateral rectal muscle, ipsilateral proptosis, swelling of the right optic nerve and enlargement of the examination revealed no papilledema, retinal hemor retrobulbar space, though retrobulbar space-occupying rhage, or edema. The patient had muscle pain in both lesions were not detected (Fig. 2). A CT scan of the calves. There was no weakness of muscles, except for the orbit showed the proptosis, but no other abnormalities plantar flexors of both ankles. The myalgia prevented could be detected. A bariumenema examination dis him from plantar flexion of the ankles. The deep tendon closed a sclerotic lesion on the descending colon that reflexes of the extremities were equal and normoactive, indicated ischemia or local inflammation. Celiac and and no abnormal reflexes were found. Sensory abnor renalweeks angiograms showed small aneurysms the liver Laboratory findings on admission were as follows: Two after admission, the patient notedinsensory malities were not noted. and right kidney. loss corresponding to the distribution of the median the white blood cell count was 19,200/mm3, with 77% nerve bilaterally. He lost 8 kg in the three weeks before neutrophils and 8% eosinophils. The platelet count treatment. Biopsy of the right gastrocnemius muscle was was 19.2 x 104/mm3. Serum electrolyte values were all performed. The muscle biopsy specimen showed cellular within normal limits. The urea nitrogen was ll mg/dl, infiltration involving a small artery and the surrounding the creatinine 0.9mg/dl. Fasting plasma glucose con connective tissue, with disruption of the elastic membrane centration was 145 mg/dl. Liver function tests showed
Fig. 2. MRI scan on admission, showing exophthalmos of the right eye, thickening of the lateral rectal muscle (arrow), and swelling of the right optic nerve, (right; TR = 400, TE = 38. left; TR - 1800, TE = 90) Internal
Medicine
Vol. 31, No. 4 (April
1992)
501
Ishida
et al
Fig. 3. Biopsy section of the muscle. (A, B) Acute inflammatory process of a small artery involving connective tissue and muscle fibers the artery. (C) Disruption of internal elastic lamina of the artery. (A) HE stain, x40. (B) HE stain, X200. (C) Elastica-van Gieson stain, X (Fig. 3). He was diagnosed to have PN based on the criteria established by the Ministry of Health and Welfare, Japan (15). He was then immediately treated with methylprednisolone (1,000mg, daily, i.v.) for three days, followed by 60mg of prednisolone orally every day. After the pulse therapy, facial pain, abdominal pain, and muscle pain subsided, and facial swelling and exophthalmos improved gradually (Fig. 1, right). One Prednisolone wassensory successfully tapered by 5mg every month later, his loss disappeared completely. two weeks to a maintenance dose of lOmg/day. Now he is being followed in the outpatient service without any relapse. Discussion The present patient had many symptoms and signs that indicated the diagnosis of PN: pyrexia, weight loss, peripheral neuritis, myalgia, hematochezia, leukocytosis, radiologically multiple aneurysms, and histologically vasculitis with disruption of the elastic membrane. These symptoms completely fulfilled the diagnostic criteria for PN established by the Ministry of Health and Welfare, Japan, in 1990 (16), as well as those promulgated by the American College of Rheumatology in 1990 (17). The biopsy revealed transmural inflammation of a small artery without granulation or scaring, which indicated an acute The present patient an elevated value of GPT. inflammatory stagehad of the disease (16). This appeared to be a result of ischemic hepatitis sec502
ondary to intrahepatic arteritis, or direct damage due to arterial inflammation, because the aneurysms on the celiac angiogram indicated arteritis in the liver. The levels of the serum immunoglobulins prior to therapy were decreased; these normalized after therapy. Two reasons were suspected: consumption of immunoglobulins and a decrease in their production. But consumption was unlikely because the complement levels were within normal limits. Rather, the decreased production seemed to be due to abnormal immuno-regulation. The abnor malities of both GPT and responded The patient presented withimmunoglobulins exophthalmos and facial to therapy. swelling as the initial manifestations. These manifestations are uncommon in PN. Five cases with exophthalmos (5, 7-10), and only one case with facial swelling (ll), secondary to PN have been reported. The