A Singular Case of Congenital Self-healing Histiocytosis with Skin, Liver and Atypical Eye Involvement

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 le...

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Bibliographic Details
Published in:Ocular immunology and inflammation 2011-10, Vol.19 (5), p.337-339
Main Authors: Parentin, Fulvio, Ventura, Giovanna, Pastore, Serena, Kiren, Valentina, Bibalo, Chiara, Pensiero, Stefano, Lepore, Loredana
Format: Article
Language:English
Subjects:
Antigens, CD1 - immunology
Convalescence
Female
Glaucoma - diagnosis
Glaucoma - etiology
Glaucoma - pathology
Glaucoma - surgery
Histiocytosis, Langerhans-Cell - complications
Histiocytosis, Langerhans-Cell - congenital
Histiocytosis, Langerhans-Cell - immunology
Humans
Infant
Intraocular Pressure - immunology
Iridocorneal Endothelial Syndrome - diagnosis
Iridocorneal Endothelial Syndrome - etiology
Iridocorneal Endothelial Syndrome - pathology
Iridocorneal Endothelial Syndrome - surgery
Liver Diseases - diagnostic imaging
Liver Diseases - etiology
Liver Diseases - immunology
S100 Proteins - immunology
Skin Diseases - etiology
Skin Diseases - immunology
Trabeculectomy
Ultrasonography
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Summary: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.
ISSN:0927-3948
1744-5078
DOI:10.3109/09273948.2010.605538