Ocular Involvement in the Histiocytoses: A Literature Review with an Illustrative Case Series

ABSTRACT

Purpose

To describe and illustrate recent reclassification and clinical descriptions of the histiocytoses, diagnosis and management, and effects on the eye and adnexae

Patients and methods

Literature review with an illustrative case series of seven patients with histiocytosis and ophthalmic involvement from a single tertiary centre.

Results

Skin lesions with signs including eyelid mass, orbital bone destruction, bizarre keratic precipitates, limbal or iris mass, haemorrhagic anterior uveitis, subretinal lesions of uncertain etiology, ischaemic retinopathy, optic neuropathy, and apparent steroid-resistant inflammations should be comprehensively investigated.

Conclusions

Ophthalmic involvement in histiocytosis is rare. Clinical manifestations vary from limited single-organ disease to lethal systemic infiltration or malignancy. Radiology may identify lesions for biopsy. Histology with appropriate markers is essential. Oncology advice may be required.

KEYWORDS:

• Histiocytosis
• Xanthogranuloma
• Langerhans
• Ocular

Acknowledgments

This research was facilitated by the Greater Manchester Local Clinical Research Network. We are grateful to Drs L McWilliam and L Irion for assistance with histology images.

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the article.