Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA.
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.
9. Golstein PE, Deviere J, Cremer M. Acute hepatitis and drug-related lupus induced by minocycline treatment. Am J Gastroenterol. 1997;92:143-146. 10. Singer SJ, Piazza-Hepp TD, Girardi LS, Moledina NR. Lupuslike reaction associated with minocycline. JAMA. 1997;277:295-296. 11. Crosson J, Stillman MT. Minocycline-related lupus erythematosus with associated liver disease. J Am Acad Dermatol. 1997;36(5 More than 44000 new cases of melanoma are detected annually in the United States, and the number is steadily increasing. The majority of patients are treated with surgical excision and may, in addition, undergo sentinel node mapping, lymph node dissection, and adjunctive treatment. The purpose of this study was to develop rational, evidence-based guidelines for following up patients with 1983 American Joint Committee on Cancer stage I (Ͻ1.5 mm thick), II (localized disease Ͼ1.5 mm thick), and III (local nodal or in-transit metastases) melanoma. The study was a retrospective review of charts of 373 patients who were followed up according to the surveillance protocol at the Yale Melanoma Unit, New Haven, Conn, between January 1988 and December 1994. Each patient was followed up for at least 2 years. The surveillance protocol included combined frequent comprehensive examination with extensive patient education. Periodic examinations included history and physical examination; laboratory tests, such as complete blood cell count, liver function tests , and serum lactate dehydrogenase level; and chest x-ray (CXR) (computed tomographic scans were done for patients with stage III melanoma).The collected data included (1) the time between first visit and recurrence; (2) whether the recurrence was detected by the patient or by a physician during a follow-up visit; (3) the method of detection for physician-detected recurrences (ie, history and physical examination, review of systems, complete blood cell count, liver function tests, serum lactate dehydrogenase level, and CXR or other imaging techniques); (4) survival; (5) location of recurrences; and (6) development of second primary melanomas.The results indicated that there were recurrences in 52 (25%) of 210 males and 26 (16%) of 163 females. Of the 373 patients, 78 (21%) had a recurrence. The proportions of patients who developed recurrence were 9 (5%) of 193, 35 (30%) of 117, and 34 (54%) of 63, for patients with stage I, II, and III disease, respectively. The median time to recurrence in patients with stages I, II, and III disease was 22 months (range, 2-61 months), 13 months (range, 2-71 months), and 11 months (range, 2-54 months), respectively. Most patients (n=62, 79%) who developed a recurrence in the follow-up period did so within the first 2 years. Six percent (n=5) of patients developed recur-(REPRINTED) ARCH DERMATOL / VOL 136, SEP 2000
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