combination of both found in this patient has not been previously reported. Furthermore, this is the first report in which an MRI scan was used for the study of exophthalmos The pathomechanism of exophthalmos and facial secondary to PN. swelling in PN has not yet been fully elucidated. Bagegni et al reported a patient who presented with bilateral proptosis due to PN (5). The CT scan showed abnormal soft tissue behind both eyes. Orbital biopsy demonstrated acute vasculitis and fibrosis the surrounding tissue. However, in other reports, theofhistologically-confirmed mechanical compression from the granulomatous tissue was suggested as the cause of exophthalmos in PN (9, 10). On the other hand, Ryan and Brady reported a Internal
Medicine
Vol. 31, No. 4 (April
1992)
PN with Facial Swelling
and Exophthalmos
case with unilateral facial swelling, and attributed the
Sheehan B, Harriman DGF, Bradshaw JPP. Polyarteritis nodosa with ophthalmic and neurological complications. Arch Ophthalmol swelling to edema in the masseter muscles (ll). In the 60: 537, 1958. Hutchinson CH. Polyarteritis nodosa presenting as posterior present case, although the cause of neither exophthalmos ischemic optic neuropathy. J R Soc Med 77: 1043, 1984. nor facial sweelling was histopathologically elucidated, Bagegni A, Lyness RW, Johnston PB, Douglas JF. Visual recovery in orbital vasculitis. Br J Ophthalmol 72: 737, 1988. the MRI scan disclosed thickening of the extraocular Moreland LW, Ball GV. Cutaneous polyarteritis nodosa. Am J muscle and enlargement of the retrobulbar space. Both Med 64: 890, 1978. Van Wein S, Merz HE. Exophthalmos secondary to periarteritis the visual impairment which was responsive to treatment nodosa. Am J Ophthalmol 56: 204, 1963. and the swelling of the optic nerve on the MRI scan, may Hope-Robertson WJ. Pseudo-tumor of the orbit as a presenting sign in periarteritis nodosa. Trans Ophthalmol Soc NZ 8: 56, 1956. support the presence of intraorbital vasculitis. These Walton EW. Pseudo tumor of the orbit and polyarteritis nodosa. findings similarly J Clin Pathol 12: 419, 1959. The MRI suggest finding that is notintraorbital peculiar tovasculitis the present case Iyota T. A case of bilateral orbital pseudo-tumor found in peri caused edema of the components in both the orbit and and is sometimes seen in other conditions, namely arteritis nodosa. Jpn Rev Clin Ophthalmol 12: 59, 1965 (in the surrounding subcutaneous tissue. inflammatory orbital pseudotumor (18, 19). Therefore, Japanese). Ryan D, Brady F. Polyarteritis nodosa: unusual cause of facial it is impossible to reach a final diagnosis simply based pain and swelling. Acta Stomatol Int 13: 17, 1987. Bullen CL, Liesegang TJ, Mcdonald TJ, Derenee RA. Ocular upon the radiological studies. But MRI is helpful in complications of Wegener's granulomatosis. Ophthalmology 90: confirming the mechanism of exophthalmos and in 279, 1983. In summary, the present case indicates that systemic Faulds JS, Wear AR. Pseudotumor of the orbit and Wegener's narrowing the differential diagnosis. granuloma. Lancet 29: 955, 1960. vasculitis, such as PN, can cause the unusual clinical Harcourt RB. Orbital granulomata associated with wide spread manifestations of exophthalmos and facial swelling. angiitis. Br J Ophthalmol 48: 673, 1964. Acknowledgments: We are very grateful to Dr. J. Nakayama of the Fukuda Y, Hashimoto H. Diagnosis of necrotizing vasculitis. in: Abe M, Omae T, Kawai T, Honma M, Eds. Collagen Disease: Department of Clinical Pathology, Shinsyu University, for his technical Naika MOOK 28, Kanehara Syuppan, Tokyo, 1985, p. 175 assistance and the histological analysis of the biopsy specimen. (in Japanese). Nagasawa T. Diagnostic criteria and classification of polyarteritis References nodosa. Intern Med 65 (Suppl): 1286, 1990 (in Japanese). Lightfoot RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Fauci AS, Haynes BS, Katz P. The spectrum ofvasculitis: clinical, polyarteritis nodosa. Arthritis Rheum 33: 1088, 1990. pathologic, immunologic, and therapeutic consideration. Ann Blodi FC, Gass JDM. Inflammatory pseudotumour of the orbit. Intern Med 89: 660, 1978. Cohen RD, Conn DL, Ilstrup DM. Clinical features, prognosis, Br J Ophthalmol 52: 79, 1968. and response to treatment in polyarteritis. Mayo Clin Proc 55: Shiga H, Yagishita A, et al. Diagnostic significance of computed tomography in unilateral exophthalmos. Neurol Med Chir 20: 146, 1980. 37,1980.
