Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children: a brief literature review.

OBJECTIVE: To describe clinical, diagnostic and therapeutic characteristics of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. DATA SOURCE: Literature review in the PubMed database by using specific descriptors to identify all articles published in the English language in the last three years; 38 articles were found. After performing selection of titles and abstract analysis, 13 out of the 38 articles were fully read. Relevant studies found in the references of the reviewed articles were also included. DATA SYNTHESIS: The PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and cervical Adenitis) is a medical condition grouped among the periodic fever syndromes. The etiology is uncertain, but possibly multifactorial, and its symptoms are accompanied by recurrent febrile episodes although weight and height development are preserved. It is a self-limiting disease of benign course with remission of two to three years without significant interference in the patient's overall development. Treatment consists of three pillars: interruption of febrile episodes, increase in the interval between episodes, and remission. CONCLUSIONS: Despite several attempts to establish more sensitive and specific criteria, the diagnosis of PFAPA syndrome is still clinical and reached by exclusion, based on the modified Marshall's criteria. The most common pharmacological options for treatment include prednisolone and betamethasone; colchicine may be used as prophylaxis, and surgical treatment with tonsillectomy can be considered in selected cases.

PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever.

We reviewed the medical records of patients with periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) to investigate the clinical course, treatment response, and association with MEFV gene mutation. Familial Mediterranean fever should be considered in patients with PFAPA who do not respond to adenotonsillectomy.

Mycobacterium fortuitum-related lymphadenitis associated with the varicella-zoster virus.

Lymphadenitis caused by non-tuberculous mycobacteria is an uncommon manifestation in immunocompetent individuals. Here, we report a case of Mycobacterium fortuitum infection in a previously healthy 9-year-old patient who developed cervical lymphadenitis evolving to a suppurative ulcer associated with a varicella-zoster virus infection. We discuss the relationship between the varicella-zoster virus and the immune response of the host as an explanation for the unusual progression of the case.

Mycobacterium fortuitum-related lymphadenitis associated with the varicella-zoster virus

Lymphadenitis caused by non-tuberculous mycobacteria is an uncommon manifestation in immunocompetent individuals. Here, we report a case of Mycobacterium fortuitum infection in a previously healthy 9-year-old patient who developed cervical lymphadenitis evolving to a suppurative ulcer associated with a varicella-zoster virus infection. We discuss the relationship between the varicella-zoster virus and the immune response of the host as an explanation for the unusual progression of the case.

Familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

We report three familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome, including a pair of monozygotic twins and their mother. It suggests that periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome may have a certain monogenetic background.

A large and massive abdominal venous thrombosis associated with the presence of a big axillary mass, lupus-like syndrome and antiphospholipid antibodies.

This case report concerns a 40-year-old patient with an unspecific abdominal pain, diarrhoea, a big axillary mass and a previous pulmonary infection. After biopsy of the axillary mass the diagnosis of lymphoma was excluded based on the presence of cells expressing polyclonal antibodies. Abdominal CT scans and angionuclear magnetic resonance showed an extensive intestinal venous thrombosis. The patient also presented positive results for C and S proteins, lupic anticoagulant factor and antiphospholipid antibodies (anticardiolipin antibodies - IgM and IgG). Treatment started with administration of recombinant tissue plasminogen activator and heparin which decreased the degree of thrombosis. Antibiotics were also administrated to treat pulmonary and abdominal infections. After 25 days, he was discharged with no signs of infection, no abdominal pain and reduction of the thrombosis. He was medicated with warfarin, hydroxichloroquine and clopidogrel. Forty-five days after discharge, abdominal CT scan showed a significant regression of thrombosis.

Reporte de dos casos de linfadenitis supurada causada por bacilo de Calmette-Guerin / Report of two cases of suppurated lymphadenitis caused by Bacillus Calmette-Guerin treated with recombinant ã-interferon

La linfadenitis supurada es una complicación poco frecuente que sigue a la vacunación con bacilo de Calmette-Guerin. Se describen los casos de dos niños con reacciones adversas graves inducidas por esta vacuna, en ambos casos, linfadenitis regional supurada y abscedada, un mes después de nacidos. Después de cursos infructuosos de cirugía y quimioterapia, ambos recibieron interferón gamma recombinante por vía intramuscular, en una dosis inicial de 50 000 UI/kg (máximo: 1 000 000 UI), diariamente durante las primeras 4 semanas, y se disminuyó luego la frecuencia de administración. Esta citoquina fue bien tolerada, solo se presentaron complicaciones con fiebre, que fueron controladas bien con antipiréticos. El interferón gamma recombinante puede constituir una nueva y efectiva alternativa terapéutica para el tratamiento de la linfadenitis supurada causada por este bacilo(AU)

