title: Unusual protrusion of conjunctiva in two neonates with harlequin ichthyosis.
abstract: BACKGROUND
  We present two patients who developed severe protrusion of the conjunctiva and chemosis secondary to Harlequin ichthyosis (HI).
  
  
  METHODS
  Case 1 was a male infant diagnosed with HI who had parchment-like appearance and conjunctival protrusion with severe chemosis. Case 2 was a female infant on whom HI had been suspected before birth through ultrasonography. She showed thickened skin over the entire body and conjunctival protrusion with severe chemosis. For both cases, a vitamin A derivative was applied and the hyperkeratotic layer was peeled off every day. Great care was taken to sterilize and moisten the ocular surface. The conjunctival protrusion gradually improved and other systemic conditions were successfully treated. HI is a rare condition, but affected infants are surviving longer than previously and hence guidelines for ocular management are now required.
  
  
  CONCLUSIONS
  Gentle and patient debridement of the hyperkeratotic skin and moisturizing were important in treating the unusual conjunctival protrusion.
authors.affiliation: Department of Ophthalmology, Oita University Faculty of Medicine, Hasama-machi, Yufu-shi, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Tomonobu
authors.initials: T
authors.lastname: Hazuku
authors.forename: Kisaburo
authors.initials: K
authors.lastname: Yamada
authors.forename: Masamoto
authors.initials: M
authors.lastname: Imaizumi
authors.forename: Toru
authors.initials: T
authors.lastname: Ikebe
authors.forename: Kei
authors.initials: K
authors.lastname: Shinoda
authors.forename: Kazuo
authors.initials: K
authors.lastname: Nakatsuka
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Sekiguchi
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Izumi
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Nishida