1/3. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/3. Intraocular histiocytosis in a 12-year-old girl without systemic disease.Isolated intraocular histiocytosis is a rare disease that may manifest by recurrent uveitis and solid subretinal masses. The course, diagnosis and treatment of isolated intraocular histiocytosis in a 12-year-old girl are presented. As extensive diagnosis and therapy with corticosteroids and tuberculostatics failed to produce satisfactory results, diagnostic-therapeutic vitrectomy was performed. The intraoperatively obtained material was examined by the methods of histopathology, cytology and immunocytochemistry, along with herpes and cytomegalovirus polymerase chain reaction. The vitreous inflammatory exudate and subretinal masses were operatively removed. Analysis of the intraoperatively obtained material pointed to histiocytosis, whereas additional examinations revealed no systemic manifestations of the disease. Chronic uveitides that respond poorly to classic immunosuppressive therapy require multidisciplinary analysis of intraocular material. Pars plana vitrectomy is an appropriate diagnostic-therapeutic operative procedure.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
3/3. Indeterminate cell histiocytosis: a rare histiocytic disorder.A 64-year-old woman, otherwise healthy, presented with multiple reddish-brown, slightly yellowish papules on the face and neck, which had developed 3 years earlier. The lesions were painless and nonpruritic and varied in diameter from 1 to 5 mm. Histological and immunohistochemical examination of cutaneous biopsies revealed a diffuse dermal infiltrate composed mainly of histiocytes which expressed both Langerhans cell as well as monocytic/macrophages cell marker characteristics. Electron microscopic studies revealed no Birbeck granules within the cytoplasm of the neoplastic cells, leading to a diagnosis of indeterminate cell histiocytosis. Indeterminate cell histiocytosis is a very rare disease characterized by the proliferation of indeterminate histiocytes which morphologically and immunophenotypically resemble langerhans cells but lack Birbeck granules.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |