Periocular necrotizing sweet syndrome – a case series and review of literature

Sweet syndrome (SS), or acute neutrophilic dermatosis, is a dermatologic condition characterized by fever and neutrophilia with tender papular or plaque-like eruptions; diagnosis is confirmed histologically and the disease typically responds to systemic corticosteroids. Ocular associations include conjunctivitis, peripheral ulcerative keratitis, scleritis, uveitis, glaucoma, and retinal detachment, with occasional periocular involvement. A rare clinical variant, necrotizing Sweet's syndrome (NSS), closely mimics necrotizing fasciitis (NF). While NF requires urgent, extensive surgical debridement, NSS requires tissue biopsy, systemic corticosteroid therapy, and systemic evaluation to rule out underlying malignancy; misdiagnosis can therefore lead to inappropriate surgery and delayed treatment. We present three patients with isolated periocular SS. One had the classical form, while two were subsequently diagnosed with hematologic disease. All three exhibited features of NSS and underwent limited surgical debridement with microbiological and histopathological work-up, which confirmed SS. Collagen sheet grafting was used for wound coverage; two patients achieved rapid and cosmetically favourable recovery, while in one patient with full-thickness margin necrosis collagen alone was insufficient and an acquired eyelid coloboma remained. These cases underscore the need to distinguish NSS from NF: despite similar clinical appearance, the management is diametrically opposite.