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

• Published in: Ocular immunology and inflammation Vol. 19; no. 5; pp. 337 - 339
• Main Authors: Parentin, Fulvio, Ventura, Giovanna, Pastore, Serena, Kiren, Valentina, Bibalo, Chiara, Pensiero, Stefano, Lepore, Loredana
• Format: Journal Article
• Language: English
• Published: England Informa Healthcare 01.10.2011
• 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
• ISSN: 0927-3948; 1744-5078
• DOI: 10.3109/09273948.2010.605538

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.