Sweet's syndrome is an uncommon skin disorder of unkown aetiology characterised by painful erythematous plaques, and associated with pyrexia, marked leucocytosis and elevated erythrocyte sedimentation rate (ESR). Although originally described as affecting the arms and legs, it has been reported on the head and neck. Periocular presentations are unusual and particularly associated with escharotic lesions. We report a case with marked bilateral periocular swelling.
A 72-year-old man developed bilateral periocular swelling overnight. He gave no history of facial trauma, sinus disease, or allergy. He denied diplopia, or visual loss. His past medical history included ischaemic heart disease for which he took oral diltiazem, bumetanide, elantan LA, aspirin, and sublingual glyceryl trinitrate. On admission, the patient was pyrexial with a temperature of 38.8°C and dyspnoeic with ankle swelling. He had gross bilateral periocular swelling and vesicular exudative lesions of the eyelids (Figure 1). There was no lymphadenopathy, or skin involvement other than the periocular area.
The corrected near vision was N8 right and left. Pupil reactions were brisk and normal. Motility was restricted particularly on the left and in all positions of gaze with mild proptosis. The anterior and posterior segments were healthy.
Clinically he was treated for probable Herpes Zoster cellulitis with secondary bacterial infection. In view of his pyrexia and likely cardiac failure, a medical opinion was sought and the following investigations were requested: ESR 100 mm/h (<20), elevated white cell count (WCC) 13.9 × 10/l (4–16), C-reactive protein 638 mg/l (<10), random glucose 25.6 mmol/l (4.5–5.6), urea 17 mg/dl (3–7), creatinine 172 μ/l (60–110), sodium 124 mmol/l (135–146), potassium 4.6 mmol/l (3.5–5.0), blood cultures that subsequently were negative and a chest X-ray (CXR) showed an enlarged heart and pulmonary oedema.
Intravenous insulin and frusemide were commenced to control his diabetes and heart failure. His cellulitis was treated with intravenous acyclovir and benzylpenicillin with flucloxacillin.
After 24 h, computed tomography (CT) was arranged of the orbits, paranasal sinuses, and brain in order to exclude orbital cellulitis, subperiosteal abscess, cavernous sinus thrombosis, or mucormycosis. The CT demonstrated only preseptal ocular and facial swelling (Figure 2).
Over the next 48 h the diffuse swelling subsided but the crusting became haemorrhagic and necrotic particularly over the left upper lid (Figure 3). A dermatology opinion suggested a dermatosis, and a skin biopsy was taken of the left upper lid.
Pathologic examination demonstrated that the epidermis was uninvolved but there was a dense polymorph infiltration of the entire thickness of the dermis (Figure 4). The blood vessels demonstrated swelling of the endothelial cells but no evidence of a true vasculitis (Figure 5, arrow). This suggested a diagnosis of Sweet's syndrome, or acute neutrophilic dermatosis.
He was commenced on oral prednisolone 30 mg daily and made a rapid recovery.