Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

Gambichler, Thilo; Kreuter, Alexander; Hoffmann, Klaus; Bechara, Falk G.; Altmeyer, Peter; Jansen, Thomas

doi:10.1186/1471-5945-1-9

Cited by 63 publications

(68 citation statements)

References 15 publications

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“…Seizures and headache were the most frequent conditions, and this finding is consistent with the data already reported in the literature (15)(16)(17)(18)(19)(20)(21)(22). Behavioral changes and learning disabilities have also been reported (23,24), while conditions such as stroke, transverse myelitis, and hemiparesis, which have been reported in adults (23,(25)(26)(27)(28), were not present.…”

Section: Discussionsupporting

confidence: 90%

“…In our series, intraparenchymal abnormalities on MRI, vascular malformations, or CNS vasculitis represented one-third of these neurologic manifestations. Although neurologic manifestations have been previously described in case reports or case series (16)(17)(18)(19)(20)(21)(22)(23)(28)(29)(30)(31), their prevalence has not been reported in a large cohort of patients and particularly in children. Indeed, because neuroradiologic examinations were performed only in symptomatic patients, it is conceivable that these changes would be observed even more frequently if all patients were screened systematically.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Localized scleroderma in childhood is not just a skin disease

Zulian

Vallongo

Woo

et al. 2005

Arthritis & Rheumatism

331

268

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Objective. Juvenile localized scleroderma is usually considered a disease that is confined to the skin and subcutaneous tissue. We studied the prevalence and clinical features of extracutaneous manifestations in a large cohort of children with juvenile localized scleroderma.Methods. Data from a multinational study on juvenile scleroderma was used for this in-depth study. Clinical features of patients with extracutaneous manifestations were compared with those of patients who had exclusively skin involvement.Results. Seven hundred fifty patients entered the study. One hundred sixty-eight patients (22.4%) presented with a total of 193 extracutaneous manifestations, as follows: articular (47.2%), neurologic (17.1%), vascular (9.3%), ocular (8.3%), gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%), and renal (1%). Other autoimmune conditions were present in 7.3% of patients. Neurologic involvement consisted of epilepsy, central nervous system vasculitis, peripheral neuropathy, vascular malformations, headache, and neuroimaging abnormalities. Ocular manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papilledema. In more than one-fourth of these children, articular, neurologic, and ocular involvements were unrelated to the site of skin lesions. Raynaud's phenomenon was reported in 16 patients. Respiratory involvement consisted essentially of restrictive lung disease. Gastrointestinal involvement was reported in 12 patients and consisted exclusively of gastroesophageal reflux. Thirty patients (4%) had multiple extracutaneous features, but systemic sclerosis (SSc) developed in only 1 patient. In patients with extracutaneous involvement, the prevalence of antinuclear antibodies and rheumatoid factor was significantly higher than that among patients with only skin involvement. However, Scl-70 and anticentromere, markers of SSc, were not significantly increased.Conclusion. Extracutaneous manifestations of juvenile localized scleroderma developed in almost onefourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Localized scleroderma in childhood is not just a skin disease

Zulian

Vallongo

Woo

et al. 2005

Arthritis & Rheumatism

331

268

View full text Add to dashboard Cite

show abstract

“…Several reports of intracerebral lesions such as infl ammation, calcifi cation and demyelination were shown on MRI and CT in patients with localised scleroderma leading to epilepsy and functional motor impairments. 7,8 Progressive hemifacial atrophy (ParryRomberg Syndrome) associated with localised scleroderma has also been reported, 2,9,10 but the relationship to these two conditions remains controversial. In most cases the term Parry-Romberg Syndrome should be used for progressive hemifacial atrophy alone.…”

Section: Discussionmentioning

confidence: 99%

“…In such cases localised scleroderma tends to be a preceding condition to progressive hemifacial atrophy which then develops several years later. 2 There is no standard modality of treatment for en coup de sabre lesions. Many drugs have been used, most with unsatisfactory results.…”

Section: Discussionmentioning

confidence: 99%

A rare case of frontal linear scleroderma (en coup de sabre) with intra-oral and dental involvement

2010

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“…'En coup de sabre' is an uncommon and seldom reported dermatological disorder; it is usually unilateral, but rare bilateral cases have been recorded. 7 It is often reported with ptosis or lid abnormalities [4][5][6][7][8][9][11][12][13][14][15][16] but concurrent oculomotor disorders are rare. 4,7,9,11,[13][14][15][16] Case report…”

Section: Introductionmentioning

confidence: 99%

Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature

Taylor¹,

Riley²,

Bowyer

2010

British and Irish Orthoptic Journal

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Aim: To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method: Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results: The main features of 'en coup de sabre' subsequently manifested on the left side of the face and head. An unusual feature was the characteristic subcutaneous cleft which also appeared on the opposite side above the right eyebrow. The left eye went on to develop a corneal ulcer with resulting poor visual acuity. An ipsilateral oculomotility disorder was then noted in addition to poor eyelid closure. Conclusion: This case highlights the need to exclude 'en coup de sabre' as a potential differential diagnosis in any case of progressive or recurrent ptosis, especially in childhood. As oculomotility may also be affected ultimately, it could lead to corneal compromise following ptosis correction.

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Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

Cited by 63 publications

References 15 publications

Localized scleroderma in childhood is not just a skin disease

Localized scleroderma in childhood is not just a skin disease

A rare case of frontal linear scleroderma (en coup de sabre) with intra-oral and dental involvement

Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature

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