1. Infectious Diseases Unit, Medical Pediatrics Department, Women’s, Children’s and Adolescent’s Division, Dona Estefânia Hospital, Central Lisbon Hospital Center, Lisbon, Portugal
2. Surgery Unit, Women’s, Children’s and Adolescent’s Division, Dona Estefânia Hospital, Central Lisbon Hospital Center, Lisbon, Portugal
- Publicado Portuguese Journal of Pediatrics Vol. 50 No. 2 (2019)
Background: 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.
Case Presentation Summary: 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 multiple antibiotic cycles including amoxicillin clavulanic acid, flucloxacilin and clindamycin, and cefuroxime and clindamycin.The appearance of redish-violet papulovesicular 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 white cells (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 hemimandible, with bone marrow invasion and cortical disruption (com sinais de sobreinfecção bacteriana). Bone biopsy revealed linfohistiocytic infiltrate (S100+, CD1a+, CD68+ and CD163+) and the mass was surgically excised and the skin biopsy showed neoplastic cells S100+ and CD1a+, consistent with multyssistem Langerhans cell histiocitosis. Bone scintigraphy revealed hypercaptation focus on the eighth left rib, right frontal and temporal bones. Brain MRI and abdominal ultrasound were normal. The child is now under a prednisolone, vincrisitine and cytarabine protocol with good clinical evolution.
Learning points/discussion: Although rare, Langerhans cell histiocytosis 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. Inflamatory/superinfection signs may be misleading and delay diagnosis.
Palavras Chave: histiocytosis, langerhans cells, mandibular mass