A rare case of solitary unifocal Langerhans cell histiocytosis with orbital extension: Diagnostic dilemma

Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case o...

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Published inOman journal of ophthalmology Vol. 11; no. 3; pp. 284 - 287
Main Authors Addenan, Maftuhim, May, Choo, Hooi, Teoh, Ismail, Fazliana, Kamalden, Tengku
Format Journal Article
LanguageEnglish
Published India Wolters Kluwer India Pvt. Ltd 01.09.2018
Medknow Publications & Media Pvt. Ltd
Medknow Publications & Media Pvt Ltd
Wolters Kluwer Medknow Publications
Subjects
Autoimmune diseases
Case Report
Chemotherapy
Diabetes
Langerhans cell histiocytosis
Leukemia
Metastasis
Neuroblastoma
Ophthalmology
orbit
Surveillance
uniforcal
Langerhans cell histiocytosis
uniforcal
orbit
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Summary:Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management.
ISSN:0974-620X
0974-7842
DOI:10.4103/ojo.OJO_149_2017