We have evaluated the human leukocyte antigen (HLA) phenotype of six children with parainflammatory leukoencephalomyelitis (PIL). Patients with PIL demonstrate an increased prevalence of the HLA-A1, -A28, -B44, -DR6, and -DR7 antigens. These HLA associations are different from those reported in other inflammatory demyelinating diseases, including multiple sclerosis, optic neuritis, Guillain-Barré syndrome, and chronic relapsing inflammatory polyneuropathy. The HLA constitution of the patient appears to be one important host factor in determining the nature of the immune response to an encephalitogenic challenge.
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Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis that is histologically characterized by an infiltration of the dermis by neutrophils. A 12-year-old adolescent female patient recently diagnosed with acute promyelocytic leukemia presented with fever and was hospitalized for antibiotic management after 22 days of being treated with a treatment protocol based on daunorubicin, all-trans retinoic acid (ATRA), and prophylaxis with dexamethasone, the patient developed erythematous skin lesions located mostly on the extremities. Lesions evolved into painful subcutaneous nodules, and one lesion evolved into a 2.
View Article and Find Full Text PDFNeonatal Sweet syndrome: a potential marker of serious systemic illness.
Pediatrics
May 2012
Department of Immunology, Sydney Children's Hospital, Randwick, New South Wales, Australia.
Sweet syndrome is an inflammatory disease characterized by fever and painful erythematous plaques with a dermal neutrophilic infiltrate. It is most common in adults, where it is often parainflammatory or paraneoplastic, but is rare in children. We describe 3 cases of neonatal Sweet syndrome, including 1 patient who had myelodysplastic syndrome and immunodeficiency, the first report of a premalignancy underlying infantile Sweet syndrome.
View Article and Find Full Text PDFAcute febrile neutrophilic dermatosis (Sweet's syndrome) in childhood and adolescence: two new patients and review of the literature on associated diseases.
Eur J Pediatr
January 2009
Department of Pediatrics and Pediatric Rheumatology, Klinikum Stuttgart, Olgahospital, Bismarckstr. 8, 70176, Stuttgart, Germany.
Objectives: The objectives of this study were to analyse the literature on Sweet's syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet's syndrome are reported.
Methods: A literature search was performed on Pub med using search terms "sweet* syndrome*" and neutrophil* dermatos*.
Transient cerebellar mutism in the course of acute cerebellitis.
Pediatr Neurol
January 2004
Department of Neurology, Pendeli Children's Hospital, Athens, Greece.
Transient mutism after posterior fossa surgery in children or associated with cerebellar hemorrhage or trauma is a recognized phenomenon. However, its association with parainflammatory cerebellitis has been rarely described. We report on a previously healthy 3-year-old child with severe cerebellitis after acute gastroenteritis of unidentified cause.
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Results of a study of 44 patients with pyoderma gangrenosum (PG) are presented. Each patient was diagnosed using standardized diagnostic criteria and followed up systematically. Thirty patients were women and 14 men.
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