Scleromalacia perforans

Scleromalacia perforans (or non-inflammatory necrotizing scleritis) is a severe and very rare form of scleritis.

Epidemiology

Associations

• rheumatoid arthritis
• Crohn disease
• ulcerative colitis
• systemic lupus erythematosus
• Behçet disease 
• relapsing polychondritis 
• granulomatosis with polyangiitis 
• rhinosporidiosis

Clinical presentation

The condition is generally asymptomatic. It is usually found on visual inspection during a formal eye examination.

Pathology

Scleromalacia perforans is a rare non-inflammatory form of anterior necrotizing scleritis, usually seen in patients with autoimmune disease.

Radiographic features

The scleral thinning may secondarily calcify, and can be seen on CT. In addition the rare sequela of atraumatic globe rupture might be . 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 .

Historically the condition has shown a limited response to conventional treatments, e.g. steroids. A recent article suggests that adalimumab may be of benefit .