Suppurative lymphadenitis is a non frequent complication following Bacillus Calmette-Guerin (BCG) vaccination. Two paediatric patients with adverse reactions induced by the BCG vaccine are presented, both with suppurative and abscessed regional lymphadenitis, one month after birth. After failed courses of surgery and chemotherapy, they were treated with 50 000 IU/Kg (maximum: 1 000 000 IU) of recombinant interferon (IFN) gamma, intramuscularly, daily during 4 weeks and 3 or 2 tpw afterwards. The first case, a nursing girl with family history of tuberculosis, had a rapid involution of the lesions since the first month of treatment, with drainage ceasing and gradual disappearance of the inflammatory signs. At the end of the 6 months of treatment, residues of the lesions were imperceptible and new adenopathies or relapses were not detected during 4 years of follow up. The second case, a boy without family history of tuberculosis, presented more lesions. The signs of marked improvement were observed in the whole affected region one year after IFN gamma started. Their treatment was extended for almost 2 years, when the scars took the normal skin pigmentation. The cytokine was well tolerated; few febrile events were recorded, well-controlled with antipyretics. We can conclude that IFN gamma could be a new effective therapeutic alternative for the treatment of the suppurated lymphadenitis caused by BCG vaccination(AU)

Reporte de dos casos de linfadenitis supurada causada por bacilo de Calmette-Guerin / Report of two cases of suppurated lymphadenitis caused by Bacillus Calmette-Guerin treated with recombinant ã-interferon

La linfadenitis supurada es una complicación poco frecuente que sigue a la vacunación con bacilo de Calmette-Guerin. Se describen los casos de dos niños con reacciones adversas graves inducidas por esta vacuna, en ambos casos, linfadenitis regional supurada y abscedada, un mes después de nacidos. Después de cursos infructuosos de cirugía y quimioterapia, ambos recibieron interferón gamma recombinante por vía intramuscular, en una dosis inicial de 50 000 UI/kg (máximo: 1 000 000 UI), diariamente durante las primeras 4 semanas, y se disminuyó luego la frecuencia de administración. Esta citoquina fue bien tolerada, solo se presentaron complicaciones con fiebre, que fueron controladas bien con antipiréticos. El interferón gamma recombinante puede constituir una nueva y efectiva alternativa terapéutica para el tratamiento de la linfadenitis supurada causada por este bacilo.

Suppurative lymphadenitis is a non frequent complication following Bacillus Calmette-Guerin (BCG) vaccination. Two paediatric patients with adverse reactions induced by the BCG vaccine are presented, both with suppurative and abscessed regional lymphadenitis, one month after birth. After failed courses of surgery and chemotherapy, they were treated with 50 000 IU/Kg (maximum: 1 000 000 IU) of recombinant interferon (IFN) gamma, intramuscularly, daily during 4 weeks and 3 or 2 tpw afterwards. The first case, a nursing girl with family history of tuberculosis, had a rapid involution of the lesions since the first month of treatment, with drainage ceasing and gradual disappearance of the inflammatory signs. At the end of the 6 months of treatment, residues of the lesions were imperceptible and new adenopathies or relapses were not detected during 4 years of follow up. The second case, a boy without family history of tuberculosis, presented more lesions. The signs of marked improvement were observed in the whole affected region one year after IFN gamma started. Their treatment was extended for almost 2 years, when the scars took the normal skin pigmentation. The cytokine was well tolerated; few febrile events were recorded, well-controlled with antipyretics. We can conclude that IFN gamma could be a new effective therapeutic alternative for the treatment of the suppurated lymphadenitis caused by BCG vaccination.

