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Sweet's syndrome associated with encephalitis
Journal of the Neurological Sciences 188 Ž2001. 95–97 www.elsevier.comrlocaterjns
Short communication
Sweet’s syndrome associated with encephalitis Kazuyuki Noda a , Yasuyuki Okuma a,) , Jiro Fukae a , Kenji Fujishima a , Keigo Goto a , Hiroko Sadamasa b, Takashi Yoshiike b, Yoshikuni Mizuno c a
Department of Neurology, Juntendo Izu-Nagaoka Hospital, 1129 Nagaoka, Izu-Nagaoka, Tagata-gun, Shizuoka 410-2295, Japan b Department of Dermatology, Juntendo UniÕersity Izu-Nagaoka Hospital, Shizuoka, Japan c Department of Neurology, Juntendo UniÕersity School of Medicine, Tokyo, Japan Received 30 October 2000; received in revised form 19 March 2001; accepted 11 May 2001
Abstract The involvement of the central nervous system ŽCNS. in Sweet’s syndrome Žacute febrile neutrophilic dermatosis. is rare. We report a 47-year-old woman who presented with acute encephalitis and was subsequently diagnosed as having Sweet’s syndrome. She developed altered consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid ŽCSF. examination disclosed neutrophilic pleocytosis without decreased glucose level. Brain magnetic resonance imaging ŽMRI. showed abnormal signal intensity lesions in the basal ganglia and the hippocampus. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet’s syndrome. Treatment with acyclovir and antibiotics failed, but the subsequent corticosteroid therapy was effective. Awareness of neurological complication in Sweet’s syndrome may avoid unnecessary empiric therapy for meningoencephalitis and will lead to a successful treatment with corticosteroids. q 2001 Elsevier Science B.V. All rights reserved. Keywords: Sweet’s syndrome; Encephalitis; Basal ganglia; MRI; Central nervous system; Behc¸et’s disease
1. Introduction
2. Case report
Acute febrile neutrophilic dermatosis, or Sweet’s syndrome, is a distinct syndrome originally described by Sweet in 1964 w1x. The four cardinal features are erythematous plaques on the skin, fever, peripheral neutrophilic leukocytosis, and a dense dermal infiltration of neutrophils without signs of vasculitis observed histologically w1x. A systemic corticosteroid therapy is highly effective w1x. While various malignant neoplasms, inflammatory or autoimmune disorders such as Behc¸et’s disease have been reported to be associated with Sweet’s syndrome w2–4x, the involvement of the central nervous system ŽCNS. has rarely been reported w5–11x. We report here a woman who suffered from acute encephalitis and was subsequently diagnosed as having Sweet’s syndrome. Treatment with corticostroids was successful.
A 47-year-old woman had recurrent episodes of fever and erythematous skin plaques since the age of 30. No diagnosis had been made but corticosteroid therapy was effective. She had experienced systemic fever and skin plaques on her right forearm and lower extremities since June 6, 2000. She was admitted to another hospital on June 17 because of fever and malaise. The peripheral neutrophil cell count was 10,800rml, and the erythrocyte sedimentation rate ŽESR. was 56 mmrh. Cerebrospinal fluid ŽCSF. examination disclosed pleocytosis of 130 cellsrml with 84% neutrophils, with a normal glucose level of 68 mgrdl. After she was diagnosed as having meningitis, treatment with ceftriaxone ŽCTRX. was started. Since she became drowsy despite the treatment, she was transferred to our hospital on June 19. On admission, physical examination revealed a fever of 37.5 8C, and skin lesions on the right forearm and the right ankle. The lesions were sharply demarcated, raised, erythematous, and partially edematous plaques. There were no oral aphthae, genital ulcers, or ophthalmological abnormalities. Pathergy reaction was negative. On neurological examination, she was drowsy
) Corresponding author. Tel.: q81-559-48-3111; fax: q81-559-485088. E-mail address: [email protected] ŽY. Okuma..
