Floppy Eyelid Syndrome.

Sir,

Floppy eyelid syndrome (FES) is a relatively under-recognized entity causing lax eyelid, chronic papillary conjunctivitis, and is frequently associated with obstructive sleep apnea (OSA), obesity, hypertension, diabetes, hyperlipidemia, and ischemic heart disease.[1] FES has been associated with pachydermoperiostosis and neurofibromatosis type 1.[2] The prevalence of FES ranges from 3.8% to 15.8% with a male preponderance and is mostly seen in middle-aged obese patients.[1] However, FES can go unrecognized, and thus the prevalence is likely underestimated. Herein, we report a case of FES with OSA.

A 51-year-old man presented with redness of both eyes and laxity of the surrounding skin that had gradually developed over the past 20 years. No previous history of recurrent eyelid edema was present. On examination, the skin over the lateral forehead and upper eyelids was velvety, hyperpigmented, and rugose with grossly visible coarse wrinkling [Figure 1]. Photophobia was apparent, and the upper eyelids could be everted easily revealing intense bulbar conjunctival hyperemia and linear papillary hypertrophy of the palpebral conjunctiva of the upper eyelid. The cornea revealed no abnormalities. He weighed 58 kg, had a height of 147 cm (body mass index: 26.8 kg/m2), and did not have any other craniofacial abnormalities.

Figure 1

Clinical image of the index patient: velvety, hyperpigmented skin with grossly visible coarse wrinkling over the lateral forehead and both upper eyelids with decreased eye opening

On further eliciting the history, he complained of mild daytime sleepiness. His Epworth Sleepiness Scale score[3] was 2 and the STOP–Bang questionnaire[4] score was 2 (suggesting a slow risk of OSA). In-laboratory full-night polysomnography was performed and severe OSA (apnea–hypopnea index of 43.5/h) was confirmed. Thyroid function tests were normal. Magnetic resonance imaging (MRI) of the brain revealed mild diffuse cerebral atrophy. Skin biopsy from the upper eyelid showed thinned-out epidermis and mild fibrosis and lymphocytic infiltrate in the dermis [Figure 2a]. Verhoeff–Van Gieson staining revealed a marked reduction in the content of the elastic tissue with fragmented elastic fibers [Figure 2b], consistent with a diagnosis of blepharochalasis. A diagnosis of floppy eyelid syndrome (FES) with OSA was ascertained. Lifestyle modifications, sleep hygiene, and weight reduction were suggested. He was also initiated on nocturnal continuous positive airway pressure (CPAP) therapy for his OSA at a pressure of 10 cm of H2O.

Figure 2

Histopathology showing thinned out epidermis and mild fibrosis and lymphocytic infiltrate in the dermis (a) (hematoxylin and eosin, 200×); marked reduction in the content of the elastic tissue (arrows) with fragmented elastic fibers (b) (Verhoeff–Van Gieson stain, 400×)

FES is characterized by lax eyelid skin (akin to blepharochalasis) easily evertible upper eyelids, and reactive conjunctival changes, i.e., papillary conjunctivitis and photophobia. FES is frequently associated with ocular and systemic diseases, notably keratoconus and OSA.[1] Various theories have been proposed regarding the pathogenesis of FES[1], such as mechanical theory (eversion of the eyelid during sleep and inflammation due to irritation of ocular surface), local ischemia, and reperfusion leading to oxidative stress and damage to the connective tissue.

All patients with FES should be evaluated for the presence of OSA, a history of snoring, daytime sleepiness and dizziness, nocturnal awakenings, headaches, uncontrolled hypertension, and family history of OSA. Ocular features of FES include blepharitis, ectropion, lid ptosis, tear film deficiency, punctate epithelial erosions, corneal scarring, ulcer, and perforation; and should be adequately evaluated.[5]

Management includes conservative (artificial tears, eyelid taping, and use of eye shields) and surgical treatment of lax eyelid to maintain proper positioning of the eyelid against the globe, which further reduces trauma and exposure to keratopathy.[1] Associated OSA should be managed by CPAP therapy. The awareness among dermatologists about FES is limited. The index case highlights a rare, but an important association of blepharochalasis with papillary conjunctivitis and OSA.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.