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Abstract #47727 Published in IGR 13-4
A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement
Parentin F; Ventura G; Pastore S; Kiren V; Bibalo C; Pensiero S; Lepore LOcular Immunology and Inflammation 2011; 19: 337-339
Purpose: To describe a rare case of congenital self-healing Langerhans cell histiocytosis (CSHLCH) presenting with atypical eye involvement. Design: Case report. Methods: A female newborn presented with purpuric lesions over the trunk, limbs, and face. Liver ultrasonography revealed hypoechogenic lesions with blurred borders. Biomicroscopy showed right posterior synechiae with fibrinoid deposits on the lens. At 7 months she presented with right acute glaucoma. Results: Biomicroscopy showed the presence of inflammatory pseudo-membrane covering the anterior surface of the lens, iris, and iridocorneal angle. Ab externo trabeculotomy was performed; access to the anterior chamber with capsulorrhexis forceps permitted a peeling of the pseudo-membrane with normalization of the intraocular pressure. Histologic examination of the membrane revealed an inflammatory tissue with CD1a and S-100 positive histiocytic cells. Conclusions: This is the first case of CSHLCH describing acute glaucoma secondary to a pseudo-inflammatory membrane with typical histiocytic cells, occluding the iridocorneal angle. (copyright) 2011 Informa Healthcare USA, Inc.
F. Parentin. Department of Surgery, Ophthalmology Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell'Istria, 65/1, I-34100 Trieste, Italy. Email: parentin@burlo.trieste.it
Classification:
9.4.15 Glaucoma in relation to systemic disease (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders)