0022-510Xr01r$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0 0 2 2 - 5 1 0 X Ž 0 1 . 0 0 5 4 1 - X
96
K. Noda et al.r Journal of the Neurological Sciences 188 (2001) 95–97
and mute. She could not respond even to simple orders. Meningeal signs were absent. Cranial nerves were intact. Muscle strength and tone were normal. Deep tendon reflexes were within normal limits, and no pathological reflexes were observed. Coordination and sensory systems were difficult to evaluate. The white blood cell count was 14,800rml, and the C-reactive protein level was 5.6 mgrdl; ESR was 25 mmrh. Other laboratory tests, including antinuclear antibodies, rheumatoid factor, anti-SS-A, anti-SS-B, anticardiolipin antibodies, and perinuclear antineutrophil cytoplasmic antibodies ŽP-ANCA. gave negative results. Levels of angiotensin converting enzyme ŽACE., thyroid function, and C3 and C4 were normal. A lumbar puncture revealed pleocytosis of 46 cellsrml with a slightly increased protein level of 64 mgrdl and a normal glucose level. Antibodies against herpes simplex virus, Japanese encephalitis virus, and cytomegalovirus were not present. A culture of a CSF sample was also negative for bacteria, tuberculosis and fungi. The DNA of herpes simplex virus was not detected in the CSF. Human leukocyte antigen ŽHLA. typing showed B-54 and CW1, which are particularly frequent in patients with Sweet’s syndrome w13x. A bone marrow aspiration study was not performed. Brain MRI showed increased signal intensity on T2weighted and FLAIR images in the bilateral caudate nucleus and putamen, left corona radiata, and right hippocampus ŽFig. 1.. T1-weighted images were unremarkable and no enhancement effect was observed. MR angiography showed normal appearance. The electroencephalogram showed a slow basic rhythm and frontal dominant delta activity. Since viral encephalitis was suspected on admission, she was treated with 1500 mg of intravenous acyclovir per
day. Antibiotics were also used. There was a slight but fluctuating recovery of consciousness. Subsequently, dermatological consultation disclosed the diagnosis of Sweet’s syndrome. Corticosteroid therapy was then started after she was diagnosed as having neuro-Sweet’s disease w10x. Intravenous administration of methylprednisolone Ž1000 mgr day for 3 days. was followed by 60 mg of oral administration of prednisolone. Erythematous plaques rapidly disappeared without scars. Her consciousness level also improved day by day. A biopsy of the skin lesion revealed a dense infiltration of the neutrophils in the dermis, which is compatible with Sweet’s syndrome. The follow-up CSF test on July 3 showed decreased cell count Ž17rml.. She was discharged from the hospital on July 8 without sequelae.
3. Discussion There has been only a few case reports on the CNS involvement in Sweet’s syndrome w5–11x. Chiba et al. first reported a patient with Sweet’s syndrome who presented with neuropsychiatric symptoms including disorientation, convulsion, myoclonus, and depression. The CT scan revealed brain atrophy, but there was no pleocytosis in the CSF w5x. Furukawa et al. w6x, Dunn et al. w7x and Martinez et al. w8x described patients with meningitis. Druschky et al. w9x reported a patient with meningitis and hemiparesis, whereas MRI failed to show any lesions. There has been only one reported case of Sweet’s syndrome with encephalitis, which involved deep brain structures determined by MRI w10x. The patient, reported by Hisanaga et al. w10x showed at least five episodes of encephalitis with lesions in various subcortical brain structures. These le-
Fig. 1. Magnetic resonance images on admission. FLAIR images show high-intensity lesions in the right hippocampus, bilateral caudate nucleus and putamen.
