TY - JOUR
T1 - A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement
AU - Parentin, Fulvio
AU - Ventura, Giovanna
AU - Pastore, Serena
AU - Kiren, Valentina
AU - Bibalo, Chiara
AU - Pensiero, Stefano
AU - Lepore, Loredana
PY - 2011/10
Y1 - 2011/10
N2 - 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.
AB - 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.
KW - Eye
KW - Glaucoma
KW - Self-healing Langerhans cell histiocytosis
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U2 - 10.3109/09273948.2010.605538
DO - 10.3109/09273948.2010.605538
M3 - Article
C2 - 21864117
AN - SCOPUS:80053636993
VL - 19
SP - 337
EP - 339
JO - Ocular Immunology and Inflammation
JF - Ocular Immunology and Inflammation
SN - 0927-3948
IS - 5
ER -