Ocular involvement in a case of systemic indeterminate cell histiocytosis -: A case report

Purpose. To describe ocular involvement in a case of systemic indeterminate cell histiocytosis, a rare disease characterized by the proliferation of indistinct histiocytes. These histiocytes resemble Langerhans cells but lack Birbeck granules. The disease usually occurs in adults, with no predisposition for either sex. The clinical features include multiple asymptomatic nodules and cutaneous papules. There are no previous reported cases in the literature regarding ocular indeterminate cell histiocytosis and its treatment. Methods. A patient with skin papules and hyperpigmentation associated with systemic involvement and corneal infiltration was diagnosed with systemic indeterminate cell histiocytosis after a skin biopsy. Immunohistochemical examination showed specific surface proteins of indeterminate cells, and electron microscopy showed Langerhans-like cells without typical Birbeck granules. Progressive and severe photophobia and corneal thickening made bilateral corneal transplant necessary. Results. Visual acuity remained stable because of the use of systemic immunosuppressors, which decrease the recurrence of infiltration after a corneal transplant. Conclusion. Indeterminate cell histiocytosis is a rare disease and there is no previous description of ocular involvement. Infiltrating lesions must be treated with corneal transplant and systemic cyclosporin if they decrease visual acuity.