👁️ Scleromalacia perforans is a severe form of necrotising anterior scleritis without inflammation, most commonly associated with rheumatoid arthritis. ⚠️ It is characterised by progressive scleral thinning with minimal pain, leading to exposure of the underlying uvea (bluish appearance). 👉 It is a vision-threatening condition and a marker of systemic autoimmune disease.

📖 Definition

A subtype of anterior scleritis with painless scleral necrosis.
Occurs without the marked inflammation seen in typical scleritis.

🦠 Aetiology & Associations

🧬 Strongly associated with rheumatoid arthritis (especially long-standing disease)
Other connective tissue diseases (rare):

Systemic lupus erythematosus
Granulomatosis with polyangiitis

Underlying mechanism: immune-mediated vasculitis of scleral vessels

⚠️ Pathophysiology

Immune complex deposition → vasculitis of episcleral/scleral vessels
Reduced blood supply → ischaemia and collagen degradation
Progressive scleral thinning → exposure of underlying uveal tissue

🔍 Clinical Features

👁️ Painless (key distinguishing feature)
🔵 Blue/grey sclera (visible uveal tissue)
🪨 Localised scleral thinning (“melting” appearance)
👁️ May have minimal redness despite severe disease
⚠️ Often occurs in patients with known RA

🚩 Red Flags

Progressive scleral thinning → risk of globe perforation
Visual disturbance
Signs of active systemic autoimmune disease

🧪 Investigations

🧠 Clinical diagnosis (ophthalmology assessment essential)
🩸 Autoimmune screen:

RF, anti-CCP (rheumatoid arthritis)
ANCA (vasculitis)

🖥️ Imaging (if needed): anterior segment OCT or ultrasound

👁️ Episcleritis vs Scleritis vs Scleromalacia Perforans

Feature	Episcleritis	Scleritis	Scleromalacia Perforans
🔥 Pain	Mild discomfort or none	Severe, deep, boring pain that may radiate to the jaw, brow, or temple	Painless ⚠️
🔴 Redness	Sectoral or diffuse redness	Diffuse, deep violaceous or dusky redness	Minimal redness despite severe disease
🎯 Vessel appearance	Superficial episcleral vessels	Deep scleral vessels with a purple-blue hue	Marked scleral thinning with visible underlying uveal tissue giving a blue-grey appearance
💧 Blanching with phenylephrine	Yes – superficial vessels blanch	No – deep scleral vessels do not blanch	No
👁️ Visual acuity	Usually normal	May be reduced	May be reduced
🧬 Underlying cause	Often idiopathic; may be associated with mild systemic disease	Often autoimmune, especially rheumatoid arthritis or systemic vasculitis	Strongly associated with long-standing rheumatoid arthritis
⚠️ Degree of inflammation	Mild, superficial inflammation	Marked deep inflammation	Minimal visible inflammation despite necrotising disease
🪨 Structural damage	None	Possible scleral thinning and necrosis	Severe scleral thinning with scleral “melting”
💥 Complications	Rare	Vision loss, keratitis, uveitis, scleral thinning, perforation	Globe rupture and permanent visual loss 🚨
💊 Treatment	Usually self-limiting; lubricants or oral NSAIDs if symptomatic	Systemic NSAIDs, corticosteroids, or immunosuppressive therapy depending on severity	Urgent systemic immunosuppression; may require scleral grafting
🚨 Urgency	Low	Urgent ophthalmology review	Ophthalmic emergency

🧠 Exam Tips

📌 Episcleritis is benign, superficial, and usually only mildly uncomfortable.
📌 Scleritis causes severe pain and is often linked to systemic autoimmune disease.
📌 Scleromalacia perforans is the classic painless blue sclera seen in long-standing rheumatoid arthritis.
📌 The absence of pain in scleromalacia perforans is a classic exam trap — it is often the most severe of the three.
📌 Phenylephrine blanching helps distinguish episcleritis from deeper scleral disease.

💊 Management

⚠️ Urgent ophthalmology referral
💊 Systemic immunosuppression is required (topicals are inadequate)

Systemic corticosteroids
DMARDs (e.g. methotrexate)
Biologics in refractory disease (e.g. rituximab)

👁️ Lubricants to protect ocular surface
🔧 Surgical intervention (e.g. scleral graft) if severe thinning/perforation risk

⚡ Complications

💥 Globe rupture
👁️ Vision loss
🧬 Progression of systemic autoimmune disease

🧠 Exam Tips

📌 Painless + blue sclera + rheumatoid arthritis = scleromalacia perforans
📌 It is a form of necrotising scleritis (but without inflammation)
📌 Requires systemic treatment, not just topical eye drops
📌 Strong association with long-standing RA

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Scleroderma perforans of the sclera (scleromalacia perforans),

📖 Definition

🦠 Aetiology & Associations

⚠️ Pathophysiology

🔍 Clinical Features

🚩 Red Flags

🧪 Investigations

👁️ Episcleritis vs Scleritis vs Scleromalacia Perforans

🧠 Exam Tips

💊 Management

⚡ Complications

🧠 Exam Tips

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