Scleromalacia Perforans

All content on Eyewiki is protected by copyright law and the Terms of Service. This content may not be reproduced, copied, or put into any artificial intelligence program, including large language and generative AI models, without permission from the Academy.

Article initiated by:

Ankita Aishwarya

All contributors:

Sahil Thakur, Ankit Agrawal, Ghazala A. D. O'Keefe, MD, Ankita Aishwarya, Edmund Tsui, MD, Paulina Liberman

Assigned editor:

Paulina Liberman

Review:

Assigned status Up to Date

 by Paulina Liberman on October 13, 2025.

add
Contributing Editors:	add

Scleromalacia Perforans

Scleromalacia perforans consists of scleral melting in a white, quiet eye. This condition also can lead to ocular perforation. © 2019 American Academy of Ophthalmology

ICD-10

H15.053

Disease Entity

Scleromalacia Perforans (SP). ICD-10: H15.053

Disease

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

History Of Disease

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 manifestation, accounting for approximately 4% of scleritis.

Etiology

It is a type III hypersensitivity reaction which occurs due to accumulation of immune complexes (Antigen-Antibody complexes).

Risk Factors

Scleromalacia perforans typically occurs in elderly women with long-standing rheumatoid arthritis. Other associated conditions are systemic lupus erythematosus, periarteritis nodosa, granulomatosis with polyangiitis (formerly Wegener granulomatosis), Behçet disease, limited scleroderma, Crohn’s disease, graft-versus-host disease. SP was also observed in porphyria and herpes-zoster infection.

General Pathology

Chronic granulomatous changes with epithelioid cells surround central, necrotic masses (collagen and non-collagen fibers, cell debris) .

Pathophysiology

Young and Watson suggest three determinants of scleral destruction: activation of scleral fibrocytes and resorption of pericellular matrix, infiltration of the scleral stroma by inflammatory cells, prolonged local vaso-occlusion. In SP dense plaques of necrotic tissues are removed and it is associated with full thickness loss of conjunctiva and insufficiency of conjunctival epithelium to resurface the exposed area.

Diagnosis

Symptoms

The onset is gradual. Most patients complains of non-specific irritation. Pain is generally absent and vision is unaffected. The change in scleral color is often detected by patient’s family, by patient looking in the mirror or by an ophthalmologist during routine examination.

Signs

Necrotic scleral plaques near the limbus without vascular congestion. Coalescence and enlargement of necrotic areas. The sclera thins and the underlying dark uveal tissue becomes visible. In many cases, the uvea is covered with only thin connective tissue and conjunctiva. A bulging staphyloma develops if intraocular pressure is elevated; spontaneous perforation is rare, although these eyes may rupture with minimal trauma.

Clinical Diagnosis

Scleromalacia perforans is a clinical diagnosis. However, laboratory testing is often necessary to evaluate any associated underlying connective tissue and autoimmune disease.

Laboratory test

Autoimmune connective tissue disorders like rheumatoid arthritis, lupus, sero-negative spondylarthropathies , granulomatosis with polyangiitis and polyarteritis nodosa should be ruled out. boratory tests include complete blood count (CBC) with differential, erythrocye sedimentation rate (ESR) or C-reactive protein (CRP), serum autoantibody screen (including antinuclear antibodies, anti-DNA antibodies, rheumatoid factor, antineutrophil cytoplasmic antibodies), urinalysis, syphilis serology, serum uric acid and sarcoidosis screen.

Differential diagnosis

Scleral Hyaline Plaque: age-related hyaline degeneration of the sclera. Plaque lies in the palpebral fissure immediately anterior to the medial rectus muscle insertion. Senile Scleromalacia: is a scleral disease which occurs by expulsion of a calcified plaque in advanced cases of senile scleral plaques.

Management

General treatment

The management of Scleromalacia perforans is fundamentally systemic and requires an aggressive, multidisciplinary approach. Protection from trauma is important.

Medical therapy

Given this is a potentially blinding disease the patient should be treated with a combination of systemic corticosteroids and immunosuppression.

Corticosteroids

Systemic corticosteroids should be used as intravenous methylprednisolone followed by oral prednisone or oral prednisone alone, according to discretion of the treating clinician. 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 include elevated intraocular pressure, decreased resistance to infection, gastric irritation, osteoporosis, weight gain, hyperglycemia, and mood changes.

Immunomodulatory agents

Due to the severity of the disease immunosuppressive therapy, supplemented with steroids is perentory 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. Between them the most commonly used are tumor necrosis factor inhibitors–TNF1 (adalimumab, infliximab), and anti-CD20 medications (rituximab).

Topical Therapy

Frequent lubricant instillation is recommended for comfort and ocular surface protection. Topical immunomodulators like cyclosporine A have also been described.

Medical 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.

Surgery

Surgical treatment of SP may be necessary in cases with exposed uvea to preserve the globe integrity. Tectonic patch grafting can be performed with the sclera (fresh or frozen globe or glycerin preserved scleral tissue), dermis, fascia lata, periosteum, aortic tissue, cartilage, cornea, pedicle-flaps of conjunctiva with Müller muscle or tarsus, synthetic material (GoreTex®) and eventually amniotic membrane.

Complications

Complications include perforation especially after minor trauma and pthisis bulbi, therefore protection from trauma is important.

Prognosis

The overall prognosis is guarded and closely tied to the control of the inflammation.