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TitreSubconjunctival epidermoid cysts in Gorlin-Goltz syndrome
ButTo describe a patient with Gorlin-Goltz syndrome with subconjunctival cysts. Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is an autosomal dominant disorder characterized by a wide range of developmental abnormalities and a predisposition to neoplasms.
MéthodesA 24-year old woman with confirmed Gorlin-Goltz syndrome presented with subconjunctival cysts in both upper eyelids. These were removed under local anesthesia and histopathological examination was performed.
RésultatsSlit-lamp examination showed bilateral subconjunctival light-yellow cysts in the palpebral conjunctiva of the upper eyelids. Anterior segment examination and fundoscopy were normal. BVCAs were 10/10. Histological examination of both upper eyelid biopsy specimen revealed multiple epidermoid cysts filled with keratic debris.
Our patient is known with de novo Gorlin-Goltz syndrome, confirmed by molecular analysis. She has multiple skin naevi and fibroma (cardiac, overian and tongue). Different odontogenic keratocysts of the mandible were surgically removed that showed epidermoid cysts with keratosis, similar to the subconjunctival cysts.
ConclusionSubconjunctival epidermoid cysts are an ocular feature of Gorlin-Goltz syndrome. Until now, only three patients have been described with this ocular sign. Histopathological examination of the subconjunctival cysts showed similarities with the jaw keratocysts found in our patient.
Auteur 1
NomDE CRAENE
InitialesS
InstitutDept Ophthalmology, Ghent Univ Hosp
VilleGhent
Auteur 2
NomBATTEAUW
InitialesA
InstitutDept Dermatology, Ghent Univ Hosp
VilleGhent
Auteur 3
NomHASPESLAGH
InitialesM
InstitutDept Dermatology, Ghent Univ Hosp
VilleGhent
Auteur 4
NomDECOCK
InitialesC
InstitutDept Ophthalmology, Ghent Univ Hosp
VilleGhent
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