Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis, clinically characterized by the classical triad: palpable purpuric skin lesions, edema and fever, and is commonly misdiagnosed as Henoch-Schönlein purpura. In addition to its sudden onset, AHEI is also characterized by its self-limited course with complete and spontaneous recovery occurring between 1 and 3 weeks. Because of the scarcity of studies on therapy with corticosteroids, the conservative approach is usually recommended. The authors report an unusual case of an one-year-old boy who presented with typical cutaneous rash of AHEI and orchitis, the latter showing complete resolution after less than 24 hours of prednisolone therapy. The authors call attention to this entity mainly as a differential diagnosis of Henoch-Schönlein purpura and to the importance of new studies to establish the benefits of corticosteroid therapy for AHEI.

The symptoms of a previously healthy 14-year-old female with an initial history of tooth pain and swelling of the left maxillary evolved to a progressive headache and altered neurological findings characterized by auditory hallucinations, sleep disturbances, and aggressiveness. She was brought to the emergency department after 21 days of the initial symptoms. An initial computed tomography (CT) scan showed frontal subdural empyema with bone erosion. The symptoms continued to evolve to brain herniation 24 hours after admission. A second CT scan showed a left internal jugular vein thrombosis. The outcome was unfavorable and the patient died on the second day after admission. The autopsy findings depicted rarefaction of the cranial bone at the left side of the frontal sinus, and overt meningitis. The severe infection was further complicated by thrombophlebitis of the left internal jugular vein up to the superior vena cava with septic embolization to the lungs, pneumonia, and sepsis. This case report highlights the degree of severity that a trivial infection can reach. The unusual presentation of the sinusitis may have wrongly guided the approach of this unfortunate case.

No abstract

Pertussis is an acute and very contagious pulmonary disease, clinically characterized by periods of coughing and paroxysms that may cause death. The disease afflicts mainly the pediatric population and is life threatening to children under the age of 1 year. Since the beginning of the second millennium, the number of cases of pertussis has increased, menacing public health, despite the availability of the pertussis vaccine. The resurgence of the disease among adults and older children creates a reservoir of infection that will afflict the unimmunized or incompletely immunized children. As newborns and infants show the highest mortality rate, immunization during pregnancy is a new strategy to reduce the burden of pertussis. The authors report the case of a newborn that presented respiratory distress accompanied by marked leukocytosis. Bronchiolitis was the initial diagnostic hypothesis, but the clinical picture became typical of pertussis when paroxysmal coughing ensued. Isolation of the Bordetella pertussis and antigenic demonstration by polymerase chain reaction were positive from respiratory secretion. Despite appropriate antibiotic therapy and intensive care management the child died and the autopsy showed characteristic diagnostic findings. The authors call attention to this diagnosis when facing respiratory failure among young children, mainly in the presence of marked leukocytosis. Thorough research on the immunization status of the patient’s social environment is of crucial importance.

No abstract

Lymphangioma is a rare, benign lesion derived from a malformation of the lymphatic system, which is more frequently found in the head, neck, and axilla. However, it may be present anywhere in the body, and the diagnosis involves adults as children with some distinct clinical features among them. In pediatric patients, abdominal cystic lymphangioma occurs mostly in the mesentery presenting abdominal pain, intestinal obstruction, or, more rarely, hemorrhage. The authors report the case of a child with a short-course history of fever, abdominal pain, and constipation. The physical examination disclosed the presence of an abdominal mass and signs of peritoneal irritation. Imaging was consistent with a cystic lesion compressing the sigmoid colon and laterally displacing the remaining loops. Exploratory laparotomy was undertaken, and a sigmoidectomy, followed by Hartman’s colostomy, was performed. Histological examination revealed the nature of the lesion as a cystic lymphangioma. The authors highlight the clinical features of this entity and call attention to this disease in the differential diagnosis of acute abdomen or abdominal pain, mainly in pediatric patients.

Sphingomonas paucimobilis is an aerobic Gram-negative bacillus that, although rare in humans, most commonly infects immunocompromised and hospitalized patients. Among the 59 pediatric cases of S. paucimobilis infection reported in the literature, the most common diagnosis involves isolated bacteremia. These cases are related to sporadic or epidemic infections. Death related to this infection occurred in only one case. The authors report a case of an 11-year-old boy with the diagnosis of Sphingomonas paucimobilis otomastoiditis and a thorough review of the literature on this infection in pediatrics. The patient presented a 20-day history of fever, otalgia, otorrhea, and progressive retroauricular swelling with protrusion of the left ear; despite 15 days of amoxicillin regimen. His past medical history included chronic bilateral otitis media, but no cause of immunosuppression was found. A brain computed tomography scan showed left otomastoiditis associated with a large circumscribed fluid collection with deep involvement of the soft tissues of the temporal region, including the subperiosteal space. Blood tests showed neutrophilia and elevated C-reactive protein. Surgical manipulation of the cited collection drained a large amount of a fetid purulent secretion. Ceftazidime and clindamycin were empirically initiated. The outcome was favorable, with fever defervescence and resolution of the scalp deformation. Culture of the drained secretion was positive for S. paucimobilis. Ciprofloxacin was scheduled for a further 10 days after discharge. The follow-up showed complete recovery. As far as we know, this is the first case of S. paucimobilis otomastoiditis, complicated with subperiosteal abscess in an immunocompetent child. The authors call attention to the increasing number of reports on S. paucimobilis infection over the years, and therefore to the importance of this pathogen, which was previously underestimated.

We evaluated 113 pediatric patients with chronic hepatitis C from 2009 to 2019 at a Brazilian tertiary center. Seventy patients received pegylated-interferon treatment. The sustained virologic response was 61.4%, and 92.8% reported side effects. Currently, we are following 39 patients with chronic hepatitis C, 24 of whom are eligible for treatment with direct-acting antivirals according to Brazilian recommendations.