Scleromalacia perforans is best classified as which form of scleritis?

Anterior necrotizing scleritis without inflammation Scleromalacia perforans is the necrotizing anterior scleritis that occurs without inflammatory signs, which is why the eye can be quiet despite progressive scleral destruction.

Posterior scleritis with exudative detachment

Which immune mechanism underlies the vascular damage of scleromalacia perforans?

Type III immune complex hypersensitivity The disorder results from autoimmune type III hypersensitivity damage to the episcleral and scleral perforating vessels, which is why immunosuppression is central to treatment.

Type I IgE-mediated hypersensitivity

Type II antibody-mediated cytotoxicity

Type IV delayed cellular hypersensitivity

What proportion of all scleritis cases is accounted for by scleromalacia perforans?

4% Scleromalacia perforans is rare, making up only about 4% of scleritis cases.

How does scleromalacia perforans typically begin before scleral necrosis develops?

Sectoral engorgement of deep episcleral vessels

A painful, elevated, intensely red nodule

Diffuse scleral oedema with globe tenderness

Which imaging confirms absence of scleral tissue in suspected scleromalacia perforans?

Anterior segment optical coherence tomography Anterior segment OCT demonstrates the absence of scleral tissue beneath the thin conjunctiva and confirms the diagnosis.

Fundus autofluorescence imaging

Why should periocular steroid injections be avoided in scleromalacia perforans?

They enhance collagenase activity and worsen scleral melting Periocular steroid injection can enhance collagenase activity, driving further scleral melting and perforation, so steroids are given systemically instead.

They raise intraocular pressure more than oral steroids

They reduce the efficacy of oral immunosuppression

They accelerate conjunctival scarring and symblepharon

Which oral drug class is the initial agent of choice in scleromalacia perforans?

Non-steroidal anti-inflammatory cyclo-oxygenase inhibitors Oral NSAIDs, selective or non-selective COX inhibitors, are used first; oral corticosteroids are reserved for patients who fail to respond.

Oral tetracyclines such as doxycycline

Antimetabolites such as methotrexate

Which general protective measure is important in managing scleromalacia perforans?

Protection of the eye from trauma No specific therapy exists, so protecting the fragile globe from trauma and treating the underlying systemic disease aggressively are the mainstays of general care.

Regular therapeutic bandage contact lens wear

Long-term topical antibiotic prophylaxis

Strict avoidance of any topical lubricant

Which immunosuppressive drug is considered most effective in non-infectious necrotizing scleritis?

Cyclophosphamide Cyclophosphamide, given orally at 2 to 3 mg/kg/day, is regarded as the most effective agent in non-infectious necrotizing scleritis; methotrexate, azathioprine, cyclosporine and mycophenolate are alternatives.

Which topical agent used in scleromalacia perforans inhibits the collagenolytic enzyme?

Topical sodium versenate Topical sodium versenate acts as a collagenolytic enzyme inhibitor. Frequent lubricants are also used, and topical cyclosporine A is described for refractory cases.

Scleromalacia Perforans: a rare, vision threating lesion

CASE REPORT

An 80-year-old woman with no previous systemic pathology, presented with bilateral progressive visual loss over the last two years.

Scleromalacia Perforans

Her last ophthalmological examination was after uneventful cataract surgery in both eyes, two years ago. No serious pathology was recorded, and her uncorrected visual acuity was 20/25 in both eyes.

During the examination, visual acuity was counting fingers in both eyes which would improve to 20/40 with -5.00cyl x 100° in the right eye and -5.00cyl x 110° in the left eye.

Slit lamp examination revealed nasal and temporal scleral thinning with the uveal shown under a thin conjunctival layer. Moreover, corneal thinning was observed in both the temporal and nasal limbus along with the presence of horizontal corneal striae extending throughout the cornea.

Anterior Segment Optical Coherence Tomography confirmed the absence of scleral tissue and the diagnosis of scleromalacia perforans.

DISEASE (Scleromalacia Perforans)

Scleromalacia perforans is an anterior necrotizing scleritis without inflammation, so-called scleromalacia perforans, a rare, severe eye disorder developing autoimmune damage of episcleral and scleral performing vessels (hypersensitivity type III).

Scleromalacia perforans was first reported by van der Hoeve in a talk given before the Royal Dutch Ophthalmological Society in 1930.

He noted that it was bilateral, began with yellow or greyish subconjunctival nodules and gradually developed into scleral necrosis with perforation and exposure of the uvea. Scleromalcia perforans (SP) is a rare (only 4% of scleritis).

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MANAGEMENT of Scleromalacia Perforans

General treatment

There is no specific therapy for Scleromalacia perforans. Protection from trauma is important. The underlying systemic disease should be treated aggressively.

Medical therapy

NSAIDS

Oral non-steroidal anti-inflammatory drugs (NSAIDs) are the initial agent of choice. These consist of non-selective or selective cyclo-oxygenase inhibitors (COX inhibitors).

Non-selective COX inhibitors such as flurbiprofen, indomethacin, and ibuprofen may be used. Indomethacin 50mg three times a day or 600mg of ibuprofen three times a day may be used. Patients using oral NSAIDs should be warned of the side effects of gastrointestinal (GI) side effects including gastric bleeding.

