Ocular Involvement in a Case of Systemic Indeterminate Cell Histiocytosis

Abstract

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.

Unusual variants of non-Langerhans cell histiocytoses

“…15 A case of systemic ICH with ocular involvement, treated with polychemotherapy and corneal transplantation, has recently been observed but a long-lasting follow-up was lacking. 26 In addition to the cases mentioned above, chemotherapy regimens, including vinblastine and 2-chlorodeoxyadenosine, respectively, were necessary in two other cases with extensive and disfiguring cutaneous disease despite the absence of visceral involvement. 5,17,22 We observed two adult male patients with ICH having widespread cutaneous eruptions without evidence of systemic disease at onset.…”

“…Two clinical subtypes seem to predominate: a solitary nodular form [11][12][13][14]16 (Fig 1, A) and a multiple papulonodular form 3,5,6,[8][9][10]15,[17][18][19][20][21][22][23][24][25][26] (Fig 1, B). The former is usually characterized by a single, soft red asymptomatic nodule about 1 cm in diameter; the latter presents with a widespread eruption of multiple, firm asymptomatic lesions ranging in size from a few millimeters to 1 cm, varying in color from dark red to brownish and covered by intact skin.…”

“…On the other hand, 24 cases with widespread cutaneous lesions have been described, 3,5,6,[8][9][10]15,[17][18][19][20][21][22][23][24][25][26] the majority of which having an indolent or selflimited course; indeed, in a number of reported cases treatment and follow-up were not stated. 3 Table I.…”

“…However, several cases, including our two, 9 required chemotherapy regimens. 5,15,17,22,[24][25][26] …”

Effects of topical 0.2% Cyclosporine A on corneal neovascularization induced by xenologous amniotic membrane implantation into a corneal stroma micropocket of rats

“…It has been systemically used since 1979 in preventing transplant and for immunological diseases (Calne et al 1979). In ophthalmology, it is used specially for decreasing cases of rejection of corneal implant and transplant (Calatayud et al 2001). Since its systemic use has a high cost and results in several collateral effects, and its topical use leads to concentration levels in corneal epithelium higher than when it is used systemically, ophthalmologists began to use eye drops or ointments for the application of cyclosporine (Ponn et al 2001, Theng et al 2002.…”