K. Noda et al.r Journal of the Neurological Sciences 188 (2001) 95–97
sions consisted of T2 high-intensity lesions in the thalamus, putamen, caudate nucleus, midbrain, and cerebral white matter. The authors coined the term Aneuro-Sweet’s disease.B Our patient also showed lesions in the bilateral caudate nucleus and putamen. Therefore, it is suggested that the basal ganglia might be one of the structures preferentially affected in neuro-Sweet’s disease. Further observations with MRI are necessary to confirm this suggestion. Interestingly, the patient reported by Hisanaga et al. also fulfilled the diagnostic criteria of Behc¸et’s disease. Behc¸et’s disease has recently been recognized as one of the underlying disorders of Sweet’s disease w2x. Particularly, about one in seven Japanese patients with Sweet’s syndrome are reported to have features of Behc¸et’s disease in Japan w2x. Other than Japanese, Uysal et al. w12x reported a non-Japanese patient with Sweet’s syndrome having features of neuro-Behc¸et’s disease. The question is whether there are differences in CNS pathology between neuroSweet’s disease and neuro-Behc¸et’s disease. The pathological change in the skin of Sweet’s syndrome is neutrophilic infiltration without vasculitis w1x, whereas one of the pathological features of Behc¸et’s disease is vasculitis. Therefore, the pathology in the CNS might also differ between the two disorders. However, since the neuropathological findings in neuro-Behc¸et’s disease vary considerably and definite vasculitis is not always observed, it is difficult to indicate the differences between the two disorders until the autopsy findings of neuro-Sweet’s disease are reported. Another interesting finding was that our patient showed HLA-B54 and CW1. It is well known that the occurrence of HLA-B54, which is present in almost 50% of the patients with Sweet’s syndrome, is significantly higher in the patients than in a control population w13x. The frequency of HLA-CW1, also present in more than 40% of the patients with Sweet’s syndrome, was not significantly higher w13x. The patient reported by Hisanaga et al. w10x also showed the combination of HLA-B54 and CW1. Whether or not this combination is characteristic of neuroSweet’s disease should be examined by further observation. Thus far, HLA analysis is only helpful in differentiating the systemic form of Sweet’s syndrome from Behc¸et’s disease; the latter is strongly associated with HLA-B51, but not with B54 w2,13x.
97
Sweet’s syndrome itself, regardless of the involvement of the CNS, may be a benign disorder unless other serious diseases such as malignancies are associated with it w4,10x. The involvement of the CNS may become more recognizable if the CSF or the brain image by MRI, or both, are more extensively examined. Awareness of the neurological complication in Sweet’s syndrome is important, because it may help to avoid unnecessary empiric therapy for meningoencephalitis, and will lead to successful treatment with systemic corticosteroids.
References w1x Sweet RD. An acute febrile neutrophillic dermatosis. Br J Dermatol 1964;76:349–56. w2x Mizoguchi M, Chikakane K, Goh K, Asahina Y, Masuda K. Acute febrile neutrophilic dermatosis ŽSweet syndrome. in Behc¸et disease. Br J Dermatol 1987;116:727–34. w3x Kemmett D, Hunter JAA. Sweet syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990;23:503–7. w4x Von den Driesch. Sweet’s syndrome Žacute febrile neutrophilic dermatosis.. J Am Acad Dermatol 1994;31:535–56. w5x Chiba S. Sweet’s syndrome with neurologic signs and psychiatric symptoms. Arch Neurol 1983;40:829. w6x Furukawa F, Toriyama R, Kawanishi T. Neutrophils in cerebrospinal fluid of a patient with acute febrile neutrophilic dermatosis ŽSweet’s syndrome.. Int J Dermatol 1992;31:610–71. w7x Dunn TR, Saperstein HW, Blederman A, Kaplan RP. Sweet syndrome in a neonate with aseptic meningitis. Pediatr Dermatol 1992; 9:288–92. w8x Martinez E, Fernandez A, Mayo J, Manrique P, Collazos J. Sweet’s syndrome associated with cerebrospinal fluid neutrophilic pleocytosis. Int J Dermatol 1995;34:73–4. w9x Druschky A, von den Driesch P, Anders M, Claus D, Neundorfer B. Sweet’s syndrome Žacute febrile neutrophilic dermatosis. affecting the central nervous system. J Neurol 1996;243:556–7. w10x Hisanaga K, Hosokawa M, Sato N, Mochizuki H, Itoyama Y, Iwasaki Y. ANeuro-Sweet diseaseB Benign recurrent encephalitis with neutrophilic dermatosis. Arch Neurol 1999;56:1010–3. w11x Pharis DB, Cerenko D, Caughman SW. Sweet’s syndrome in a patient with idiopathic progressive bilateral sensorineural hearing loss. J Am Acad Dermatol 2000;42:932–5. w12x Uysal H, Vahaboglu H, Inan L, Vahaboglu G. Acute febrile neutrophilic dermatosis ŽSweet’s syndrome. in neuro-Behc¸et disease. Clin Neurol Neurosurg 1993;95:319–22. w13x Mizoguchi M, Matsuki K, Mochizuki M, Watanabe R, Ogawa K, Harada S, et al. Human leukocyte antigen in Sweet’s syndrome and its relationship to Behc¸et’s disease. Arch Dermatol 1988;124:1069– 73.