Tuberculosis ganglionar a forma de fiebre de origen desconocido: a propósito de un caso / Ganglionary tuberculosis as fever of unknown origin: apropos of a case

La tuberculosis, tanto en su forma pulmonar como extrapulmonar constituye una enfermedad reemergente al nivel mundial, asociada con insuficiencias de los programas de control sanitario o con el síndrome de inmunodeficiencia adquirida. El modo de presentación de las formas extrapulmonares se relaciona con síntomas constitucionales, que incluyen la fiebre y con signos dependientes del órgano afectado. Se presentó el caso de una adenitis granulomatosa en etapa o categoría 3, grupo integrado por aquellos casos nuevos de formas menos grave de tuberculosis extrapulmonar (TBe) con confirmación histopatológica del bacilo. Se enfatizó sobre la presentación a forma de fiebre de origen desconocido (FOD), al reunir los criterios necesarios por haber estado ingresada en otro centro hospitalario por más de 1 mes, sin haber llegado al diagnóstico a pesar de haber sido estudiada, y se resaltó la necesidad imperiosa de la búsqueda de este diagnóstico ante todo paciente con cuadro adénico febril e historia familiar de TB (AU)

Tuberculosis, both in its pulmonary and extrapulmonary form is a reemerging disease in the world associated with insufficiencies of the health control programs or with the acquired immunodeficiency syndrome. The mode of presentation of the extrapulmonary forms is related to constitutional symptoms that include fever, and to signs depending on the affected organ. The case of a stage III granulomatous adenitis, a group composed of those new cases of less severe forms of extrapulmonary tuberculosis with histopathological confirmation of the bacillus, was presented. Emphasis was made on the presentation as a FUO, on having the necessary criteria for having been admitted in another hospital for more than a month without a definitive diagnosis despite having been studied. The pressing need to search this diagnosis in every patient with febrile adenic picture and family history of TB was stressed(AU)

Tuberculosis ganglionar a forma de fiebre de origen desconocido: a propósito de un caso / Ganglionary tuberculosis as fever of unknown origin: apropos of a case

La tuberculosis, tanto en su forma pulmonar como extrapulmonar constituye una enfermedad reemergente al nivel mundial, asociada con insuficiencias de los programas de control sanitario o con el síndrome de inmunodeficiencia adquirida. El modo de presentación de las formas extrapulmonares se relaciona con síntomas constitucionales, que incluyen la fiebre y con signos dependientes del órgano afectado. Se presentó el caso de una adenitis granulomatosa en etapa o categoría 3, grupo integrado por aquellos casos nuevos de formas menos grave de tuberculosis extrapulmonar (TBe) con confirmación histopatológica del bacilo. Se enfatizó sobre la presentación a forma de fiebre de origen desconocido (FOD), al reunir los criterios necesarios por haber estado ingresada en otro centro hospitalario por más de 1 mes, sin haber llegado al diagnóstico a pesar de haber sido estudiada, y se resaltó la necesidad imperiosa de la búsqueda de este diagnóstico ante todo paciente con cuadro adénico febril e historia familiar de TB.

Tuberculosis, both in its pulmonary and extrapulmonary form is a reemerging disease in the world associated with insufficiencies of the health control programs or with the acquired immunodeficiency syndrome. The mode of presentation of the extrapulmonary forms is related to constitutional symptoms that include fever, and to signs depending on the affected organ. The case of a stage III granulomatous adenitis, a group composed of those new cases of less severe forms of extrapulmonary tuberculosis with histopathological confirmation of the bacillus, was presented. Emphasis was made on the presentation as a FUO, on having the necessary criteria for having been admitted in another hospital for more than a month without a definitive diagnosis despite having been studied. The pressing need to search this diagnosis in every patient with febrile adenic picture and family history of TB was stressed.

A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

OBJECTIVE: We carried out a prospective, randomized, controlled trial to clarify the effect of tonsillectomy on the clinical course of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. STUDY DESIGN: Twenty-six consecutive children (mean age 4.1 years) with at least 5 PFAPA attacks were recruited from 3 tertiary care pediatric hospitals during 1999-2003 and randomly allocated to tonsillectomy or follow-up alone. They were all followed up with symptom diaries for 12 months. Tonsillectomy was allowed after 6 months in the control group if the attacks recurred. RESULTS: Six months after randomization all 14 children in the tonsillectomy group and 6/12 children in the control group (50%) were free of symptoms (difference 50%, 95% confidence interval 23% to 75%, P < .001). Tonsillectomy was performed on 5/6 of the patients in the control group who still had symptoms after 6 months. The remaining unoperated child in the control group had recurrences of the fever episodes throughout the follow-up, but the symptoms became less severe, and the parents did not choose tonsillectomy. CONCLUSION: Tonsillectomy appeared to be effective for treating PFAPA syndrome. The fever episodes ceased without any intervention in half of the control subjects. We conclude that although the mechanisms behind this syndrome are unknown, tonsillectomy can be offered as an effective intervention for children with PFAPA.

