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Linear Scleroderma

Frontal Linear Scleroderma en Coup de Sabre


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]
  • 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]
  • 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]
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]
Falling
  • Up to 15% of morphea patients may fall into this previously unrecognized category. [4] Cause [ edit ] Physicians and scientists do not know what causes morphea.[en.wikipedia.org]
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]
Ptosis
  • She had complete ptosis of the left eye. Veins was clearly seen through the atrophic skin in the left temporal region.[ncbi.nlm.nih.gov]
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]
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]
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]
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]
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]
Localized Scleroderma
  • The two main types are systemic scleroderma and localized scleroderma. The milder forms are most often seen in women between the ages of 30 and 50; the more severe forms usually affect men, blacks, and older persons.[medical-dictionary.thefreedictionary.com]
  • Table 1 Variants of localized scleroderma recently classified by Tuffanelli [2] I.[ncbi.nlm.nih.gov]
  • There are two forms of scleroderma: localized scleroderma and systemic sclerosis. Localized scleroderma can be seen more frequently in children than the systemic form. It may involve patches of the skin on the trunk, arms, legs, or head.[childrensnational.org]
  • […] patients body that can be detected in patients with localized scleroderma.[healthengine.com.au]
  • "The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993". J. Rheumatol. 24 (1): 73–80. PMID 9002014. Further reading [ edit ] JAMA Dermatology Patient Page. Morphea (Localized Scleroderma. Nicole M. Fett, MD.[en.wikipedia.org]
Skin Discoloration
  • Discolored (lighter or darker) skin Joint tightness Symptoms of scleroderma may resemble other medical conditions or problems.[childrensnational.org]
  • LSCS, usually developing in the first or second decade of life, presents as band-like sclerotic lesions with more or less marked skin discoloration of the frontoparietal area.[ncbi.nlm.nih.gov]
Indurated Skin Lesion
  • Abstract Background Linear scleroderma "en coup de sabre" (LSCS) usually affects one side of the face and head in the frontoparietal area with band-like indurated skin lesions. The disease may be associated with facial hemiatrophy.[ncbi.nlm.nih.gov]

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]
Sex distribution
Age distribution

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]

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