Short communication
Sweet’s syndrome associated with encephalitis Kazuyuki Noda a , Yasuyuki Okuma a,) , Jiro Fukae a , Kenji Fujishima a , Keigo Goto a , Hiroko Sadamasa b, Takashi Yoshiike b, Yoshikuni Mizuno c a
Department of Neurology, Juntendo Izu-Nagaoka Hospital, 1129 Nagaoka, Izu-Nagaoka, Tagata-gun, Shizuoka 410-2295, Japan b Department of Dermatology, Juntendo UniÕersity Izu-Nagaoka Hospital, Shizuoka, Japan c Department of Neurology, Juntendo UniÕersity School of Medicine, Tokyo, Japan Received 30 October 2000; received in revised form 19 March 2001; accepted 11 May 2001
Abstract The involvement of the central nervous system ŽCNS. in Sweet’s syndrome Žacute febrile neutrophilic dermatosis. is rare. We report a 47-year-old woman who presented with acute encephalitis and was subsequently diagnosed as having Sweet’s syndrome. She developed altered consciousness following fever and erythematous skin plaques in the extremities. Cerebrospinal fluid ŽCSF. examination disclosed neutrophilic pleocytosis without decreased glucose level. Brain magnetic resonance imaging ŽMRI. showed abnormal signal intensity lesions in the basal ganglia and the hippocampus. Skin biopsy revealed a dense dermal infiltration of neutrophils, which is compatible with Sweet’s syndrome. Treatment with acyclovir and antibiotics failed, but the subsequent corticosteroid therapy was effective. Awareness of neurological complication in Sweet’s syndrome may avoid unnecessary empiric therapy for meningoencephalitis and will lead to a successful treatment with corticosteroids. q 2001 Elsevier Science B.V. All rights reserved. Keywords: Sweet’s syndrome; Encephalitis; Basal ganglia; MRI; Central nervous system; Behc¸et’s disease
1. Introduction
2. Case report
Acute febrile neutrophilic dermatosis, or Sweet’s syndrome, is a distinct syndrome originally described by Sweet in 1964 w1x. The four cardinal features are erythematous plaques on the skin, fever, peripheral neutrophilic leukocytosis, and a dense dermal infiltration of neutrophils without signs of vasculitis observed histologically w1x. A systemic corticosteroid therapy is highly effective w1x. While various malignant neoplasms, inflammatory or autoimmune disorders such as Behc¸et’s disease have been reported to be associated with Sweet’s syndrome w2–4x, the involvement of the central nervous system ŽCNS. has rarely been reported w5–11x. We report here a woman who suffered from acute encephalitis and was subsequently diagnosed as having Sweet’s syndrome. Treatment with corticostroids was successful.
A 47-year-old woman had recurrent episodes of fever and erythematous skin plaques since the age of 30. No diagnosis had been made but corticosteroid therapy was effective. She had experienced systemic fever and skin plaques on her right forearm and lower extremities since June 6, 2000. She was admitted to another hospital on June 17 because of fever and malaise. The peripheral neutrophil cell count was 10,800rml, and the erythrocyte sedimentation rate ŽESR. was 56 mmrh. Cerebrospinal fluid ŽCSF. examination disclosed pleocytosis of 130 cellsrml with 84% neutrophils, with a normal glucose level of 68 mgrdl. After she was diagnosed as having meningitis, treatment with ceftriaxone ŽCTRX. was started. Since she became drowsy despite the treatment, she was transferred to our hospital on June 19. On admission, physical examination revealed a fever of 37.5 8C, and skin lesions on the right forearm and the right ankle. The lesions were sharply demarcated, raised, erythematous, and partially edematous plaques. There were no oral aphthae, genital ulcers, or ophthalmological abnormalities. Pathergy reaction was negative. On neurological examination, she was drowsy
) Corresponding author. Tel.: q81-559-48-3111; fax: q81-559-485088. E-mail address: [email protected] ŽY. Okuma..
