The scleromalacia perforans

Subtype of anterior necrotizing scleritis in granulomatosis with polyangiitis

Abstract

Introduction: granulomatosis with polyangiitis (GPA) is the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with the highest rate of eye involvement. The scleromalacia perforans subtype of anterior necrotizing scleritis is a rare but severe form of this disease.

Clinical case: we present the case of a 44-year-old male patient debuting with c-ANCA positive GPA with nasal, sinus, pulmonary, renal, and ocular involvement and documented necrotizing scleritis (scleromalacia perforans subtype). This was associated with the formation of an anterior eye staphyloma, placing him at risk of eye and vision loss.

Conclusion: it is crucial for clinicians to understand GPA’s ocular effects, as this allows rapid diagnostic guidance and treatment modification, seeking to minimize eye and vision loss.