Internal
Medicine
Vol. 31, No. 4 (April
1992)
503
Unilateral Facial Swelling and Exophthalmos in a Patient with Polyarteritis Nodosa Kazuyuki Ishida, Tsukasa Yokota, Yoshiaki Wada, Masahito Yamada and Hiroshi Tsukagoshi A patient with polyarteritis nodosa (PN) presenting with exophthalmos and facial swelling, which are rarely found in PN, is reported. The patient, a 27-year-old male, complained of painful facial swelling and diplopia. Physical examinations showed facial swelling around the right orbit and exophthalmos. After admission, he experienced myalgia in both calves. Laboratory studies disclosed leukocytosis and liver dysfunction. Celiac and renal angiograms showed aneurysms. A biopsy specimen of his left calf showed an arterial inflammatory process. The patient was diagnosed as having PN. He had an excellent response to corticosteroids, with prompt improvement. (Internal Medicine 31: 500-503, 1992) Key words: ocular proptosis, systemic vasculitis, MRI scan, muscle biopsy, steroid therapy
Introduction Polyarteritis nodosa (PN) is a systemic disease in volving small- and medium-sized arteries and veins of many organ systems, including the kidney, heart, gastrointestinal tract, muscle and nervous system, with widely varied manifestations (1). Morbidity and mor tality are related to the type and degree of organ system involvement The eye, orbit,(1). and skin are affected in about 10% of patients with PN, as types of systemic vasculitis or as local, atypical forms (2, 3). Common ophthalmologic compli cations of PN, are episcleritis, hypertensive retinopathy, central retinal artery occlusion, choroidal insufficiency, extraocular muscle dysfunction and ischemic optic neuropathy (2, 4, 5). Cutaneous involvement of PN is also common, which includes ulcers, livedo, nodules and gangrene (6). However exophthalmos and facial swelling secondary to PN are very rare (5, 7-ll). Such symptoms are commonly found in otherwith connective Here, we report a case of PN presenting exoph tissue diseases like Wegener's granulomatosis (12-14). thalmos and facial swelling.
referred from an oral surgeon to our neurologic clinic. He complained of painful swelling of the right face and double vision. The patient had been in good health until one month prior to admission to our hospital, when he noticed painful unilateral facial swelling around the right eye, followed by a lowatgrade fever and diplopia. He underwent examination a dental hospital, was
diagnosed as having Quincke's edema, and was treated with oral prednisolone (30mg daily), resulting in im provement of his symptoms. However, three weeks later the same symptoms recurred, with additional symptoms of impaired vision of the right eye, lower abdominal pain, myalgia in both At ourand initial evaluation in calves. January, he had a remittent fever up to 40°C, his blood pressure was 134/90mmHg, and pulse rate was 88/min. There was an obvious swelling on the right side of the face around the orbit with a puffy eyelid, a narrow palpebral fissure, slight proptosis, and an injected conjunctiva on the sideor(Fig. left). There were no abnormalities inright the oral nasal1,cavity. Lymphadenopathy was absent. The chest was clear to percussion and auscultation, and palpation of the abdomen was negative. Nodules, skin rash, and edema were not found. Neurological examination revealed Case Report diplopia in all directions. The ocular movements were within normal limits. Visual acuity was 0.2 on the right In January 1990, a 27-year-old man, an engineer, was and 0.5 on the left. Visual fields were intact. Funduscopic From the Department of Neurology, Faculty of Medicine, Tokyo Medical and Dental University, Tokyo Received for publication May 9, 1991; Accepted for publication October 25, 1991 Reprint requests should be addressed to Dr. Kazuyuki Ishida, the Kakeyu Hospital, 1308 Nishiuchi, Maruko-machi, Chiisagata-gun, Naga 386-03, Japan 500
Internal
Medicine
Vol. 31, No. 4 (April
1992)
PN with Facial
Swelling
and Exophthalmos
