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Published on: October 27, 2023
Isolated eyelid neonatal Langerhans cell histiocytosis
Abdelrahman M Elhusseiny1,2, Jamal O Azhari1, Tom Kornhauser1
1Department of Ophthalmology, Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.
Insights
This case report details an extremely rare instance of neonatal Langerhans cell histiocytosis (LCH) presenting as an isolated eyelid mass in an infant. Early diagnosis and treatment are crucial for this rare condition.
Area of Science:
- Pediatric Pathology
- Dermatology
- Ophthalmology
Background:
- Langerhans cell histiocytosis (LCH) involves clonal proliferation of bone marrow-derived phagocytic cells.
- Congenital/neonatal LCH is exceptionally rare, with few documented cases.
- Eyelid masses in infants can have diverse etiologies, including LCH.
Purpose of the Study:
- To report the first known case of neonatal LCH presenting as an isolated eyelid mass.
- To highlight the importance of considering LCH in the differential diagnosis of infantile eyelid lesions.
- To discuss the management of localized LCH in neonates.
Main Methods:
- Case presentation of a 3-week-old female infant with an eyelid swelling.
- Initial misdiagnosis as chalazion, followed by biopsy and histopathological confirmation of LCH.
- Staging work-up to rule out multisystem involvement and subsequent local steroid injection for treatment.
Main Results:
- Histopathology confirmed Langerhans cell histiocytosis (LCH) as the cause of the eyelid mass.
- The infant had localized disease without evidence of multisystem involvement.
- The lesion was treated with local steroid injection.
Conclusions:
- This is the first reported case of neonatal LCH presenting as an eyelid mass.
- Ophthalmologists should include LCH in the differential diagnosis for eyelid lesions in infants under one month.
- Prompt recognition and appropriate management are vital for rare neonatal presentations of LCH.
Abstract:
Langerhans cell histiocytosis (LCH) is a condition characterized by clonal proliferation of the phagocytic cells derived from the bone marrow. In this article, we present an exceedingly rare case of congenital/neonatal LCH in a 3-week-old girl who initially presented with an isolated swelling of the eyelid, initially misdiagnosed as a chalazion. Subsequently, a biopsy was performed, and histopathological evaluation confirmed the diagnosis of LCH. A staging work-up revealed no evidence of multisystem involvement, and thus, local steroid injection was performed as the initial treatment for the residual lesion. Cases of localized LCH that manifest as eyelid masses are rare, and most reported cases involve children over the age of one year. To the best of our knowledge, this case represents the first reported instance of neonatal LCH presenting as an eyelid mass. Although neonatal LCH is rare, ophthalmologists must be aware of this presentation and include it in the differential diagnosis for eyelid lesions in infants during the first month of life.
