Citation, DOI, disclosures and article data
Citation:
Bell D, Sharma R, Yap J, et al. Scleromalacia perforans. Reference article, Radiopaedia.org (Accessed on 18 Apr 2024) https://doi.org/10.53347/rID-59747
Scleromalacia perforans, or non-inflammatory necrotizing scleritis, is a severe and very rare form of scleritis.
Associations
The condition is generally asymptomatic. It is usually found on visual inspection during a formal eye examination.
Scleromalacia perforans is a rare non-inflammatory form of anterior necrotizing scleritis, usually seen in patients with autoimmune disease.
The scleral thinning may secondarily calcify, and can be seen on CT. In addition, the rare sequela of atraumatic globe rupture might be present. Other manifestations of the underlying autoimmune disease may also be present.
Treatment and prognosis
The condition is usually asymptomatic. Rupture of the globe is rare and usually only seen in those with coexisting markedly elevated intraocular pressure, i.e. severe glaucoma 1.
Historically the condition has shown a limited response to conventional treatments. Treatment options include 6:
non-steroidal anti-inflammatory drugs (NSAIDs)
topical treatments: glucocorticoids, tacrolimus, cyclosporin
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systemic immunosuppressants:
glucocorticoids
TNF-alpha inhibitors
other immunosuppressants: cyclophosphamide, mycophenolate, azathioprine, methotrexate, rituximab, tocilizumab, tofacitinib
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1. Wu CC, Yu HC, Yen JH, Tsai WC, Liu HW. Rare extra-articular manifestation of rheumatoid arthritis: scleromalacia perforans. (2005) The Kaohsiung journal of medical sciences. 21 (5): 233-5. doi:10.1016/S1607-551X(09)70193-7 - Pubmed
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2. Lawuyi LE, Gurbaxani A. Refractory necrotizing scleritis successfully treated with adalimumab. (2016) Journal of ophthalmic inflammation and infection. 6 (1): 37. doi:10.1186/s12348-016-0107-y - Pubmed
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3. Reddy SC, Tajunisah I, Rohana T. Bilateral scleromalacia perforans and peripheral corneal thinning in Wegener's granulomatosis. (2011) International journal of ophthalmology. 4 (4): 439-42. doi:10.3980/j.issn.2222-3959.2011.04.22 - Pubmed
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4. Bothra N, Mittal R, Tripathy D. Scleromalacia Perforans from Long-Standing Bulbar Conjunctival Rhinosporidiosis. (2016) Ophthalmology. 123 (7): 1493. doi:10.1016/j.ophtha.2016.04.010 - Pubmed
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5. Sakellariou G, Berberidis C, Vounotrypidis P. A case of Behcet's disease with scleromalacia perforans. (2005) Rheumatology (Oxford, England). 44 (2): 258-60. doi:10.1093/rheumatology/keh445 - Pubmed
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6. Dutta Majumder P, Agrawal R, McCluskey P, Biswas J. Current Approach for the Diagnosis and Management of Noninfective Scleritis. Asia-Pacific Journal of Ophthalmology. 2021;10(2):212-23. doi:10.1097/apo.0000000000000341 - Pubmed
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