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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 1  |  Page : 78-80

Central nervous system vasculitis associated with linear scleroderma

Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan Xili, Chongwen District, Beijing 100050, China

Date of Submission12-Nov-2012
Date of Decision13-Nov-2012
Date of Acceptance08-Dec-2012
Date of Web Publication4-Mar-2013

Correspondence Address:
Zai-qiang Zhang
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan Xili, Chongwen District, Beijing 100050
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.108019

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How to cite this article:
Zhang Zq, Tian D. Central nervous system vasculitis associated with linear scleroderma . Neurol India 2013;61:78-80

How to cite this URL:
Zhang Zq, Tian D. Central nervous system vasculitis associated with linear scleroderma . Neurol India [serial online] 2013 [cited 2023 Feb 6];61:78-80. Available from: https://www.neurologyindia.com/text.asp?2013/61/1/78/108019


Linear scleroderma (LScs) represents a unique form of localized scleroderma and occurs primarily in pediatric population. [1] Neurological complications have been described with linear scleroderma en coup de sabre. However, pathological information regarding the nature of these lesions is in dispute. In this report, we describe the neuropathological features in two patients with LScs.

 » Case 1 Top

A 30-year-old female, a known case of LScs since age 6, developed diplopia and generalized seizures during fourth-month pregnancy and felt left- sided numbness and weakness. Neurological examination showed the impaired memory, decreased light response on the right, decreased sensation and weakness on the left side with up going plantar response. Skin examination revealed a linear, indurated plaque with alopecia of right frontal-parietal scalp. Antimitochondrial antibody and SCL-70 were positive. Skull X-ray showed right frontal bone hypodensity. Magnetic resonance imaging (MRI) showed right thalamus, right parieto-occiptal lobe, and paraventricular hyperintese signals on T2-weighted sequence and also right hemisphere atrophy [Figure 1]a and b and intense contrast enhancement of right thalamic lesion [Figure 1]c. Scleroderma "en coup de sabre" was seen with focal thinning of the scalp, and atrophy of the cranial bone and underlying cerebral hemisphere [Figure 1]d. MR-angiography showed paucity of right middle cerebral artery. Scalp biopsy showed collagen deposition and inflammatory cells infiltration. Pathology of brain biopsy showed reactive vessels proliferation, thickened vascular wall, perivascular inflammatory cells infiltrating in the subcortical tissue, multiple small infarcts, basophilia mineral deposition in white matter, obliterated vessels, and reactive astrocyte hyperplasia around the infarction. The astrocyte expressed glial fibrillary acidic protein, and perivascular lymphocyte expressed CD45RO [Figure 2]a-d.
Figure 1: (a) Axial T2 weighted image showing right cerebral peduncle atrophy and abnormal signals at the right temporal lobe. (b) Axial T2 weighted image revealing hyperintensity at right thalamus. (c) Axial gadolinium enhanced T1 weighted image demonstrating the right thalamic lesion, with marked enhancement. (d) Scleroderma "en coup de sabre" is seen with focal atrophy of the the scalp, cranial bone and the underlying hemisphere

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Figure 2: (a) Small vessels show perivascular inflammatory cells infiltrate with thickened vascular wall (H and E, × 200) (b) Micro infarcts, some basophilia mineral deposition in the white matter (H and E, × 400) (c) Perivascular lymphocyte stained with CD45RO. (CD45RO immunostaining, × 400) (d) Astrocyte around the infarct lesion - glial fibrillary acidic protein staining (GFAP immunostaining, × 400)

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 » Case 2 Top

A 27-year-old women, a known case of LScs since age 5, presented with 3- month history of recurrent right forehead and temple headaches and episodic vomiting. A regional sclerotic skin lesion (3 × 8 cm) with hair loss extending from right forehead to parietal scalp was noted. The SCL-70 was positive. Cranial MRI revealed a cyst-like lesion with white matter demyelination in right temporo-occipital lobe and basal ganglia, and patchy enhancement of the cyst and the adjacent leptomeninges [Figure 3]a-d. Biopsy was performed keeping glioma as a possibility. Pathology showed vessels with marked inflammatory cell infiltration with adjacent hemosiderin deposition, reactive astrocyte hyperplasia around the areas of necrosis, white matter demyelination, and reactive astrocyte proliferation [Figure 4]a-f.
Figure 3: (a) Axial T1-weighted image showing a cyst-like lesion in tempo-occipital lobe and basal ganglia (b, c) Axial T2- weighted image demonstrating hyperintense cystic lesion in right tempo-occipital lobe and basal ganglia (d) Coronal gadolinium enhanced T1-weighted image showing patchy enhancement of the lesion and adjacent leptomeninges

