Elevated Intraocular Pressure in Periorbital Sweet’s Syndrome

Abstract

A 66-year-old immunocompromised man presented with cellulitis around the left eye that was initially concerning for necrotizing fasciitis. Exam findings were remarkable for exquisite periocular tenderness with rigid, immobile eyelids resulting from severe erythema, edema, and induration. Given the concern for orbital compartment syndrome and a necrotizing infection, the patient was taken urgently to the operating room for debridement of the eyelid skin as well as an urgent lateral canthotomy and cantholysis. His eye exam revealed 360° of hemorrhagic chemosis, no relative afferent pupillary defect, and an ipsilateral elevated intraocular pressure of 35 mm Hg. No visual acuity measurement could be obtained secondary to the patient’s altered mental status. His intraocular pressure normalized after treatment with antihypertensive drops and further extension of the canthotomy. Histopathological analysis showed extensive neutrophilic infiltrate of the dermis which was compatible with a diagnosis of Sweet’s syndrome.

Periorbital necrotizing sweet syndrome: A report of two cases mimicking necrotizing soft tissue infections

“…To our knowledge, there have only been two prior reports of nSS involving the periorbital region. 3 , 4 One patient had very focal necrosis limited to the eyelid margin; SS in this case was felt to be associated with inflammatory bowel disease and psoriatic arthritis, and was successfully treated with oral corticosteroids. 3 The other patient had a history of AML, and his initial presentation resembled NF which was nonresponsive to antimicrobials and serial debridement; he also improved with corticosteroid treatment.…”

“… 3 The other patient had a history of AML, and his initial presentation resembled NF which was nonresponsive to antimicrobials and serial debridement; he also improved with corticosteroid treatment. 4 Notably, the latter patient underwent canthotomy-cantholysis during debridement and was initiated on eye pressure-lowering drops for an elevated IOP of 35 mmHg. 4 Direct ocular manifestations of SS, such as elevated IOP, conjunctivitis, and other forms of ocular/orbital inflammation, have been well documented in the literature.…”

“… 4 Notably, the latter patient underwent canthotomy-cantholysis during debridement and was initiated on eye pressure-lowering drops for an elevated IOP of 35 mmHg. 4 Direct ocular manifestations of SS, such as elevated IOP, conjunctivitis, and other forms of ocular/orbital inflammation, have been well documented in the literature. 8 One study with biopsy data of ocular tissue affected by SS reported histopathology that resembled that of the cutaneous lesions.…”

“…Limited reports have documented necrotizing Sweet syndrome (nSS), with only two involving the periorbital region. 2 , 3 , 4 NF is primarily treated with aggressive surgical debridement. SS, as with other neutrophilic dermatoses, shows pathergy and is instead treated with corticosteroids and immunosuppression.…”