BACKGROUNDRosai-Dorfman disease is a rare non-Langerhans histiocytic disease characterized by lymph node enlargement and extranodal involvement, such as nonpruritic skin lesion, involvement of the nasal and paranasal sinuses, central nervous system (CNS), ocular, renal and other visceral involvement. Rosai-Dorfman disease can occur as an isolated disorder or in association with autoimmune and neoplastic diseases. Its prevalence is 1:200,000 and it is most often seen in adolescents and adults. To date, we have about 1,000 published cases. We present a case report of a patient with fever of undetermined origin whose first symptom was an important facial edema.
CASE REPORTWe described a 14-year-old male patient admitted at our tertiary referral hospital due to fever of undetermined origin, facial edema and abdominal pain. Additionally, it was noted lymph node enlargement in cervical and inguinal chains. Laboratory tests revealed neutropenia and thrombocytopenia, with mild abnormal inflammatory biomarkers. Infectious serologies were negative and myelogram presented no atypic cells. Computed tomography scans showed marked thoracic lymph nodes, abdominal adenomegaly and hepatosplenomegaly. Investigation ruled out chronic granulomatous disease by a normal dihydrorhodamine test. A peripheric inguinal lymph node was biopsied. The pathology showed granulomas, with histological and immunohistochemical findings consistent with Rosai-Dorfman disease, allowing the differentiation with IgG4 disease and sarcoidosis.
CONCLUSIONWe reported an adolescent with Rosai-Dorfman disease whose first presentation was characterized by fever of undetermined origin and important facial edema, a previously undescribed feature of this disease. The lymph node biopsy was fundamental in establishing the diagnosis.
Rosai-Dorfman disease is a rare non-Langerhans histiocytic disease characterized by lymph node enlargement and extra-nodal involvement. Extranodal disease occurs in 40% of cases and common sites of involvement are: skin, nasal cavity, bone, orbital tissue and central nervous system. Lesions are usually plaques, nodules or papules of insidious growth and no associated symptoms. We report here an atypical case of Rosai Dorfman in an adolescent with fever of undetermined origin, without significant lymphadenopathy and diffuse facial edema.
Objective: The objective of this study was to assess interpersonal nonsexual violence against children and adolescents in a tertiary university hospital. Methods: A cross-sectional study was performed in 240 patients under nonsexual violence situation for 15 consecutive years. Data analyses included demographic data, hospital referral site, type and author of nonsexual violence, legal referral, laboratorial and imaging examinations, and outcomes. Results: Nonsexual violence situation was diagnosed in 240 (0.1%) of 295,993 patients for 15 years: 148 (61.7%) in children and 92 (38.3%) in adolescents. Out of 240, the most frequent types of violence were negligence in 156 (65.0%), physical 62 (25.8%), psychological/emotional aggression 52 (21.7%), Munchausen by proxy syndrome 4 (1.7%), and bullying/cyberbullying in 3 (1.3%). Out of 123, the most common pediatric chronic conditions were chronic kidney disease 24 (19.5%), human immunodeficiency virus 14 (11.4%), prematurity 9 (7.3%), cerebral palsy 8 (6.5%), and asthma 8 (6.5%). Further comparison between children versus adolescent under nonsexual violence situation revealed significant difference between the hospital referral sites. The frequency of patients under violence referred from outpatient clinics was significantly reduced in children versus adolescents (27.7 vs. 62%), whereas emergency department was higher in the former group (57.4 vs. 25.0%; p<0.001). All types of violence situations and pediatric chronic conditions were similar in both groups (p>0.05). Conclusions: Nonsexual violence against our pediatric population was rarely diagnosed in a tertiary hospital, mainly negligence, physical, and psychological/emotional aggression. Approximately two-thirds of violence diagnosis occurred in children, referred mainly by the emergency department. In contrast, approximately one-third of violence diagnosis occurred in adolescents, referred mostly by outpatient clinics.
BACKGROUNDPolyarteritis nodosa (PAN) is a rare necrotizing vasculitis of medium and small size arteries. This primary vasculitis in pediatric populations may be triggered by infectious, mainly caused by Streptococcus. There are very few cases reported associated with tuberculosis, especially in adults. Therefore, we reported here a case of PAN possibly triggered after pulmonary tuberculosis.
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