Unusual Manifestations of Orbital Langerhans Cell Histiocytosis: Conjunctival Hyperemia and Ocular Hypertonia

Author(s): Lina Boualila, Adam Tagmouti, Basma Mrini, Nouredine Boutimzine, Lalla Ouafae Cherkaoui, Hafsa Elouazzani, Nadia Cherradi, Rania Bouanane, Hamza Sbai, Firdaous Touarsa

Langerhans cell histiocytosis (LCH) is a rare disease defined by the pathological proliferation of the monocyte-macrophage lineage and dendritic cells. Orbital involvement typically manifests as a solitary lesion that carries a favorable prognosis. We describe the clinical and histological profile of an orbital LCH case. The patient exhibited unifocal unisystem disease. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and CD68 immunohistochemistry. We reviewed the different ophthalmic manifestations of LCH and treatment strategies. As LCH may solely involve the orbit, treatment is based on the degree of organ involvement. LCH should be included in the differential diagnosis of tumors of the ocular adnexa, especially in young children.

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