0022-510Xr01r$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0 0 2 2 - 5 1 0 X Ž 0 1 . 0 0 5 4 1 - X
96
K. Noda et al.r Journal of the Neurological Sciences 188 (2001) 95–97
and mute. She could not respond even to simple orders. Meningeal signs were absent. Cranial nerves were intact. Muscle strength and tone were normal. Deep tendon reflexes were within normal limits, and no pathological reflexes were observed. Coordination and sensory systems were difficult to evaluate. The white blood cell count was 14,800rml, and the C-reactive protein level was 5.6 mgrdl; ESR was 25 mmrh. Other laboratory tests, including antinuclear antibodies, rheumatoid factor, anti-SS-A, anti-SS-B, anticardiolipin antibodies, and perinuclear antineutrophil cytoplasmic antibodies ŽP-ANCA. gave negative results. Levels of angiotensin converting enzyme ŽACE., thyroid function, and C3 and C4 were normal. A lumbar puncture revealed pleocytosis of 46 cellsrml with a slightly increased protein level of 64 mgrdl and a normal glucose level. Antibodies against herpes simplex virus, Japanese encephalitis virus, and cytomegalovirus were not present. A culture of a CSF sample was also negative for bacteria, tuberculosis and fungi. The DNA of herpes simplex virus was not detected in the CSF. Human leukocyte antigen ŽHLA. typing showed B-54 and CW1, which are particularly frequent in patients with Sweet’s syndrome w13x. A bone marrow aspiration study was not performed. Brain MRI showed increased signal intensity on T2weighted and FLAIR images in the bilateral caudate nucleus and putamen, left corona radiata, and right hippocampus ŽFig. 1.. T1-weighted images were unremarkable and no enhancement effect was observed. MR angiography showed normal appearance. The electroencephalogram showed a slow basic rhythm and frontal dominant delta activity. Since viral encephalitis was suspected on admission, she was treated with 1500 mg of intravenous acyclovir per
day. Antibiotics were also used. There was a slight but fluctuating recovery of consciousness. Subsequently, dermatological consultation disclosed the diagnosis of Sweet’s syndrome. Corticosteroid therapy was then started after she was diagnosed as having neuro-Sweet’s disease w10x. Intravenous administration of methylprednisolone Ž1000 mgr day for 3 days. was followed by 60 mg of oral administration of prednisolone. Erythematous plaques rapidly disappeared without scars. Her consciousness level also improved day by day. A biopsy of the skin lesion revealed a dense infiltration of the neutrophils in the dermis, which is compatible with Sweet’s syndrome. The follow-up CSF test on July 3 showed decreased cell count Ž17rml.. She was discharged from the hospital on July 8 without sequelae.
3. Discussion There has been only a few case reports on the CNS involvement in Sweet’s syndrome w5–11x. Chiba et al. first reported a patient with Sweet’s syndrome who presented with neuropsychiatric symptoms including disorientation, convulsion, myoclonus, and depression. The CT scan revealed brain atrophy, but there was no pleocytosis in the CSF w5x. Furukawa et al. w6x, Dunn et al. w7x and Martinez et al. w8x described patients with meningitis. Druschky et al. w9x reported a patient with meningitis and hemiparesis, whereas MRI failed to show any lesions. There has been only one reported case of Sweet’s syndrome with encephalitis, which involved deep brain structures determined by MRI w10x. The patient, reported by Hisanaga et al. w10x showed at least five episodes of encephalitis with lesions in various subcortical brain structures. These le-
Fig. 1. Magnetic resonance images on admission. FLAIR images show high-intensity lesions in the right hippocampus, bilateral caudate nucleus and putamen.
