1 - Unidade de Infecciologia, Área da Mulher, Adolescente e Criança, Hospital Dona Estefânia, CHLC
2 - Serviço de Cirurgia Pediátrica, Área da Mulher, Adolescente e Criança, Hospital Dona Estefânia, CHLC
- 36th Annual Meeting of the European Society for Paediatric Infectious Diseases, Malmo, Sweden, sob a forma de poster
Resumo:
Introdução: Mandibular mass with inflammatory signs presents a clinical conundrum: osteomyelitis, parotiditis, chronic recurrent multifocal osteomyelitis and tumoral masses must be considered. Timely and accurate diagnosis is mandatory to avoid unnecessary exams and to start appropriate therapy, thus improving prognosis.
Relato de caso: Ten-month-old boy with a 2-month history of painful progressive enlarging unilateral mandibular mass, with inflammatory signs, oral mucosae purulent discharge, and intermittent fever, with no improvement after two antibiotic cycles in the last month and a half (amoxicillin clavulanic acid, and flucloxacilin and clindamycin). The appearance of redish-violet papulo vesicular lesions, some with central erosion, were noted in the face, trunk and limbs in the previous month. Laboratory testing revealed: 9.8 g/dL hemoglobin, 19000 WBC (56% neutrophils), CRP 2 mg/dL, SR 26mm/h and lactate dehydrogenase 601 UI/L. Facial CT showed an expansive lesion on the right masseter muscle and hemi-mandible, with bone marrow invasion and cortical disruption (with signs of bacterial super infection). Antibiotherapy was changed to cefuroxime and clindamycin, with fever resolution. Bone biopsy revealed lympho histiocytic infiltrate (S100+, CD1a+, CD68+ andCD163+) and the mass was surgically excised; skin biopsy showed neoplastic cells S100+ and CD1a+, consistent with multisystem Langerhans cell histiocytosis (LCH). Bones cintigraphy revealed hyper captation focus on the eighth left rib, right frontal and temporal bones. Brain MRI and abdominal ultrasound were normal. The child is now under aprednison, vincristine and cytarabine protocol with good clinical evolution.
Conclusões: Although rare, LCH must be excluded when a child presents with a facial bone mass. The prognosis is worse in multisystem involvement and when diagnosis is made at an early age. Timely diagnosis is crucial for prompt therapy institution. Inflammatory/superinfection signs may be misleading and delay diagnosis.
Palavras Chave: Langerhans cell histiocytosis; mandibular mass