Langerhans Cell proliferation Presenting as Multiple Eyelid Lesions: A Case Report and Literature Review
Author: Munazzah Chou
Base Hospital / Institution: Moorfields Eye Hospital
ePoster presentation
Abstract ID: 25-575
Purpose
This report describes an unusual presentation of Langerhans Cell proliferation involving the eyelids. The case highlights the importance of systemic evaluation with PET/CT and genetic and molecular tests such as BRAF mutation to help exclude malignancy.
Methods
A 33-year-old woman presented with multiple bilateral asymptomatic upper eyelid margin lesions (Image), which developed sequentially over 12-months. The patient was systemically well, with no prior history of systemic disorders. Small violaceous skin papules were also noted on the right abdomen and lower back. Shave excision biopsy was performed on all eyelid lesions.
Results
The morphological histopathological appearances and immunoprofile were highly suggestive of Langerhans Cell histiocytosis (image) and positive for CD1a and S100 with prominent epidermal and dermal Langerhans cells. She was referred to a multidisciplinary team for systemic evaluation, including a PET scan excluding other FDG avid enlarged nodal, visceral or osseus disease. BRAF V600E variant using PCR assay on abdominal and back skin lesions were negative concluding a reactive proliferation of Langherhans cells and excluding a malignant or histiocytic disorder.
Conclusion
Histiocytoses are a diverse group of rare disorders characterised by the pathological infiltration of tissue by myeloid cells. Langerhan cell histiocytosis (LCH) is most common type of histiocytosis. Presentation is variable. Systemic disease often involves bone (80%), skin (33%), pituitary, lymph nodes, and less commonly CNS, liver, spleen, and lungs. Localised lesions respond well to biopsy, curettage, and intralesional steroids. Systemic corticosteroids and chemotherapy are used in systemic disease, and MAPK inhibitors such as vemurafenib in refractory cases. Prognosis depends on age and extent of disease, with ~90% 5-year survival. Poor prognosis is linked to liver, spleen, lung, or CNS involvement, and neurodegenerative forms.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Parinaz | Shams | Moorfields Eye Hospital |
