Unusual presentation of Rosai-Dorfman disease in a 14-month-old Italian child: a case report and review of the literature
| Autor: | Lorenzo Iughetti, Francesco di Dio, Elena Coccolini, I. Mariotti, Patrizia Bruzzi, Barbara Predieri |
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| Jazyk: | angličtina |
| Předmět: |
Pediatrics
medicine.medical_specialty Lymph node biopsy Lymphadenopathy Case Report Disease 03 medical and health sciences 0302 clinical medicine Biopsy medicine Humans Parotid Gland 030212 general & internal medicine Pediatrics Perinatology and Child Health Toddler Rosai–Dorfman disease Histiocyte Parotid swelling Histiocytosis Rosai-Dorfman disease Steroid therapy medicine.diagnostic_test business.industry Infant medicine.disease 030220 oncology & carcinogenesis Pediatrics Perinatology and Child Health Differential diagnosis Histiocytosis Sinus business |
| Zdroj: | BMC Pediatrics |
| ISSN: | 1471-2431 |
| DOI: | 10.1186/s12887-016-0595-9 |
| Popis: | Background Rosai-Dorfman disease (RDD) is a rare form of histiocytosis characterized by histiocyte proliferation within lymph nodes and extranodal tissue. Here we report an unusual presentation of RDD in an Italian toddler. Moreover, we reviewed the pediatric case reports published between 2004 and 2014, focusing in particular on medical therapy. Case presentation We report the case of a 14-month-old child who developed a progressive swelling of the right parotid, associated with systemic symptoms and abnormal blood tests. During diagnostic work-up, cervical, intraparotid, and unilateral hilar lymphadenopathies were found. Histopathological and immunohistochemistry studies of a cervical lymph node biopsy established the diagnosis of RDD, with positive PCR for Epstein - Barr virus on the biopsy specimen. Oral steroid therapy was started with progressive reduction in size of all lesions, resolution of systemic symptoms, and normalization of blood tests. Conclusion RDD is generally considered a benign and self-limiting form of histiocytosis, usually associated with favorable prognosis. However, complications are not infrequent and fatal cases were reported even in children. Efforts should be made to establish the best therapeutic strategy for this disease, as no well-defined guidelines exist. Finally, RDD should be included in differential diagnosis of lymphadenopathy and parotid swelling even in very young children. Electronic supplementary material The online version of this article (doi:10.1186/s12887-016-0595-9) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
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