K. Noda et al.r Journal of the Neurological Sciences 188 (2001) 95–97
sions consisted of T2 high-intensity lesions in the thalamus, putamen, caudate nucleus, midbrain, and cerebral white matter. The authors coined the term Aneuro-Sweet’s disease.B Our patient also showed lesions in the bilateral caudate nucleus and putamen. Therefore, it is suggested that the basal ganglia might be one of the structures preferentially affected in neuro-Sweet’s disease. Further observations with MRI are necessary to confirm this suggestion. Interestingly, the patient reported by Hisanaga et al. also fulfilled the diagnostic criteria of Behc¸et’s disease. Behc¸et’s disease has recently been recognized as one of the underlying disorders of Sweet’s disease w2x. Particularly, about one in seven Japanese patients with Sweet’s syndrome are reported to have features of Behc¸et’s disease in Japan w2x. Other than Japanese, Uysal et al. w12x reported a non-Japanese patient with Sweet’s syndrome having features of neuro-Behc¸et’s disease. The question is whether there are differences in CNS pathology between neuroSweet’s disease and neuro-Behc¸et’s disease. The pathological change in the skin of Sweet’s syndrome is neutrophilic infiltration without vasculitis w1x, whereas one of the pathological features of Behc¸et’s disease is vasculitis. Therefore, the pathology in the CNS might also differ between the two disorders. However, since the neuropathological findings in neuro-Behc¸et’s disease vary considerably and definite vasculitis is not always observed, it is difficult to indicate the differences between the two disorders until the autopsy findings of neuro-Sweet’s disease are reported. Another interesting finding was that our patient showed HLA-B54 and CW1. It is well known that the occurrence of HLA-B54, which is present in almost 50% of the patients with Sweet’s syndrome, is significantly higher in the patients than in a control population w13x. The frequency of HLA-CW1, also present in more than 40% of the patients with Sweet’s syndrome, was not significantly higher w13x. The patient reported by Hisanaga et al. w10x also showed the combination of HLA-B54 and CW1. Whether or not this combination is characteristic of neuroSweet’s disease should be examined by further observation. Thus far, HLA analysis is only helpful in differentiating the systemic form of Sweet’s syndrome from Behc¸et’s disease; the latter is strongly associated with HLA-B51, but not with B54 w2,13x.
97
Sweet’s syndrome itself, regardless of the involvement of the CNS, may be a benign disorder unless other serious diseases such as malignancies are associated with it w4,10x. The involvement of the CNS may become more recognizable if the CSF or the brain image by MRI, or both, are more extensively examined. Awareness of the neurological complication in Sweet’s syndrome is important, because it may help to avoid unnecessary empiric therapy for meningoencephalitis, and will lead to successful treatment with systemic corticosteroids.
References w1x Sweet RD. An acute febrile neutrophillic dermatosis. Br J Dermatol 1964;76:349–56. w2x Mizoguchi M, Chikakane K, Goh K, Asahina Y, Masuda K. Acute febrile neutrophilic dermatosis ŽSweet syndrome. in Behc¸et disease. Br J Dermatol 1987;116:727–34. w3x Kemmett D, Hunter JAA. Sweet syndrome: a clinicopathologic review of twenty-nine cases. J Am Acad Dermatol 1990;23:503–7. w4x Von den Driesch. Sweet’s syndrome Žacute febrile neutrophilic dermatosis.. J Am Acad Dermatol 1994;31:535–56. w5x Chiba S. Sweet’s syndrome with neurologic signs and psychiatric symptoms. Arch Neurol 1983;40:829. w6x Furukawa F, Toriyama R, Kawanishi T. Neutrophils in cerebrospinal fluid of a patient with acute febrile neutrophilic dermatosis ŽSweet’s syndrome.. Int J Dermatol 1992;31:610–71. w7x Dunn TR, Saperstein HW, Blederman A, Kaplan RP. Sweet syndrome in a neonate with aseptic meningitis. Pediatr Dermatol 1992; 9:288–92. w8x Martinez E, Fernandez A, Mayo J, Manrique P, Collazos J. Sweet’s syndrome associated with cerebrospinal fluid neutrophilic pleocytosis. Int J Dermatol 1995;34:73–4. w9x Druschky A, von den Driesch P, Anders M, Claus D, Neundorfer B. Sweet’s syndrome Žacute febrile neutrophilic dermatosis. affecting the central nervous system. J Neurol 1996;243:556–7. w10x Hisanaga K, Hosokawa M, Sato N, Mochizuki H, Itoyama Y, Iwasaki Y. ANeuro-Sweet diseaseB Benign recurrent encephalitis with neutrophilic dermatosis. Arch Neurol 1999;56:1010–3. w11x Pharis DB, Cerenko D, Caughman SW. Sweet’s syndrome in a patient with idiopathic progressive bilateral sensorineural hearing loss. J Am Acad Dermatol 2000;42:932–5. w12x Uysal H, Vahaboglu H, Inan L, Vahaboglu G. Acute febrile neutrophilic dermatosis ŽSweet’s syndrome. in neuro-Behc¸et disease. Clin Neurol Neurosurg 1993;95:319–22. w13x Mizoguchi M, Matsuki K, Mochizuki M, Watanabe R, Ogawa K, Harada S, et al. Human leukocyte antigen in Sweet’s syndrome and its relationship to Behc¸et’s disease. Arch Dermatol 1988;124:1069– 73.