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Kikuchi disease is a self-limited disorder of unknown etiology characterized by focal painful lymphadenitis, fever, and weight loss that can be mistaken for malignancy. Diagnosis is established by node biopsy. Kikuchi disease is endemic in Asia; 10 cases have been reported in the US to date. We report 3 cases and review other US cases., (Copyright © 2014 Mosby, Inc. All rights reserved.)

Objective: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome., Study Design: Patients enrolled in a PFAPA registry were contacted and surveyed., Results: Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses., Conclusions: In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Objective: To determine how frequently Connecticut primary care physicians are diagnosing and treating patients with chronic Lyme disease., Study Design: A survey was mailed to a random 33% sample of primary care physicians in Connecticut, which asked how many cases of Lyme disease and chronic Lyme disease they diagnosed and treated in the last 3 years., Results: The survey had a response rate of 39.1%. Physician respondents (n = 285) fit in one of 3 groups. Group 1 included 6 of 285 (2.1%) physicians who diagnose and treat patients with chronic Lyme disease. Group 2 included 137 of 285 (48.1%) physicians who are undecided on the existence of chronic Lyme disease, but did not diagnose or treat any patients with chronic Lyme disease. Group 3 included 142 of 285 (49.8%) physicians who do not believe in the existence of chronic Lyme disease., Conclusion: A small percentage (2.1%) of primary care physicians diagnose and treat patients for presumed chronic Lyme disease., (Copyright © 2010 Mosby, Inc. All rights reserved.)

Background and Objective: A recombinant lipoprotein vaccine against Lyme disease, containing 30 microg of Borrelia burgdorferi outer surface protein A (OspA) with aluminum adjuvant, has been shown in a large US field trial of subjects >/=15 years of age to offer 76% efficacy against clinical Lyme disease after 3 injections given at 0, 1, and 12 months. Lyme disease is also an important problem in children; thus, OspA vaccine trials in children are needed. The purpose of this study was to investigate the safety and immunogenicity of 2 different doses of lipoprotein OspA with aluminum adjuvant vaccine in healthy children 5 to 15 years of age in a double-blind, randomized study., Study Design: In a double-blind study, 250 children from the Czech Republic were randomly assigned to receive 15 microg or 30 microg of OspA vaccine at 0, 1, and 2 months. Serum samples, obtained before vaccination and 1 month after the second and third doses, were analyzed for antiOspA antibody. Solicited and unsolicited symptoms were collected from diary cards., Results: Local pain at the injection site was reported by approximately 76% of the 250 children. Headaches (after 5% to 18% of the injections) and malaise (after 2% to 16% of the injections) were the most frequently reported general symptoms. Local and generalized symptoms were not different between the 15 microg and 30 microg groups, and all symptoms resolved within 4 days. Both doses were highly immunogenic, with the 30 microg dose eliciting higher antibody levels. Seroconversion occurred in 99% of the 250 children., Conclusions: The OspA vaccine against Lyme disease was well tolerated and highly immunogenic in children.

Objectives: To describe the presentation, clinical course, therapeutic response, and long-term follow-up of patients with a syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA)., Study Design: Patients with PFAPA (n = 94) referred over a 10-year period completed a registry form and provided medical records. Follow-up telephone calls were made in late 1997 to determine the persistence of episodes and sequelae., Results: PFAPA episodes lasted 4.8 days (95% confidence interval 4.5 to 5.1) and recurred every 28 days (confidence interval 26 to 30), with a maximal temperature of 40.5 degrees C (confidence interval 40. 4 degrees to 40.6 degrees ). Of the 83 children available for follow-up, 34 no longer had episodes. In the remainder the episodes did not differ in character but recurred less frequently over time. The affected children had no long-term sequelae. Glucocorticoids were highly effective in controlling symptoms. Tonsillectomy and cimetidine treatment were associated with remission in a small number of patients., Conclusions: PFAPA is a not uncommon cause of periodic fever in children. In some children the syndrome resolves, whereas symptoms in others persist. Long-term sequelae do not develop. The syndrome is easily diagnosed when regularly recurring episodes of fever are associated with aphthous stomatitis, pharyngitis, or cervical adenitis.

We describe a patient with chronic congenital idiopathic neutropenia whose neutropenia resolved on four occasions during chloramphenicol therapy. This is the second report of chloramphenicol-responsive chronic neutropenia. Chloramphenicol, in addition to its antimicrobial action, has immune-modulating activity, which may explain the reversal of neutropenia.

Study Objective: To determine whether orally administered acyclovir is of therapeutic benefit for varicella in otherwise healthy adolescents, and to compare the severity of the disease in adolescents with that in younger children., Design: Multicenter, randomized, placebo-controlled, double-blind trial., Setting: Patients' homes and university hospital clinics., Patients: Sixty-eight adolescents between 13 and 18 years of age with varicella entered the study. Of the 62 adolescents with laboratory-confirmed varicella who were included in the final analysis, 31 received acyclovir and 31 received placebo., Interventions: Placebo or an 800 mg acyclovir tablet was given orally four times daily for 5 days, beginning within 24 hours of onset of rash., Measurements and Main Results: Acyclovir recipients had significant reductions in times to cessation of new lesion formation (p less than 0.001), maximum number of lesions (p = 0.019), and defervescence (p = 0.045). Mean constitutional illness score was significantly reduced on day 4 (0.5 vs 1.5, p = 0.05), as was the mean number of residual hypopigmented lesions present on 28-day follow-up examination (22.7 vs 92.7, p = 0.018). Two complications, both bacterial superinfections, occurred in placebo recipients. Adverse experiences and varicella-zoster virus antibody titers measured 28 days after enrollment were similar in both treatment groups. Comparison of placebo recipients with children 2 to 12 years of age participating in a companion study indicated that varicella is more severe in adolescents: mean maximum total lesions (421 vs 347, p = 0.003), mean maximum constitutional illness score (3.1 vs 2.2, p = 0.032), and mean number of residual lesions (92.7 vs 33.2, p = 0.01) were all greater in the adolescent population., Conclusions: Oral acyclovir therapy is safe and effective for treatment of varicella in otherwise healthy adolescents; this may be an appropriate subgroup for treatment with antiviral drugs because the disease is more severe in them than in younger children.

The six classic exanthems of childhood have many similar physical findings. Familiarity with them, as well as with important laboratory data, allows early and accurate diagnosis of these often confusing diseases. Recognition and treatment of possible sequelae also improve prognosis. In this review we discuss epidemiology, etiology, clinical manifestations, pathologic and laboratory findings, differential diagnosis, therapy, and prevention of each of the exanthems.