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 i ndistinct histiocytes. These histiocytes resemble Langerhans cells but lack Birbeck granules. The disease usually occurs in adults, with no predisposi tion for either sex. The clinical features include multiple asymptomatic no dules and cutaneous papules. There are no previous reported cases in the li terature regarding ocular indeterminate cell histiocytosis and its treatmen t. Methods. A patient with skin papules and hyperpigmentation associated wi th systemic involvement and corneal infiltration was diagnosed with systemi c indeterminate cell histiocytosis after a skin biopsy. Immunohistochemical examination showed specific surface proteins of indeterminate cells, and e lectron microscopy showed Langerhans-like cells without typical Birbeck gra nules. Progressive and severe photophobia and corneal thickening made bilat eral corneal transplant necessary. Results. Visual acuity remained stable b ecause of the use of systemic immunosuppressors, which decrease the recurre nce of infiltration after a corneal transplant. Conclusion. Indeterminate c ell histiocytosis is a rare disease and there is no previous description of ocular involvement. Infiltrating lesions must be treated with corneal tran splant and systemic cyclosporin if they decrease visual acuity.