total bilirubin 0.9 mg/dl, lactic dehydrogenase 364 U/l, glutamic oxaloacetic transaminase 38U/l, glutamic pyruvic transaminase (GPT) 122U/l, alkaline phos phatase 582U/l, and gamma-glutamyl transpeptidase 237U/l. The creatine kinase level was normal and the amylase value was slightly high. The erythrocyte sedi mentation rate was 78mm/h, C-reactive protein (CRP) was 6+. Tests for rheumatoid factor, antinuclear antibody, and circulating immune complexes were negative. A quantitative analysis of immunoglobulins disclosed that IgG was 616mg/dl, IgM below 46mg/dl, and IgA 167mg/dl. The serum C3 level was 154mg/dl, C4 51mg/ dl, and CH50 49.3 U/ml. A test for HBs antigen was nega tive. Thyroid function tests were normal. Although Fig. 1. Appearance of the patient before treatment, showing Aurinary roentgenogram of thenormal, chest showed no abnor findings were stools were positive for swelling of the right face and proptosis (left), and after treatmentmalities. An MRI scan of the orbit revealed thickening occult blood. (right). of the right lateral rectal muscle, ipsilateral proptosis, swelling of the right optic nerve and enlargement of the examination revealed no papilledema, retinal hemor retrobulbar space, though retrobulbar space-occupying rhage, or edema. The patient had muscle pain in both lesions were not detected (Fig. 2). A CT scan of the calves. There was no weakness of muscles, except for the orbit showed the proptosis, but no other abnormalities plantar flexors of both ankles. The myalgia prevented could be detected. A bariumenema examination dis him from plantar flexion of the ankles. The deep tendon closed a sclerotic lesion on the descending colon that reflexes of the extremities were equal and normoactive, indicated ischemia or local inflammation. Celiac and and no abnormal reflexes were found. Sensory abnor renalweeks angiograms showed small aneurysms the liver Laboratory findings on admission were as follows: Two after admission, the patient notedinsensory malities were not noted. and right kidney. loss corresponding to the distribution of the median the white blood cell count was 19,200/mm3, with 77% nerve bilaterally. He lost 8 kg in the three weeks before neutrophils and 8% eosinophils. The platelet count treatment. Biopsy of the right gastrocnemius muscle was was 19.2 x 104/mm3. Serum electrolyte values were all performed. The muscle biopsy specimen showed cellular within normal limits. The urea nitrogen was ll mg/dl, infiltration involving a small artery and the surrounding the creatinine 0.9mg/dl. Fasting plasma glucose con connective tissue, with disruption of the elastic membrane centration was 145 mg/dl. Liver function tests showed
Fig. 2. MRI scan on admission, showing exophthalmos of the right eye, thickening of the lateral rectal muscle (arrow), and swelling of the right optic nerve, (right; TR = 400, TE = 38. left; TR - 1800, TE = 90) Internal
Medicine
Vol. 31, No. 4 (April
1992)
501
Ishida
et al
Fig. 3. Biopsy section of the muscle. (A, B) Acute inflammatory process of a small artery involving connective tissue and muscle fibers the artery. (C) Disruption of internal elastic lamina of the artery. (A) HE stain, x40. (B) HE stain, X200. (C) Elastica-van Gieson stain, X (Fig. 3). He was diagnosed to have PN based on the criteria established by the Ministry of Health and Welfare, Japan (15). He was then immediately treated with methylprednisolone (1,000mg, daily, i.v.) for three days, followed by 60mg of prednisolone orally every day. After the pulse therapy, facial pain, abdominal pain, and muscle pain subsided, and facial swelling and exophthalmos improved gradually (Fig. 1, right). One Prednisolone wassensory successfully tapered by 5mg every month later, his loss disappeared completely. two weeks to a maintenance dose of lOmg/day. Now he is being followed in the outpatient service without any relapse. Discussion The present patient had many symptoms and signs that indicated the diagnosis of PN: pyrexia, weight loss, peripheral neuritis, myalgia, hematochezia, leukocytosis, radiologically multiple aneurysms, and histologically vasculitis with disruption of the elastic membrane. These symptoms completely fulfilled the diagnostic criteria for PN established by the Ministry of Health and Welfare, Japan, in 1990 (16), as well as those promulgated by the American College of Rheumatology in 1990 (17). The biopsy revealed transmural inflammation of a small artery without granulation or scaring, which indicated an acute The present patient an elevated value of GPT. inflammatory stagehad of the disease (16). This appeared to be a result of ischemic hepatitis sec502
