scleromalacia perforans

Scleromalacia perforans is a rare and severe form of anterior necrotizing scleritis, characterized by the progressive thinning and necrosis of the scleral tissue. It typically presents as a blackish-blue hue visible through a thin sclera [3][5]. This condition can lead to significant ocular morbidity due to its insidious onset, slow progression, and lack of symptoms until the bare choroid is seen under the thin layer of the conjunctiva [14][15].

Scleromalacia perforans is often associated with autoimmune diseases, such as rheumatoid arthritis, and can affect both eyes. It is a painless form of necrotizing scleritis that has no obvious inflammatory signs [7]. The condition can ultimately result in ocular perforation, making it a serious and potentially sight-threatening disorder.

It's worth noting that scleromalacia perforans is a rare condition, accounting for only 4% of scleritis cases [8], and its exact cause remains unclear. However, it is often linked to underlying autoimmune diseases or systemic inflammation [1][4].

Scleromalacia Perforans Signs and Symptoms

Scleromalacia perforans, a rare form of necrotizing anterior scleritis, presents with distinct signs and symptoms. These include:

• Thinning of the sclera: The white part of the eye becomes thin and translucent, making it visible to the naked eye.
• Eye pain: Although some cases may be painless, others can cause severe eye pain, often accompanied by tenderness in the affected area.
• Blurred vision: As the sclera thins, it can lead to blurred vision or changes in visual acuity.
• Necrotic scleral plaques: Small areas of dead tissue (necrosis) may appear on the surface of the sclera, often near the limbus (the border between the cornea and sclera).
• Coalescence and enlargement of necrotic areas: Over time, these small areas can merge and grow larger.
• Scleral thinning and translucency: The affected area may become so thin that it becomes translucent, allowing the underlying dark uveal tissue to be visible.

Key Points

• Scleromalacia perforans is a rare form of necrotizing anterior scleritis without inflammation [3][9].
• It can occur in both painful and painless forms [10][13].
• The symptoms can vary widely, but often include eye pain, blurred vision, and changes in the appearance of the sclera [11][12].

References

[1] Scleromalacia perforans is a rare ocular manifestation of rheumatoid arthritis which can potentially lead to blindness and is a late consequence in the course... (Source: 4) [2] Scleromalacia perforans is a painless form of necrotizing scleritis that has no obvious inflammatory signs. (Source: 14) [3] A rare form of necrotizing anterior scleritis without pain can be called scleromalacia perforans. (Source: 9) [4] Scleral melting in a white, quiet eye is known as scleromalacia perforans. (Source: 10)

Scleromalacia perforans, a rare form of necrotizing anterior scleritis without pain, can be diagnosed through a combination of clinical evaluation and laboratory tests.

Clinical Evaluation

• A thorough eye examination by an ophthalmologist is essential to identify signs of scleral thinning and translucency [11].
• The absence of pain despite significant scleral changes can be a key diagnostic feature [11].

Imaging Studies

• Ultrasonography (A-scan and B-scan) is considered the most helpful test in diagnosing posterior scleritis, which may also be relevant for scleromalacia perforans [4].
• Computed tomography (CT) scans or ultrasounds may be ordered to evaluate posterior scleritis [7].

Laboratory Tests

• Complete blood count (CBC) with differential and erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) can help identify underlying autoimmune diseases [2].
• Laboratory tests are often necessary to evaluate any associated underlying connective tissue and autoimmune disease, such as rheumatoid arthritis, lupus, sero-negative spondylarthropathies, granulomatosis with polyangiitis, or Crohn's disease [10].

Other Diagnostic Tests

• A B-scan is considered the most helpful test in the diagnosis of posterior scleritis, which may also be relevant for scleromalacia perforans [14].
• Laboratory testing can help identify underlying autoimmune diseases that may be associated with scleromalacia perforans, such as rheumatoid arthritis or lupus [10].

It's worth noting that the diagnosis of scleromalacia perforans involves a comprehensive approach combining clinical evaluation, imaging studies, and laboratory tests.

Treatment Options for Scleromalacia Perforans

Scleromalacia perforans, a rare and serious eye condition, requires prompt and effective treatment to prevent further damage and vision loss. The primary goal of treatment is to control the underlying autoimmune condition, reduce inflammation, and prevent further scleral thinning.

Oral Non-Steroidal Anti-Inflammatory Drugs (NSAIDs)

• Oral NSAIDs are often the initial agent of choice for treating scleromalacia perforans [10].
• These medications include non-selective or selective cyclo-oxygenase inhibitors (COX inhibitors) that help reduce inflammation and pain.
• Examples of oral NSAIDs used to treat scleromalacia perforans include ibuprofen, naproxen, and celecoxib.

Immunosuppressive Therapy

• In some cases, immunosuppressive therapy may be necessary to control the underlying autoimmune condition [5].
• Medications such as cyclophosphamide, rituximab, and infliximab can be effective in treating scleromalacia perforans associated with rheumatoid arthritis.
• These medications target specific components of the immune system and can help reduce inflammation and prevent further damage.

Corticosteroids

• Corticosteroids remain a mainstay of short-term treatment for scleritis, including scleromalacia perforans [14].
• Mild to moderate scleral inflammation may respond well to treatment with topical corticosteroid or nonsteroidal anti-inflammatory drug (NSAID).

Surgical Treatment

• In some cases, surgical therapy may be required to address extensive scleral thinning and prevent globe rupture [6].
• Surgical treatment is usually reserved for patients who have perforation of the sclera or are at risk of perforation.

It's essential to note that each patient's response to treatment may vary, and a comprehensive treatment plan should be tailored to individual needs. Consultation with an eye specialist or rheumatologist is crucial in determining the best course of treatment for scleromalacia perforans.

References: [5] S Yangzes · 2019 · Cited by 9 — [10] Scleromalacia perforans consists of scleral melting in a white, quiet eye. ... Oral non-steroidal anti-inflammatory drugs (NSAIDs) are often the initial agent of choice for treating scleromalacia perforans. [14] Corticosteroids remain the mainstay of short-term treatment of scleritis; mild to moderate scleral inflammation may respond well to treatment with nonsteroidal antiinflammatory drug or topical corticosteroid.