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Título

GUARDIANS OF THE OCULAR SURFACE: LESSONS FROM A CHALLENGING CASE OF LANGERHANS CELL HISTIOCYTOSIS WITH EYELIDS INVOLVEMENT

Introdução

Langerhans Cell Histiocytosis (LCH) consists of a clonal proliferation of histiocytes. The systemic manifestations depend on the affected organ.(1) Ocular and orbital involvement is uncommon. Exclusive eyelid involvement is rare, with few cases described.(2,3) We report a rare case of LCH with eyelid involvement, which resulted in severe complications to the ocular surface and increased the morbidity of the disease.

Métodos

A 53-year-old Brazilian female presented a single solid nodule on the upper left eyelid margin with progressive growth in 2005, excised at the Department of Dermatology. The histopathological analysis of the specimen revealed histiocytes with eosinophilic cytoplasm, immunohistochemical positivity for S-100 protein, CD-1a, CD-68, CD-34 (figure 1), with a diagnostic conclusion of LCH. At that time, the patient denied other similar lesions on her body. In 2021, the patient was referred, for the first time, to the Department of Ophthalmology. She presented incomplete eyelid closure of the left eye (LE) with consequent exposure of the ocular surface, which, according to the patient's report, started after the diagnostic procedure of excising the eyelid nodule. Definitive tarsorrhaphy was performed. In July 2022, the patient had a corneal ulcer in her LE, resulting in corneal leukoma and visual impairment. Treatment was initiated in the LE with sodium hyaluronate 0,15% lubricant every 2 hours, combined with ophthalmic ointment containing 10,000 IU/g Retinol Acetate + 25mg/g Amino Acids + 5mg/g Methionine + 5mg/g Chloramphenicol 4 times a day. Eight months after the treatment, the patient experienced an improvement in LE signs and symptoms. However, she complained of a new onset of excessive tearing in the right eye (RE) and a new growing nodule in the right epicanthus, associated with multiple nodules throughout the body, indicating disease progression. Slit lamp examination of LE and RE is described respectively in figure 2 and 3. The treatment to RE was prescribed to improve protection of the ocular surface.

Resultados

Although the biopsy excision of the eyelid lesion was essential for defining the etiology, protecting the ocular surface is crucial and emphasizes the importance of multidisciplinary assistance in managing heterogeneous challenging cases.

Conclusões

Unveiling this case of LCH, we learned the eyelid’s key role in ocular surface triumph. Lastly, it is important for ophthalmologists to be aware of the presentation and management of LCH.

Palavras Chave

Langerhans Cell Histiocytosis; eyelids; ocular surface.

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