ondary to intrahepatic arteritis, or direct damage due to arterial inflammation, because the aneurysms on the celiac angiogram indicated arteritis in the liver. The levels of the serum immunoglobulins prior to therapy were decreased; these normalized after therapy. Two reasons were suspected: consumption of immunoglobulins and a decrease in their production. But consumption was unlikely because the complement levels were within normal limits. Rather, the decreased production seemed to be due to abnormal immuno-regulation. The abnor malities of both GPT and responded The patient presented withimmunoglobulins exophthalmos and facial to therapy. swelling as the initial manifestations. These manifestations are uncommon in PN. Five cases with exophthalmos (5, 7-10), and only one case with facial swelling (ll), secondary to PN have been reported. The combination of both found in this patient has not been previously reported. Furthermore, this is the first report in which an MRI scan was used for the study of exophthalmos The pathomechanism of exophthalmos and facial secondary to PN. swelling in PN has not yet been fully elucidated. Bagegni et al reported a patient who presented with bilateral proptosis due to PN (5). The CT scan showed abnormal soft tissue behind both eyes. Orbital biopsy demonstrated acute vasculitis and fibrosis the surrounding tissue. However, in other reports, theofhistologically-confirmed mechanical compression from the granulomatous tissue was suggested as the cause of exophthalmos in PN (9, 10). On the other hand, Ryan and Brady reported a Internal
Medicine
Vol. 31, No. 4 (April
1992)
PN with Facial Swelling
and Exophthalmos
case with unilateral facial swelling, and attributed the
Sheehan B, Harriman DGF, Bradshaw JPP. Polyarteritis nodosa with ophthalmic and neurological complications. Arch Ophthalmol swelling to edema in the masseter muscles (ll). In the 60: 537, 1958. Hutchinson CH. Polyarteritis nodosa presenting as posterior present case, although the cause of neither exophthalmos ischemic optic neuropathy. J R Soc Med 77: 1043, 1984. nor facial sweelling was histopathologically elucidated, Bagegni A, Lyness RW, Johnston PB, Douglas JF. Visual recovery in orbital vasculitis. Br J Ophthalmol 72: 737, 1988. the MRI scan disclosed thickening of the extraocular Moreland LW, Ball GV. Cutaneous polyarteritis nodosa. Am J muscle and enlargement of the retrobulbar space. Both Med 64: 890, 1978. Van Wein S, Merz HE. Exophthalmos secondary to periarteritis the visual impairment which was responsive to treatment nodosa. Am J Ophthalmol 56: 204, 1963. and the swelling of the optic nerve on the MRI scan, may Hope-Robertson WJ. Pseudo-tumor of the orbit as a presenting sign in periarteritis nodosa. Trans Ophthalmol Soc NZ 8: 56, 1956. support the presence of intraorbital vasculitis. These Walton EW. Pseudo tumor of the orbit and polyarteritis nodosa. findings similarly J Clin Pathol 12: 419, 1959. The MRI suggest finding that is notintraorbital peculiar tovasculitis the present case Iyota T. A case of bilateral orbital pseudo-tumor found in peri caused edema of the components in both the orbit and and is sometimes seen in other conditions, namely arteritis nodosa. Jpn Rev Clin Ophthalmol 12: 59, 1965 (in the surrounding subcutaneous tissue. inflammatory orbital pseudotumor (18, 19). Therefore, Japanese). Ryan D, Brady F. Polyarteritis nodosa: unusual cause of facial it is impossible to reach a final diagnosis simply based pain and swelling. Acta Stomatol Int 13: 17, 1987. Bullen CL, Liesegang TJ, Mcdonald TJ, Derenee RA. Ocular upon the radiological studies. But MRI is helpful in complications of Wegener's granulomatosis. Ophthalmology 90: confirming the mechanism of exophthalmos and in 279, 1983. In summary, the present case indicates that systemic Faulds JS, Wear AR. Pseudotumor of the orbit and Wegener's narrowing the differential diagnosis. granuloma. Lancet 29: 955, 1960. vasculitis, such as PN, can cause the unusual clinical Harcourt RB. Orbital granulomata associated with wide spread manifestations of exophthalmos and facial swelling. angiitis. Br J Ophthalmol 48: 673, 1964. Acknowledgments: We are very grateful to Dr. J. Nakayama of the Fukuda Y, Hashimoto H. Diagnosis of necrotizing vasculitis. in: Abe M, Omae T, Kawai T, Honma M, Eds. Collagen Disease: Department of Clinical Pathology, Shinsyu University, for his technical Naika MOOK 28, Kanehara Syuppan, Tokyo, 1985, p. 175 assistance and the histological analysis of the biopsy specimen. (in Japanese). Nagasawa T. Diagnostic criteria and classification of polyarteritis References nodosa. Intern Med 65 (Suppl): 1286, 1990 (in Japanese). Lightfoot RW, Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Fauci AS, Haynes BS, Katz P. The spectrum ofvasculitis: clinical, polyarteritis nodosa. Arthritis Rheum 33: 1088, 1990. pathologic, immunologic, and therapeutic consideration. Ann Blodi FC, Gass JDM. Inflammatory pseudotumour of the orbit. Intern Med 89: 660, 1978. Cohen RD, Conn DL, Ilstrup DM. Clinical features, prognosis, Br J Ophthalmol 52: 79, 1968. and response to treatment in polyarteritis. Mayo Clin Proc 55: Shiga H, Yagishita A, et al. Diagnostic significance of computed tomography in unilateral exophthalmos. Neurol Med Chir 20: 146, 1980. 37,1980.
Internal
Medicine
Vol. 31, No. 4 (April
1992)
503
