The ‘Connective Tissue Diseases’

Goodfield, M. J. D.; Jones, Stephen; Veale, Douglas J.

doi:10.1002/9781444317633.ch51

Cited by 62 publications

(80 citation statements)

References 2,157 publications

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“…Systemic Sclerosis is a multi system, multistage connective tissue disease characterized by vasculopathy, fibrosis and degenerative changes in the skin and internal organs and production of autoantibodies [1]. This disease may involve one or many internal organs including heart,lungs,gastrointestinal tract.…”

Section: Introductionmentioning

confidence: 99%

“…The main highlight of these criteria was that it differentiated the two main subsets of Systemic Sclerosis: Diffuse form of SSc (dcSSc) and Limited Cutaneous form of SSc (lcSSc.). The LeRoy classification is shown in Table 1 [6]. The main advantage of this classification is its ease of use in everyday practice and wide acceptance [7].…”

Section: Introductionmentioning

confidence: 99%

“…A couple of years, after this attempt by Barnett, the first standard classification criteria for Systemic Sclerosis were developed by the American College of Rheumatology (ACR) in 1980 [6]. The ACR criteria have the advantage of being well researched and validated in a large population of patients along with 92% sensitivity and 96% specificity [7].…”

Section: Introductionmentioning

confidence: 99%

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Classification criteria of systemic sclerosis: Journey so far

Bains¹

2017

Our Dermatol Online

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Classification criteria of systemic sclerosis: Journey so far

Bains¹

2017

Our Dermatol Online

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“…Not infrequently there is atrophy of the corresponding part of the face and cheek, with facial asymmetry which usually occurs within a year. 1 This condition may also be associated with atrophy of one side of the face. 'En coup de sabre' is an uncommon and seldom reported dermatological disorder; it is usually unilateral, but rare bilateral cases have been recorded.…”

Section: Introductionmentioning

confidence: 99%

“…An ivory irregular sclerotic plaque can subsequently develop, sometimes with telangiectatic vessels coursing over it together with hyperpigmentation at the edge. 1 Scleroderma 'en coup de sabre' is a term used to describe a lesion of LS which is specifically localised to the frontal parietal section of the face and scalp. A linear depressed groove appears on the frontoparietal region extending into the scalp producing a linear zone of alopecia, which may be preceded by a bleaching of the hair.…”

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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Efalizumab in the Treatment of Discoid Lupus Erythematosus

Usmani

Goodfield²

2007

Arch Dermatol

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Background: Discoid lupus erythematosus is a chronic inflammatory condition in which the pathogenesis and the role of cell-mediated immunity remains unclear. Currently, the most effective treatments for severe disease are thalidomide, methotrexate, and cyclosporin, although the evidence for this is limited. Efalizumab is a monoclonal antibody directed against CD11a, the ␣-subunit of the leukocyte-functioning antigen 1, with a current license for use in psoriasis. Because discoid lupus erythematosus is known to be predominantly T-cell mediated, our aim was to use efalizumab as a T-cell modulator in patients with recalcitrant disease. Observations: Thirteen patients received efalizumab, with treatment responses varying from good to excellent in 12 of 13 patients. There was a significant reduction in the cutaneous lupus activity and severity score (CLASS) score after therapy with efalizumab (P=.002). Conclusions: We have presented efalizumab as a novel alternative treatment for patients with difficult discoid lupus erythematosus. The response to treatment in 12 patients was very encouraging, with the mean time to response being 5.5 weeks. However, patient numbers were small, and many remain in the early stages of therapy. A prospective randomized study with a long-term follow-up is required, especially in light of recent findings to evaluate both the effectiveness and safety profile of this monoclonal antibody.

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The ‘Connective Tissue Diseases’

Cited by 62 publications

References 2,157 publications

Classification criteria of systemic sclerosis: Journey so far

Classification criteria of systemic sclerosis: Journey so far

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

Efalizumab in the Treatment of Discoid Lupus Erythematosus

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