Sight-Threatening Ocular Manifestations of Acute Febrile Neutrophilic Dermatosis (Sweet's Syndrome)

Abstract

Sweet's syndrome is a primarily dermatologic disorder with many features of systemic inflammation. It is generally characterized by a neutrophilic dermatosis in the setting of fever and an elevated white blood cell count. Inflammation has been described to occur in many organ systems including the lung, bone, liver, spleen, brain and eye. Ocular inflammation is a well-known comorbidity that may occur in the setting of Sweet's syndrome, including conjunctivitis, episcleritis, scleritis, iritis and choroiditis, among other forms. In the current article, we have compiled a series of cases that describe three separate patients who demonstrated a rare form of ocular involvement in Sweet's syndrome, retinal vasculitis. The evidence from these three cases and other reports in recent ophthalmologic literature suggest overlapping of ocular manifestations of Sweet's syndrome and the closely related Behçet's disease. It is important to be aware of the sometimes challenging differential between these two disorders and their sight-threatening complications.

Severe Sweet's Syndrome with Elevated Cutaneous Interleukin-1β after Azathioprine Exposure: Case Report and Review of the Literature

“…Inflammation in some of these organs can cause serious long-term quality of life impairment. The sight-threatening ocular manifestations merit particular mention [22]. Cutaneous biopsies reveal an inflammatory infiltrate consisting mainly of mature neutrophils in the upper dermis.…”

An acute acral papulovesicular eruption

“…The most important clinical clue is a decrease in visual acuity. Inflammation of anterior structures may also cause patients to complain of blurry vision . Hence, any visual impairment in Sweet syndrome should promptly be referred to an ophthalmologist to prevent ocular morbidity.…”

Diagnostic Criteria in Dermatology

“…[5,6,7] It can be associated with recurrent aseptic meningoencephalitis, known as Neuro-Sweet disease. [8] Su and Liu proposed a set of major and minor criteria for the diagnosis of Sweet's Syndrome in 1986. [9] Von den Driesch modified these diagnostic criteria in 1994, according to which both major and two minor criteria were required for diagnosis of Sweet's Syndrome (Table 2).…”