Patients with renal compromise must be cautioned against renal toxicity. NSAIDS that are selective COX-2 inhibitors may have fewer GI side effects but may have more cardiovascular side effects.

Corticosteroids

Oral corticosteroids may be used in patients unresponsive to COX inhibitors or those with the posterior or necrotizing disease. A typical starting dose may be 1mg/kg/day of prednisone which should be tapered.

Periocular injection of steroids should be avoided as they may enhance collagenase activity and lead to further scleral melting and perforations.

Different side effects of steroids that should be taken care of include elevated intraocular pressure, decreased resistance to infection, gastric irritation, osteoporosis, weight gain, hyperglycemia, and mood changes.

Immunomodulatory agents

Since it is an autoimmune condition leading to vessel damage (type III hypersensitivity), immunosuppressive therapy, supplemented with steroids is recommended to treat the destructive process.

Consultation with a rheumatologist or internal medicine specialist is recommended. Cyclophosphamide is known as the most effective drug in patients with non-infectious necrotizing scleritis (oral dose 2-3 mg/kg/d). Other immunosuppressive drugs like methotrexate (7.5-20 mg weekly), azathioprine (starting dose 2,5 mg/kg/d), cyclosporine (2.5-5.0 mg/kg/d), and mycophenolate mofetil (2-3 g/d)are well described.

“Biologics” are the new group of modifying immune response agents. There are some reports describing: tumor necrosis factor inhibitors–TNF1 (etanercept, infliximab), the interleukin-2 receptor blocker (daclizumab), the interleukin-1 receptor antagonist (anakinra), the anti lymphocyte medicament (rituximab, alemtuzumab) in ocular diseases, including scleritis.

Topical Therapy

Frequent lubricant installation. Topical Sodium Versenate is an inhibitor of the collagenolytic enzyme. For refractory cases, topical cyclosporine A is described.

Scleromalacia Perforans follow up

Adjustment of medications and dosages is based on the level of clinical response. Laboratory testing may be ordered regularly to follow the therapeutic levels of the medication, to monitor for systemic toxicity, or to determine treatment efficacy.

REFERENCES

American Academy of Ophthalmology. Scleromalacia perforans.
Kopacz D, Maciejewicz P, Kopacz M Scleromalacia Perforans– What We Know and What We Can Do. J Clinic Experiment Ophthalmol 2013; S2:009.
W. A. Manschot . Senile scleral plaques and senile scleromalacia.Br J Ophthalmol. 1978;Jun; 62(6): 376–380.
Brad Bowling. Kanski’s Clinical Ophthalmology, A Systemic Approach.8th Edition (2016).Chapter 8:Episclera and Sclera. p259.
Sakellariou G, Berberidis C, Vounotrypidis P A case of Behcet’s disease with scleromalacia perforans. Rheumatology (Oxford) 2005;44: 258-260.
Evans PJ, Eustace P Scleromalacia perforans associated with Crohn’s disease. Treated with sodium versenate (EDTA). Br J Ophthalmol 1973;57: 330-335.
Norman Ashton And H. E. Hobbs Effect Of Cortisone On Rheumatoid Nodules Of The Sclera (Scleromalacia Perforans). 1952; Br J. Ophthal.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

Scleromalacia perforans is best classified as which form of scleritis?
Scleromalacia perforans is the necrotizing anterior scleritis that occurs without inflammatory signs, which is why the eye can be quiet despite progressive scleral destruction.
Which immune mechanism underlies the vascular damage of scleromalacia perforans?
The disorder results from autoimmune type III hypersensitivity damage to the episcleral and scleral perforating vessels, which is why immunosuppression is central to treatment.
What proportion of all scleritis cases is accounted for by scleromalacia perforans?
Scleromalacia perforans is rare, making up only about 4% of scleritis cases.
How does scleromalacia perforans typically begin before scleral necrosis develops?
The condition is bilateral and starts with yellow or greyish subconjunctival nodules that gradually progress to scleral necrosis with perforation and uveal exposure.
Which imaging confirms absence of scleral tissue in suspected scleromalacia perforans?
Anterior segment OCT demonstrates the absence of scleral tissue beneath the thin conjunctiva and confirms the diagnosis.
Why should periocular steroid injections be avoided in scleromalacia perforans?
Periocular steroid injection can enhance collagenase activity, driving further scleral melting and perforation, so steroids are given systemically instead.
Which oral drug class is the initial agent of choice in scleromalacia perforans?
Oral NSAIDs, selective or non-selective COX inhibitors, are used first; oral corticosteroids are reserved for patients who fail to respond.
Which general protective measure is important in managing scleromalacia perforans?
No specific therapy exists, so protecting the fragile globe from trauma and treating the underlying systemic disease aggressively are the mainstays of general care.
Which immunosuppressive drug is considered most effective in non-infectious necrotizing scleritis?
Cyclophosphamide, given orally at 2 to 3 mg/kg/day, is regarded as the most effective agent in non-infectious necrotizing scleritis; methotrexate, azathioprine, cyclosporine and mycophenolate are alternatives.
Which topical agent used in scleromalacia perforans inhibits the collagenolytic enzyme?
Topical sodium versenate acts as a collagenolytic enzyme inhibitor. Frequent lubricants are also used, and topical cyclosporine A is described for refractory cases.