Blepharochalasis

Anil Kumar¹ , GA Surwade² , US Khaire² , Reshakiran Shende²

¹ Department of Medicine, Government Medical College, Aurangabad, Maharashtra, India
² Department of Biochemistry, Government Medical College, Aurangabad, Maharashtra, India

Correspondence Address:
Anil Kumar
Department of Medicine, Govt. Medical College, Aurangabad - 431001
India

Introduction

Blepharochalasis is a rare degenerative disease unique to the skin of the lids, clinically characterised by primary bilateral swelling followed by progressive loss of subcutaneous tissue resulting in fine wrinkling and the skin of the upper lid hangs in thin folds.[1] It is also termed ptosis atonica, ptosis adiposa and dermatolysis palpebrum. However the term blepharochalasis (Greek meaning eye lid relaxation) coined by Fuchs in 1896 seems to be most appropriate.[2] The condition starts with recurrent attacks of bilateral lid oedema in the pubertal age, the skin of the upper lid becomes atrophic, discoloured and redundant with tortuous blood vessels. The patient often presents for ptosis due to stretching of the levator palpebrae superioris muscle. Not more than half a dozen cases of blepharochalasis are reported in Indian literature. We report two cases with followup for 5 years.

Case Report

Case 1: An 18-year-old female was referred as a case of recurrent angioedema of upper lids. The patient was alright upto the age of 13 years when she developed bilateral lid oedema which subsided without any treatment. Upto the age of sixteen years she had several attacks of lid oedema at the interval of 5 to 8 months which persisted for two to three days each time. For the last 3 years she had not experienced lid oedema but developed difficulty in opening the eyes completely. Her systemic examination was normal. There was nontoxic puberty goitre but there was no lip oedema. Local examination revealed lax and wrinkled skin of both the upper lids. The redundant skin folds extended over the margins of the lids, tortuous blood vessels were seen in the atrophic skin. Skin on the body was normal. The family history was noncontributory to her illness. Her haematological profile, urinalysis, kidney function, liver function and thyroid function tests were within normal limits. [Figure - 1] The patient was subjected to cosmetic blepharoplasty. The skin tissue obtained during surgical procedure was subjected to histopathological examination. Histopathologically there were scattered and fragmented elastic fibers and also proliferation of capillary endothelium with mild lymphocytic infiltration in dermis and subcutaneous tissue. After discharge the patient was followed-up for five years which revealed no progression in the laxity of the skin.

Case 2: A 25-year-old female presented with progressive laxity and wrinkling of the upper eye lids of 5 years duration. The patient attained menarche at the age of 13 years and since then during her premenstrual phase she noticed intermittent lid oedema which continued till the age of 20 years. On several occasions she underwent haematological investigations, urinalysis and kidney function tests and endocrinal assay, the results of which revealed no abnormality. For last five years, she had no lid oedema but the eye lid skin became lax and wrinkled resulting in difficulty in opening the eyes completely. Her systemic examination was normal. The body skin revealed no abnormality. Routine haematological investigations, urinalysis, kidney function and liver function tests were within the normal limits. With the clinical diagnosis of blepharochalasis, the patient was referred for cosmetic surgery. Histopathology of skin tissue obtained revealed diminished depth of rete pegs and loss of elastic fibres at most of the places. There was proliferation of capillary endothelium, which substantiated the diagnosis of blepharochalasis. The patient was seen after 2 and 5 years of initial presentation which revealed no progression in the laxity of lids.

Discussion

The exact etiology of blepharochalasis is not known. However, the condition being commonly reported during puberty, the role of endocrines is difficult to rule out.[3] In one third of the cases in which the disease starts before the age of 10 years the autosomal dominant heredity probably plays an important role.[1] It however, remains unexplained why the skin of the upper lid is selectively involved. Three stages of blepharochalasis are described.[1] One the intermittent oedematous swelling of upper lids which is usually misinterp, eted until the permanent changes occur. The lid swelling is painless and transient which lasts for a day or two with mild redness of skin resembling angioedema. This state is called the oedema stage. In the second stage the skin becomes discoloured, reddish brown, heavily venuled and flabby and hangs over the lashes and the power to lift the eye lid is decreased. This is the stage of atonicptosis. In the third stage with further progression there is relaxation of the tissues of orbital septum and when the orbital fat drops into the relaxed lid weighing down the skin in full heavy transverse folds, narrowing of palpebral tissue occurs which results in interference with vision. The lacrimal gland may be pulled below the orbital margin giving the face tired debauchery. This is the advanced stage termed as ptosis adiposa. Most of the reported cases presented in the second stage. Though the upper lids are commonly involved, lower lid[4] and unilateral involvement[2] may be seen.

The disorder can be diagnosed with the characteristic history and typical skin lesions.[3],[4],[5]

The redundant eye lids narrow the palpebral fissure interfering with vision, in some patients corneal ulceration may result due to inturning of eye lashes.[6] The only effective treatment is correction of the deformities by plastic surgery, however, subsequent attacks of lid oedema may interfere with surgical repair.[7]

In both the cases presented, the diagnosis of blepharochalasis was made clinically and was substantiated by histopathological examination. Nontoxic thyroid goitre in the absence of lip edema could not be typed as (Leffer) Asher syndrome in one of our cases. Since there was no recurrence of lid oedema the surgery was fruitful in restoring and maintaining the young look in our patients.

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