Presentation
Clinical features of different stages of the disease are presented. [ncbi.nlm.nih.gov]
Szewka First published October 17, 2016, DOI: A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting “stuck” with upward gaze. [n.neurology.org]
There is no test specific for confirmation of a diagnosis of scleroderma, but x-rays, skin biopsies, and tests for antinuclear antibodies, gamma globulin, sedimentation rate, and latex fixation can provide evidence that the disease is present. [medical-dictionary.thefreedictionary.com]
An antibody test may help distinguish the type of scleroderma present. In addition to a complete medical history and physical examination, diagnostic procedures for scleroderma may include: Electrocardiogram (EKG or ECG). [childrensnational.org]
Entire Body System
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Asymptomatic
Asymptomatic T2 hyperintensities were noted on MRI ( figure 2 ). Scalp morphea was confirmed pathologically. Formal rheumatologic evaluation was otherwise unremarkable. [n.neurology.org]
The lesion was asymptomatic and not preceded by trauma. Over the succeeding years, the sclerotic changes and atrophy gradually spread over the almost complete left frontoparietal area. [ncbi.nlm.nih.gov]
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Lymphadenopathy
Nummular Headache Associated With Linear Scleroderma. ( 27477768 ) Camacho-Velasquez JL 2016 20 Treatment of Linear Scleroderma (en Coup de Sabre) With Dermal Fat Grafting. ( 26700540 ) Barin EZ...Tan O 2016 21 Linear scleroderma with prominent multiple lymphadenopathy [malacards.org]
Jaw & Teeth
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Difficulty Opening the Mouth
Patients with scleroderma have great difficulty opening their mouths wide enough to allow dental work to be done. [medical-dictionary.thefreedictionary.com]
Eyes
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Enophthalmos
(C) Axial T2-weighted MRI orbits reveals right enophthalmos. Footnotes Author contributions: Lauren E. Hock: analysis and interpretation of data, drafting and revising the manuscript. [n.neurology.org]
( 29445726 ) Khamaganova I 2017 18 Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. ( 27754909 ) Hock LE...Szewka AJ 2016 19 Nummular Headache Associated With Linear Scleroderma. ( 27477768 ) Camacho-Velasquez [malacards.org]
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Diplopia
Szewka First published October 17, 2016, DOI: A 28-year-old woman with alopecia since childhood presented with 4 years of progressive intermittent diplopia and right eye getting “stuck” with upward gaze. [n.neurology.org]
( 29445726 ) Khamaganova I 2017 18 Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. ( 27754909 ) Hock LE...Szewka AJ 2016 19 Nummular Headache Associated With Linear Scleroderma. ( 27477768 ) Camacho-Velasquez [malacards.org]
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Blepharoptosis
[…] rk P 2012 46 A case of linear scleroderma involving cerebellum with vertigo. ( 24653878 ) Choi EJ...Lee SH 2012 47 Acquired pediatric blepharoptosis related to linear scleroderma. ( 20055335 ) Kao TY...McCulley TJ 2011 48 Digital image processing for [malacards.org]
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Lacrimation
Experience with Microsurgical Reconstruction of Hemifacial Atrophy and Linear Scleroderma. ( 30511981 ) Chen JT...Siebert JW 2018 13 Adie Pupil as the Initial Presentation of Localized En Coup de Sabre Scleroderma. ( 28668815 ) Laxer RM...Pope E 2017 14 Lacrimal [malacards.org]
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Lagophthalmos
(B) Right enopthalmos due to suspected orbital fat fibrosis; also inferior lagophthalmos. Figure 2 Brain and orbital MRI (A) Coronal T2-weighted MRI demonstrates nonenhancing hyperintensities in the right frontal lobe and (B) right thalamus. [n.neurology.org]
Musculoskeletal
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Fracture
[…] of Localized En Coup de Sabre Scleroderma. ( 28668815 ) Laxer RM...Pope E 2017 14 Lacrimal Drainage System Involvement in Linear Scleroderma. ( 27930422 ) Singh S...Naik MN 2017 15 A case of systemic sclerosis with linear scleroderma developing after fracture [malacards.org]
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Muscle Spasm
[…] anti-Interleukin-6 therapy. ( 31096996 ) Magro CM...Shapiro L 2019 5 Cone beam computed tomography for the assessment of linear scleroderma of the face. ( 29298697 ) Di Giovanni C...Zulian F 2018 6 Characterization and Treatment of Unilateral Facial Muscle [malacards.org]
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Morning Stiffness
Symptoms of systemic sclerosis may include: Thickening and swelling of the tips of the fingers Pale and tingly fingers that may become numb when exposed to cold or when emotionally upset (called Raynaud phenomenon) Joint pain Morning stiffness Taut, shiny [childrensnational.org]
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Myopathy
Besides, non-neurogenic myopathy associated with LSCS has previously been reported [ 11, 12 ]. However, cranial nerve palsy coexisting with LSCS or PRS is a rarity. [ncbi.nlm.nih.gov]
Neurologic
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Vertigo
[…] rk P 2012 46 A case of linear scleroderma involving cerebellum with vertigo. ( 24653878 ) Choi EJ...Lee SH 2012 47 Acquired pediatric blepharoptosis related to linear scleroderma. ( 20055335 ) Kao TY...McCulley TJ 2011 48 Digital image processing for [malacards.org]
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Irritability
The skin should be protected from irritation that could lead to breakdown and the introduction of infectious agents. Calcium deposit sites are assessed regularly to note any signs of prolonged pressure, ulceration, or infection. [medical-dictionary.thefreedictionary.com]
Treatment
[…] r I 2015 26 Delay in Diagnosis of Congenital Linear Scleroderma until Adulthood. ( 25775624 ) Lipson J...Kapur S 2015 27 Autologous fat transplantation for the treatment of linear scleroderma en coup de sabre. ( 25842477 ) Ibler KS...Siemssen PA 2015 [malacards.org]
Treatment for scleroderma Specific treatment for scleroderma will be determined by your child's doctor based on: Your child's overall health and medical history Extent of the condition Your child's tolerance for specific medications, procedures, and therapies [childrensnational.org]
Treatment. Treatment includes drugs such as penicillamine, immunosuppressives, and antiinflammatory agents. Ulcers that form on the knuckles, elbows, and other bony prominences as a result of calcium deposits are treated topically with ointments. [medical-dictionary.thefreedictionary.com]
Combined treatment with calcipotriol ointment and low-dose ultraviolet A1 phototherapy in childhood morphea. [ncbi.nlm.nih.gov]
Generally methotrexate is the treatment of choice, however there are many other drugs that have been used in the treatment of this condition. Methotrexate is combined with corticosteroids as an adjunct during the first 2 or 3 months of therapy. [healthengine.com.au]
Prognosis
Statistics Risk Factors Progression Symptoms Clinical Examination How is it Diagnosed Prognosis Treatment References What is Localized Scleroderma? Scleroderma is a disorder with an unknown cause. [healthengine.com.au]
Etiology
Lateralization of LSCS possibly indicates a neuropathological etiology of the disease. [ncbi.nlm.nih.gov]
Epidemiology
"Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study". Rheumatology (Oxford). 45 (5): 614–20. doi : 10.1093/rheumatology/kei251. PMID 16368732. [en.wikipedia.org]
Prevention
Mouth care and proper brushing and flossing, as well as periodic dental checks, are necessary to prevent cavities and periodontal disease. [medical-dictionary.thefreedictionary.com]
Physical therapy helps to maintain the health of the joint by preventing complications such as joint contractures and deformities, while also helping to improve mobility of the joint and strength of the surrounding muscles. [healthengine.com.au]