Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.
G Ital Dermatol Venereol
PMID:[Sebaceous linear nevus syndrome with hemimegalencephaly. Report of a case]. 276 20

We review the medical issues and emergencies potentially encountered in the practice of general or surgical dermatology. Traditional guidelines have largely consisted of dated extrapolations from the nondermatologic literature concerning procedures that are primarily irrelevant to dermatology. This article outlines a rational approach to organizing an office emergency plan for anaphylaxis, stroke, status epilepticus, myocardial infarction, and hypertensive crisis. We discuss the literature that has influenced current office behavior regarding endocarditis prophylaxis, the use of electrosurgery with pacemakers, arrhythmogenic drug interactions, vasovagal syncope, lidocaine "allergy," and bleeding complications from oral anticoagulants. Recommendations for managing these issues in a dermatologic context are provided.
J Am Acad Dermatol 1997 Jan
PMID:Medical issues and emergencies in the dermatology office. 948 99

The term hypomelanosis of Ito (HI) is applied to individuals with skin hypopigmentation following the lines of Blaschko (type 1a of patterns indicative of somatic mosaicism as defined by Happle). Even though originally described as a purely cutaneous disease, subsequent reports of HI have included a 30-94% association with multiple extracutaneous manifestations. The frequency of extracutaneous associations has led many authors to consider HI to be neurocutaneous disorder. We report a male infant with cutaneous hypomelanosis along the lines of Blaschko distributed on the left half of the body who developed status epilepticus. Neuroimaging studies disclosed an angiomatous enlargement of the right choroid plexus and a gyral pattern of cortical and subcortical calcification in the right occipital region. Thus a diagnosis could be made of HI and associated Sturge-Weber syndrome-like leptomeningeal angiomatosis. This previously unreported association lends further support to the consideration of hypomelanosis of Ito as a marker of somatic mosaicism with frequently associated neurologic abnormalities. A relationship between HI and Sturge-Weber syndrome, two neuroectodermal disorders with a genetic mosaicism basis, might be possible due to nonallelic twin-spotting which in the embryologic period would define an abnormal development of neural, vascular, and cutaneous structures.
Pediatr Dermatol
PMID:Hypomelanosis of Ito with Sturge-Weber syndrome-like leptomeningeal angiomatosis. 1243 59

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.
Pediatr Dermatol
PMID:A pruritic linear urticarial rash, fever, and systemic inflammatory disease in five adolescents: adult-onset still disease or systemic juvenile idiopathic arthritis sine arthritis? 1546 68