Blepharochalasis is a rare disorder that typically affects upper lids. It is characterised by intermittent oedema of the lids, with frequent recurrence. It produces eyelid tissue relaxation and latter atrophy. It may be unilateral in some cases. In addition to recurrent attacks of oedema, it is associated with ‘cigarette-paper’ skin and subcutaneous telangiectasia.
The term blepharochalasis was coined by Fuchs in1896. The term blepharochalasis is frequently misused to indicate redundant skin of the ageing eyelid, a common condition more accurately termed as dermatochalasis.
The word blepharochalasis originates from the Greek word blepharon meaning eyelid and chalasis meaning relaxing.
References
Biswas Arnab. Ptosis Surgery. Jaypee Brothers Medical Publishers (P) Ltd 2010. P 102.
Yanoff Myron, Sassani Joseph W. Ocular Pathology Seventh Edition. Elsevier Saunders 2015. P 155.
http://emedicine.medscape.com/article/1214014-overview
http://eyewiki.aao.org/Blepharochalasis_Syndrome
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1043545/pdf/brjopthal00212-0013.pdf
Fuchs E. Über Blepharochalasis (Erschlaffung der Lidhaut). Wien Klin Wschr 1896; 9: 109- 110.
Patient may present with symptoms like
Eyelid changes are exacerbated by normal process of ageing.
Occasionally, attacks are aggravated by a triggering event such as fever, weeping, upper respiratory tract infection.
Blepharophimosis may be a form of chronic angioedema with localised vascular dilatation and extravasation of proteinaceous fluid. Orbital fat may have increased vascularity and dilated capillaries.
Triggers:
Multiple triggers may be the factors including
Pathogenesis
Pathogenesis is uncertain, but this condition may be immunogenic in origin. There is abundance of IgA deposits around elastin fibers. Infrequently there may be familial occurrence (autosomal dominant inheritance).
Associations
Sometimes blepharochalasis is associated with systemic illnesses such as
Rarely, blepharochalasis may be associated with agenesis of the kidney, vertebral abnormalities, and congenital heart defects.
Pathology
Pathology typically shows loss of elastic fibers, Lymphoedema, vasculitis and epithelial atrophy. Stretching of the aponeurotic fibers of levator palpebrae superioris muscle due to recurrent low grade inflammation produces aponeurotic ptosis (drooping) of the lids.
Diagnosis depends upon history of disease and clinical examination. There are no characteristic laboratory findings.
I. History:
Patient gives history of repeated episodes of painless swelling of one or both eyelids with subsequent thinning of skin, usually affecting people between the ages of 10-20 years. Oedema is initially seen frequently in upper lids. The frequency of attacks is variable. It may be associated with a preceding period of physical or emotional stress. Occasionally, there may be history of allergy.
II. Clinical features:
Patient shows clinical features such as lid or conjunctival oedema, proptosis, ptosis, telangiectasia of vessels, ectropion, entropion, skin parchment, or pseudoepicanthal folds. The skin hangs down over the upper eyelid.
The disease may be separated into
A. Early phase
It may be further divided as
B. Late phase.
Differential diagnosis
Nothing much can be done to ameliorate acute phase of blepharochalasis. Avoidance of triggering factors may diminish the frequency of attacks.
Medical therapy:
Supportive medical therapy may be provided in acute and active phase of the disease.
No pharmacologic agents have proven benefits in the treatment of blepharochalasis. The ability of antihistamines, sympathomimetics, steroids, mast cell stabilisers, and cold compresses to improve symptoms of acute attacks has not been determined.
Surgical therapy:
Surgical therapy may be required in quiescent and late stages of the disease.
Surgery is performed after at least six months of disease inactivity. This is performed to correct anatomical defects being produced due to repeated attacks of lid swelling.
Corrective procedures include
Prognosis
The initial onset is usually before the age of 20 years, with unilateral or bilateral swelling of the eyelids and conjunctiva which lasts for hours to several days.
Proptosis indicates orbital involvement. The frequency and severity of repeated episodes tend to lessen with increasing age.
However, unexpected episodes may occur after an extended period of inactivity.
Complications and sequelaes may be
