A Case of Orbital Langerhans Cell Histiocytosis in an Adult

Authors

  • Inês Carneiro Serviço de Oftalmologia. Centro Hospitalar Universitário do Porto. Porto.
  • António Friande Serviço de Oftalmologia. Centro Hospitalar Universitário do Porto. Porto.
  • Maria Araújo Serviço de Oftalmologia. Centro Hospitalar Universitário do Porto. Porto.

DOI:

https://doi.org/10.20344/amp.10179

Keywords:

Adult, Histiocytosis, Langerhans-Cell, Orbital Diseases

Abstract

Langerhans cell histiocytosis remains an enigmatic disease with a very heterogeneous presentation. We describe a rare case of orbital Langerhans cell histiocytosis in a 39-year-old female patient who presented right orbital pain and edema of the upper right eyelid. Surgery showed a friable lesion and underlying bone irregularity. Morphological aspects and immunohistochemical profile favored the diagnosis of Langerhans cell histiocytosis, which was confirmed with evidence of Langerin expression. The staging tests did not reveal any organ involvement, so we decided to follow the algorithm proposed by Euro Histio Net: in case of unifocal disease and in a single organ, clinical surveillance was preferred. This case aims to raise awareness of a manifestation of Langerhans cell histiocytosis, which should always be considered as a differential diagnosis in adults with osteolytic orbital lesions.

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Published

2019-09-02

How to Cite

1.
Carneiro I, Friande A, Araújo M. A Case of Orbital Langerhans Cell Histiocytosis in an Adult. Acta Med Port [Internet]. 2019 Sep. 2 [cited 2024 Oct. 6];32(9):617-20. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/10179

Issue

Section

Case Report