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5,270 results
  • Anti-GAD epileptic encephalopathy in a toddler with Parry-Romberg syndrome. [Journal Article]
    Neurol Sci 2019Sotgiu S, Consolaro A, … Picco P
  • Parry-Romberg syndrome (PRS) is a progressive facial hemiatrophy often associated with severe epilepsy. Although an immune-mediated vasculitic pathogenesis is widely assumed, no CNS-specific autoantibody has been described so far. A 2-year-old boy was admitted for a status epilepticus preceded by fever, restlessness, insomnia, and left facial rash. Cerebrospinal fluid was positive for glutamic ac…
  • Characteristics of coexisting localized scleroderma and inflammatory arthritis. [Journal Article]
    Eur J Rheumatol 2019; :1-5Reiff D, Crayne CB, … Cron RQ
  • CONCLUSIONS: In this cohort of pediatric LS, 9% of patients had coexisting inflammatory arthritis. The characteristics of this cohort varied widely. All patients received MTX initially and showed a resolution of LS lesions. However, in the majority of patients, the arthritis failed to respond to MTX and TNFi combination therapy. These results suggest that inflammatory arthritis coexisting with LS may be less likely to respond to traditional inflammatory arthritis or JIA therapies.
  • Linear Morphea With Inflammatory Myositis. [Journal Article]
    J Hand Surg Am 2019Morris MS, Matcuk G, … Stevanovic M
  • Morphea is an autoimmune disorder characterized by sclerosis and inflammation of the skin and soft tissues. Early diagnosis and treatment are essential to minimize morbidity such as joint contracture. In this report, we present the case of a 19-year-old man with linear morphea with inflammatory myositis who presented to our clinic 1 year after symptom onset with severe elbow flexion contracture. …
  • Linear Patch of Alopecia in a Child: Trichoscopy Reveals the Actual Diagnosis. [Case Reports]
    Skin Appendage Disord 2019; 5(6):409-412Sonthalia S, Agrawal M, … Goldust M
  • Linear scleroderma en coup de sabre (LSCS) is a clinical variant of morphea that presents with a linear alopecic patch over the frontal scalp. Linear alopecia areata may present as a close differential in the incipient stages of LSCS in the pediatric population, especially when the binding down and pigmentary changes characteristic of LSCS have not developed. Histopathology may also be noncontrib…
  • Overview of Juvenile localized scleroderma and its management. [Review]
    World J Pediatr 2019Li SC, Zheng RJ
  • CONCLUSIONS: JLS is associated with a risk for disabling and disfiguring morbidity for the growing child. Identifying active disease is important for guiding treatment, but often difficult because of the paucity of markers and lack of a universal skin activity feature. More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets. Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.
  • A case of multiple cerebral cavernomas associated with en coup de sabre scleroderma. [Journal Article]
    Pediatr Dermatol 2019Alwash N, Dinani N, Felton J
  • Linear scleroderma is a form of localized scleroderma that presents as band-like sclerotic lesions. When it occurs on the scalp or forehead, it is called "en coup de sabre scleroderma" (ECDS). Extracutaneous associations, mostly neurologic, have been described with ECDS. We report a patient with ECDS associated with multiple cerebral cavernomas. This serves to highlight the importance of neuroima…
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