A singular case of congenital self-healing histiocytosis with skin, liver and atypical eye involvement

Fulvio Parentin, Giovanna Ventura, Serena Pastore, Valentina Kiren, Chiara Bibalo, Stefano Pensiero, Loredana Lepore

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)337-339
Number of pages3
JournalOcular Immunology and Inflammation
Volume19
Issue number5
DOIs
Publication statusPublished - Oct 2011

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Keywords

  • Eye
  • Glaucoma
  • Self-healing Langerhans cell histiocytosis

ASJC Scopus subject areas

  • Ophthalmology
  • Immunology and Allergy

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