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Figure 4: (a) A micro vessel showing marked inflamed cells infiltrate (H and E, × 200) (b, c) Hemosiderin deposition evident adjacent to some of the inflamed vessels (H and E: b × 400, c × 200) (d) Reactive astrocyte hyperplasia around the necrotic area. (H and E, × 400) (e) White matter demyelination (FAST-BLUE staining, × 200) (f) Reactive astrocyte expressing glial fibrillary acidic protein (GFAP immunostaining, × 400)

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Neurological complications are the most common complications of LScs and these include complex partial seizures, focal deficits, trigeminal neuralgia, cerebral aneurysm, encephalopathy, cognitive disturbances, subdural hygroma, facial nerve palsy, oculomotor nerve palsy and migraine. [1],[2] Both the patients had linear, indurated plaque with alopecia in right fronto-pariatal scalp in adolescence and the clinical manifestations and immunological test supported the diagnosis of LScs. Both the patients had ipsilateral hemispheric atrophy, white matter demyelination, hippocampal atrophy, and paucity of cerebral vessels similar to the findings reported previously. [2],[3],[4],[5],[6] Second patient presented with features consistent with intracranial space-occupying lesion. Mechanism by which the ipsilateral cerebral hemisphere involves remains unclear. One theory proposes that LScs with cerebral involvement may represent a neurocutaneous syndrome of vascular dysgenesis. Chung et al. [7] reported a patient with LScs in whom radiographic studies demonstrated a focal parenchymal lesion in the left frontal lobe just below the scleroderma lesion on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels, but no evidence of inflammation or vascular wall fibrinoid necrosis. They postulated that LScs represents a developmental disorder rather than localized forms of autoimmune disease. In our first patient, the paucity of ipsilateral middle cerebral artery branches and ipsilateral cerebral atrophy probably suggests vascular dysplasia. An alternative hypothesis suggests intracerebral vaculitis as the pathological basis for neurological complications in LScs. Stone et al. [6] described a patient with LScs. Brain pathology showed a focal inflammatory process centered around blood vessels with infiltration and multiple focal cerebral infarcs. Astrocytic reaction was evident. Dubeau et al. [8] reported pathological evidence for gliosis, neuronal loss and perivascular inflammatory cells infiltration. In our two patients, the pathology showed classical vasculitis. In LScs brain involvement is ipsilateral to the skin lesion. Vasculitis, probably, is an important mechanism in the disease pathogenesis of LScs and vascular displasia or neurocutaneus hypothesis may also contribute in the pathogenesis.

 » References Top

1.Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics 2006:117:e132-6.  Back to cited text no. 1
2.Appenzeller S, Montenegro MA, Dertkigil SS, Sampaio-Barros PD, Marques-Neto JF, Samara AM, et al. Neuroimaging findings in scleroderma en coup de sabre. Neurology 2004;62:1585-9.  Back to cited text no. 2
3.Grosso S, Fioravanti A, Biasi G, Conversano E, Marcolongo R, Morgese G, et al. Linear scleroderma associated with progressive brain atrophy. Brain Dev 2003;25:57-61.  Back to cited text no. 3
4.Kanzato N, Matsuzaki T, Komine Y, Saito M, Saito A, Yoshio T, et al. Localized scleroderma associated with progressing ischemic stroke. J Neurol Sci 1999;163:86-9.  Back to cited text no. 4
5.Verhelst HE, Beele H, Joos R, Vanneuville B, Van Coster RN. Hippocampal atrophy and developmental regression as first sign of linear scleroderma "en coup de sabre". Eur J Paediatr Neurol 2008;12:508-11.  Back to cited text no. 5
6.Stone J, Franks AJ, Guthrie JA, Johnson MH. Scleroderma ''en coup de sabre'': Pathological evidence of intracerebral inflammation. J Neurol Neurosurg Psychiatry 2001;70:382-5.  Back to cited text no. 6
7.Chung MH, Sum J, Morrell MJ, Horoupian DS. Intracerebral involvement in scleroderma en coup de sabre: Report of a case with neurolopathologic findings. Ann Neurol 1995;37:679-81.  Back to cited text no. 7
8.Dubeau F, Andermann F, Robitaille Y. Morphoea or focal scleroderma of the brain: Intractable epilepsy and clinicopathologic correlation. Epilepsia 1988;29:712-3.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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