HomeHealth articlesblepharochalasis syndromeWhat Is Blepharochalasis Syndrome?

Blepharochalasis Syndrome - Causes, Symptoms, Diagnosis, Treatment, and Complications

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Blepharochalasis refers to a rare, painless, and recurring swelling of the eyelids. Read the article to know more in detail.

Medically reviewed by

Dr. Asha Juliet Barboza

Published At March 24, 2023
Reviewed AtApril 21, 2023

Introduction

G.J. Beer was the first to describe blepharochalasis syndrome in an 1817 textbook, and Ernst Fuchs named it in 1896. Most of the current knowledge about the epidemiology of this disorder comes from published case reports. According to a 2009 review, the average age of onset is about 11 years old, children before puberty. This condition is thought to affect males and females equally; however, more cases have been identified in females to date.

What Is Blepharochalasis Syndrome?

Blepharochalasis is a rare inflammatory condition that causes recurring painless swelling of the eyelids (edema). There may also be redundant eyelid skin folds. Changes in the position of the eyelids and pigmentary skin changes are also possible. Blepharochalasis most commonly affects the upper eyelids. Most people have bilateral blepharochalasis, which affects both eyes at the same time. Unilateral blepharochalasis is the swelling of only one eyelid during an episode, which is less common, but it does occur in some cases. Blepharochalasis patients have episodes every few months. Typically, episodes come and go (recur) for a few years before entering a quiescent stage (dormant condition). Each episode typically lasts a few days. It usually appears in childhood or adolescence.

Experts are unsure about the causes of blepharochalasis. While blepharochalasis will not impair vision, the swelling may cause the eyelid to block a part of the vision and reduce peripheral vision while experiencing an episode. Even if it does not harm the eyes, the skin and tissue on the eyelids can become permanently stretched out. This results in atrophy and thinning. This can be caused by swelling and shrinking during and after an episode of blepharochalasis.

What Causes Blepharochalasis Syndrome?

Blepharochalasis has an unknown cause. Some doctors believe that issues with the body's immune system may be the reason. However, it is also possible that the condition may be genetic (runs in families).

There are several theories about the etiology of blepharochalasis. Unlike dermatochalasis, which is an involutional change in the skin of the eyelids, blepharochalasis is assumed to be a manifestation of angioneurotic edema. In addition, hormonal changes and puberty may play a role in disease modulation. Bee stings, physical activity, crying, wind exposure, and stressful events have all been reported as triggers for attacks. However, in the majority of cases, no trigger can be identified.

What Are the Symptoms of Blepharochalasis Syndrome?

Inflammation of the upper eyelids is the most common symptom of blepharochalasis. During an episode, most people will experience swelling for a few days. The affected skin does not leave an indentation when poked (called non-pitting edema). These reoccurring attacks of exacerbation followed by remission can have an impact on the skin of the eyelids, altering their appearance.

In addition, blepharochalasis can cause the following signs and symptoms:

  • Thin and Wrinkled Skin - Multiple edema attacks can cause eyelid skin to become so stretched that it resembles wrinkled parchment paper.

  • Bagginess - As a result of repeated stretching, the eyelid's skin gradually loses shape and effectiveness (atrophy), leading to redundant skin folds.

  • Discolored or Spider Veins - As edema attacks recur, eyelid pigmentation can become reddish-brown or bronze-colored, with visible thread-like red lines (telangiectasia).

  • Absence of Pain - Unlike inflammation-related swelling, this type of eyelid swelling is not painful.

  • Obscured Vision- Blepharochalasis does not impair vision or affect any parts of the eye that aid in vision. The swelling can impair vision by making the upper eyelids feel heavy and the lower eyelids become abnormally positioned. Furthermore, the lax skin caused by repeated attacks can cause upper eyelids to hang over the edge of the eye, restricting or obscuring fields of vision.

  • Eye Irritations - The lower eyelids can fold inward (entropion) over time, causing eyelashes to rub on the eyeball or outward (ectropion), impacting tear drainage.

As they have a similar appearance, swollen eyelids associated with blepharochalasis are frequently confused with puffy eyelids. However, swelling is most likely temporary with blepharochalasis, whereas puffy eyelids may be permanent. Eyelid bagginess or atrophy caused by blepharochalasis can be confused with ptosis. The eye muscle responsible for opening the upper eyelid is weak or paralyzed in ptosis, which causes drooping. Ptosis can occur with other conditions, such as blepharochalasis, dermatochalasis, or blepharitis.

How Is Blepharochalasis Diagnosed?

An eye care specialist will perform an eye exam to diagnose blepharochalasis. They will examine the eyelids and eyes (including the inner portion). A blood test may be required to rule out infections. Before diagnosing blepharochalasis, an eye care specialist will usually rule out other conditions that cause swelling in your eyelids. This is known as a differential diagnosis. Some conditions that have symptoms similar to blepharochalasis include:

What Is the Treatment for Blepharochalasis?

The majority of people do not require treatment for blepharochalasis. It is uncommon and causes no harm or pain to the eyes, an eye care specialist will focus on managing the swelling when one has an episode. They may advise taking anti-inflammatory medications. Some medications used to treat blepharochalasis swelling include:

  • Corticosteroids.

  • Over-the-counter (OTC) non-steroidal anti-inflammatory drugs (NSAIDs).

  • Antihistamines.

  • Immunosuppressants.

If the skin on the eyelids is permanently damaged or stretched out due to swelling, it may require eyelid surgery (blepharoplasty).

What Are the Complications of Blepharochalasis?

Complications of blepharochalasis include ptosis, difficulty closing the eyelids completely, which causes dryness, and chronic inflammation of the eyelids. Chronic headaches, forehead wrinkles, and an aged appearance may occur in severe cases. Furthermore, autoimmune diseases have been linked to blepharochalasis, and patients with this condition are more likely to develop thyroid eye disease, lupus, and rheumatoid arthritis. To avoid these complications, Blepharochalasis must be diagnosed and treated as soon as possible.

Conclusion

Blepharochalasis is not painful and will not impair vision. Most people experience flare-ups of inflammation every few months for years. It usually does not require any treatment. Some people require surgery to repair the skin on their eyelids if blepharochalasis causes them to stretch out and droop. Even though blepharochalasis is rarely dangerous, an eye doctor must be consulted as soon as any swelling is noticed in or around the eyes. Other conditions with similar symptoms can spread to or harm the eyes if not treated promptly. The sooner a new swelling or discoloration is examined, and managed, less likely is the complications.

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Dr. Asha Juliet Barboza
Dr. Asha Juliet Barboza

Ophthalmology (Eye Care)

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