Tumor necrosis factor receptor-associated periodic syndrome in a young adult who had features of periodic fever, aphthous stomatitis, pharyngitis, and adenitis as a child.

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) was diagnosed in a 22-year-old man after a 1-year history of periodic fever, myalgia, conjunctivitis, cervical lymphadenopathy, and oral ulcers. As a child he had signs and symptoms suggestive of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. This report indicates the importance of considering TRAPS as a cause of periodic fever in older children and adults and that TRAPS may present with signs and symptoms suggestive of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in young children.

Herpes simplex virus type 2 infection associated with urinary retention in the absence of genital lesions.

A 14-year-old boy had inguinal lymphadenitis and associated urinary retention; herpetic genital and anal lesions were absent. Lymph node biopsy established the presence of infection with herpes simplex virus type 2. We recommend that this infection be considered in the differential diagnosis of adolescent patients with urinary retention and constipation, even when the diagnostic clue provided by genital ulcers is absent.

Doença da arranhadura do gato / Cat-scratch disease

Descriçäo de um caso de doença da arranhadura de gato ou linforreticulose benigna, que envolve gânglios linfáticos que drenam os sítios dérmicos ou conjuntivais primários de inoculaçäo. A doença é autolimitada e benigna, mas pode, por vezes, progredir para infecçäo sistêmica grave e recorrente com encefalite, neuroretinite e osteomielite. Seu agente etiológico é a Rochalimaea benselae, um bacilo Gram-negativo pleomórfico de pequenas dimensöes, membro do subgrupo a-2 das a - proteobactérias

[Brachial plexus neuropathy following pyogenic cervical adenophlegmon]. / Névralgie brachiale amyotrophiante au décours d'un adénophlegmon cervical à pyogènes.

BACKGROUND: Brachial plexopathy is rare in children; it may follow a non-specific respiratory infection or occur after a specific viral disease or immunization. CASE REPORT: An 8 year-old girl was admitted suffering from presuppurative acute cervical adenitis. She was given penicillin V, netilmicin and corticosteroids. The adenitis gradually resolved over a period of ten days. There was no evidence of a specific bacterial infection. The patient suffered from pain localized to her right shoulder 15 days after admission; this pain was resolved within 3-4 days but was followed by paralysis affecting the upper brachial roots without sensory signs. EMG performed 15 days later showed signs of denervation. Amyotrophy set in rapidly but recovery under physiotherapy was complete 5 months later. CONCLUSION: This plexopathy resembling the Parsonage-Turner syndrome could be allergic in pathogenesis although similar cases after bacterial, possibly streptococcal, infection remain to be confirmed.

Evaluation of oral erythromycin and local instillation of INH therapy, in infants with BCG lymphadenitis - abstract

The most common adverse effect of BCG vaccination is regional lymphadenitis, but the treatment of this complication is controversial. A group of 69 patients, with BCG adenitis following vaccination were randomly assigned to receive either "no therapy" or oral erythromycin for one month. Systemic erythromycin therapy caused earlier resolution of the lymph node but failed to prevent progression to abscess formation. Patients who developed subsequent abscesses (36) and another group who had abscess at the time of presentation (27) showed more rapid resolution when treated with single instillation of isoniazid (50mg) into the abscess cavity, compared to oral erythromycin therapy (AU)

Abscesses and caseous lymphadenitis in goats in tropical semi-arid north-east Brazil.

This study reports the incidence of abscesses in relation to Corynebacterium pseudotuberculosis infection in goats in north-east Brazil. Among 656 goats examined periodically over almost two years 41.6% were found with superficially palpable abscesses. Most of the infected areas were in the anterior half of the body in front of the pre-scapular region; the inguinal region formed the other prominent area. Among 486 dead or slaughtered goats, 56 (11.5%) revealed abscesses in the internal organs. Lungs and epididymis were affected most, liver, spleen, udder and lateropharyngeal nodes were next in order and kidneys and mediastinal nodes were least affected. Thus the overall incidence of external and internal abscesses was 28.8%; of all the abscesses 27.7% were due to C. pseudotuberculosis and the rest due to infections of Corynebacterium pyogenes and several species of streptococci or staphylococci. The growth of C. pseudotuberculosis-infected and non-infected goats was of the same order and it seems that caseous lymphadenitis is not the cause of much financial loss in north-east Brazil but in view of its high incidence remedial measures should be considered necessary in regions where the goat meat industry is likely to become more intensive and important.