Posters

Herpes simplex keratitis and episcleritis in keratosis follicularis (Darier's disease)

Poster Details

First Author: M. Radia UNITED KINGDOM

Co Author(s):                        

Abstract Details

Purpose:

Keratosis Follicularis (Darier's disease) is an autosomal dominant dermatological disorder characterised by abnormal epidermal differentiation and loss of normal cell-to-cell adhesion. Darier's disease is rare (prevalence 2.7 in 100,000) and ocular sequelae have been reported in few cases: commonly dry eye and Sjogrens syndrome. This is the first report to describe a case of recurrent herpes simplex virus (HSV) keratitis and episcleritis in a 47 year-old man suffering from Darier's disease.

Setting:

Outpatient setting, Department of Ophthalmology, Queens Hospital, Romford, London, UK

Methods:

During the course of our contact with the patient, detailed clinical notes were kept. Informed consent for publication of the clinical case and photography in this journal was obtained for submission. PubMed was searched from February through July 2014 using the following search terms: 'Darier's disease,' and 'Ophthalmic conditions', 'Herpes Simplex Keratitis', 'Episcleritis.' Relevant manuscripts were retrieved and a literature review was carried out.

Results:

Darier's disease is caused by mutations in the ATP2A2 gene (12q23-24.1), which encodes the sarcoplasmic/endoplasmic reticulum calcium pump. Loss of function in this gene leads to impaired desmosome function, especially in ectodermal tissues. Clinically the condition rarely remits but can be controlled with oral Acitretin. Cutaneous herpetic infections with or without secondary bacterial/fungal overgrowth in Darier's as described here are widely reported and are thought to be opportunistic: the result of an epithelium weakened by poor desmosome function in combination with a mild inflammatory insult. A combination of weak cell-cell adhesion in a pro-inflammatory environment led to self-perpetuating inflammatory cycle in the episclera as on skin and cornea.

Conclusions:

The patient's condition predisposed him towards developing ocular complications due to several factors: impaired desmosome function leading to poor cell-to-cell adhesion in the corneal epithelium, dry eye and HSV invasion of inflamed periocular skin combining to allow viral colonisation of a poorly protected cornea.

Financial Disclosure:

NONE