Evaluation of Cheek Edema in an Infant Reveals Langerhans Cell Histiocytosis
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Published on: October 13, 2023
Asimakis D Asimakopoulos1, Eugene Panosetti1, Alexandra Papoudou-Bai2
1Department of Oto-Rhino-Laryngology & Head and Neck Surgery, Hospital Centre of Luxembourg, Luxembourg.
Insights
Langerhans cell histiocytosis (LCH) can manifest as isolated bone lesions in infants, presenting as facial swelling. Early diagnosis is crucial for effective management of this rare hematological disorder.
Area of Science:
- Pediatric Hematology
- Pediatric Oncology
- Dermatology
Background:
- Langerhans cell histiocytosis (LCH) is a rare hematological disorder.
- Skin rash is a common initial symptom, followed by bone involvement in approximately 50% of cases.
- Infantile LCH often presents with diverse clinical manifestations.
Observation:
- A 5-month-old infant presented with left hemifacial swelling.
- Initial treatment for presumed infection with antibiotics was ineffective.
- Persistent swelling and new fever prompted advanced imaging and biopsy.
Findings:
- Imaging revealed mandibular osteolysis, suggesting osteomyelitis or histiocytosis X.
- Tissue biopsy confirmed the diagnosis of Langerhans cell histiocytosis.
- The case highlights LCH presenting as an isolated bony lesion in infancy.
Implications:
- Langerhans cell histiocytosis should be considered in the differential diagnosis of infantile bone osteolysis.
- Prompt diagnosis and management are essential for favorable outcomes in pediatric LCH.
- This case underscores the varied presentations of LCH in young children.
Background:
Langerhans cell histiocytosis is a rare hematological disorder. Skin rash is the typical early feature, and bony involvement is the second most common presentation.
Methods:
We present a case of a 5-month-old female infant with left hemifacial swelling, initially treated for infection with antibiotics. However, due to persistence of swelling and new onset fever, further evaluation with ultrasonography, CT scan, FDG PET/CT and eventually biopsy was performed.
Results:
Imaging methods revealed mandibular osteolysis indicative of either osteomyelitis or histiocytosis X. Tissue biopsy was diagnostic for Langerhans cell histiocytosis.
Conclusion:
Langerhans cell histiocytosis may present in infancy with a variety of symptoms, included an isolated bony lesion. Langerhans cell histiocytosis, despite its rarity, should be included in the differentiated diagnosis, when bone osteolysis is found.
