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Sir,

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.

Case report

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.

Figure 1
figure 1

Bilateral periocular swelling with exudtive vesicles more marked on the left.

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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).

Figure 2
figure 2

CT demonstrates preseptal and facial swelling.

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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.

Figure 3
figure 3

Crusting escharotic appearance especially on the left.

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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.

Figure 4
figure 4

Unremarkable epidermis (black arrow) with haemorrhagic dermis infiltrated by polymorphs.

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Figure 5
figure 5

Arrow shows swollen endothelial lining, but lack of inflammatory changes of blood vessels denoting absence of a true vascilities.

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He was commenced on oral prednisolone 30 mg daily and made a rapid recovery.

Comment

Sweet's syndrome, also known as acute neutrophilic dermatosis, was first described by Dr Robert D Sweet in 1964.1 It is characterised by abrupt onset fever, painful erythematous skin papules and plaques, and neutrophilic leucocytosis. It may be idiopathic, drug induced,2 associated with haematological malignancy or immunologic conditions such as rheumatoid arthritis, and inflammatory bowel disease.

The main histological feature is the infiltration of the dermis by mature neutrophils and absence of vasculitis although in lesions of longer duration a secondary vasculitis may develop.3

It is important to note that the diagnosis of Sweet's syndrome is based on clinical suspicion. von den Driesch4 has proposed diagnostic criteria which must include typical skin lesions and histology accompanied by other features. In atypical cases, the diagnostic features include a markedly raised ESR, leucocytosis, and pyrexia but no evidence of infection. Pathologic investigation is invaluable in making the diagnosis since up to a third of cases will have recurrent episodes.

Various case reports of ocular manifestations have been described such as scleritis.5 One report is of a 40- year- old man presenting with an erythematous vesiculopustular facial eruption that had prominent black escharotic crust particularly over the periorbital area.6 This was identical to our patient except that marked preseptal swelling was absent and in our patient the escharotic appearance developed during the course of the disease.

Two interesting ocular manifestations have been described in patients with systemic diseases presenting with Sweet's syndrome. Wilson et al7 describe a patient with rheumatoid arthritis who had separate episodes of Sweet's syndrome, pyoderma gangrenosum, pustular vasculitis, and peripheral ulcerative keratitis (PUK). While PUK is a recognised entity in association with rheumatoid arthritis alone, they comment on the inflammatory spectrum of these conditions and the effective response to cyclosporine.

Morgan and Callen8 report a 38-year-old man with acute myelogenous leukaemia presenting with a left cellulitis and later with haemorrhagic pustules on his hands. Biopsy revealed a neutrophil infiltrate of the dermis.

The pathogenesis of Sweet' syndrome is unknown. Gassuddin et al9 propose an immunologic mechanism in which there is an imbalance between T helper cell types and cytokine secretion. The release of cytokines occurs in response to an unidentified trigger that results in chemoattraction of leucocytes. This may explain the rapid response to immunosuppressive therapy.

This unusual skin condition should be considered in all cases of periorbital oedema of unknown cause.