Article Text

Scleritis associated with acute febrile neutrophilic dermatosis (Sweet’s syndrome)
  1. TERESA C CHEN,
  2. DEBRA A GOLDSTEIN,
  3. HOWARD H TESSLER
  1. Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago,
  2. Illinois, USA
  3. Department of Medicine, University of Illinois Hospitals, Chicago, Illinois, USA
  4. Department of Pathology, University of Illinois Hospitals, Chicago, Illinois, USA
  1. JOHN P QUINN
  1. Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago,
  2. Illinois, USA
  3. Department of Medicine, University of Illinois Hospitals, Chicago, Illinois, USA
  4. Department of Pathology, University of Illinois Hospitals, Chicago, Illinois, USA
  1. CATALINO M BAUTISTA
  1. Department of Ophthalmology, University of Illinois Eye and Ear Infirmary, Chicago,
  2. Illinois, USA
  3. Department of Medicine, University of Illinois Hospitals, Chicago, Illinois, USA
  4. Department of Pathology, University of Illinois Hospitals, Chicago, Illinois, USA
  1. Debra Goldstein, MD, University of Illinois Eye and Ear Infirmary, Department of Ophthalmology, 1855 West Taylor Street, Chicago, IL 60612, USA.

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Editor,—Sweet’s syndrome, or acute febrile neutrophilic dermatosis, is characterised by (1) fever, (2) neutrophilic leucocytosis, (3) the abrupt appearance of 0.5–12.0 cm painful erythematous nodules and plaques, especially on the face, neck, and limbs, and (4) a histological pattern of dense dermal infiltrates of mature neutrophils without vasculitis.1 2 Patients are usually middle aged women and may have extracutaneous manifestations involving the eyes, kidneys, joints, liver, and lungs. Sweet’s syndrome is associated with underlying malignancy, especially haematological, in 10% to 54% of patients2 3 and with systemic inflammatory disorders. There may be a prodromal respiratory illness and an elevated erythrocyte sedimentation rate (ESR).

Ocular involvement is reported in 4% to 72% of cases and may manifest as conjunctivitis, episcleritis, subconjunctival haemorrhage, inflammatory glaucoma, iritis, or limbal nodules.3-6 We describe a case of a patient with Sweet’s syndrome and scleritis.

CASE REPORT

A 78 year old white man presented to his ophthalmologist with a 2 week complaint of a red, sore right eye without decreased vision. After a week of worsening symptoms on topical ciprofloxacin drops for presumed conjunctivitis, the ophthalmologist switched the patient to topical prednisolone acetate 0.125% for possible episcleritis. The persistence of ocular symptoms despite topical prednisolone acetate 0.125% as well as a 6 week history of numerous erythematous slightly tender papules over his extremities prompted referral to our hospital.

The patient’s ocular history was significant for endophthalmitis in the left eye after extracapsular cataract extraction 3 years earlier. Additional history included a laryngeal mass biopsy 1 year earlier showing non-specific inflammation, recurrent facial basal cell cancer, 4 months of episodic fever up to 102°F, and right mastoiditis and otitis media 1 month before initial presentation.

At our initial examination, best corrected visual acuity was right eye 6/6 and left eye light perception with projection. Moderate injection was noted medially in the right eye, with pronounced tenderness to palpation. Application of 10% neosynephrine blanched the superficial vessels, revealing deep injection of scleral vessels as well as three broad scleral nodules with a central 3.0 mm yellow white avascular area (Fig 1). The left cornea had diffuse stromal oedema with an irregular superior pannus. The anterior chamber was deep and quiet in the right eye but flat in the left eye. The remainder of the anterior and posterior segment examinations were not significant. Examination of his arms and legs revealed multiple erythematous papules ranging from 2–10 cm in diameter.

Figure 1

Photograph of right eye with medial scleral nodules.

Treatment with prednisolone acetate 0.125% four times a day to the right eye was continued, and oral prednisone 60 mg daily was added.

The possibility of Sweet’s syndrome led to further examination and dermatology consultation. Significant findings included an ESR of 117 and a WBC of 16.4 × 109/l (neutrophils 31, lymphocytes 33, monocytes 34, bands 0). Blood cultures, uric acid, ANCA, ANA, RF, FTA-ABS, RPR, ACE, lysozyme, HLA-B27, and chest x ray were unremarkable. A bone scan showed intense uptake of the left knee suggesting osteomyelitis, inflammatory disease, or neoplasm. A skin biopsy revealed dermal neutrophilic infiltrates without vasculitis, consistent with Sweet’s syndrome (Fig 2). Special stains for organisms (AFB, PAS, B&B, GMS stains) were negative.

Figure 2

Skin biopsy with diffuse neutrophilic infiltrate and dermal oedema but without vasculitis (original magnification × 340).

A subsequent bone marrow biopsy demonstrated active cellular marrow with proliferation of all three cell lines and an excess of “blast” cells, most of which were myeloid and a minority of which were monocytes. This suggested a myelodysplastic syndrome, consistent with refractory anaemia with excess blasts (RAEB).

Over the next 2 weeks, the patient’s scleritis began to resolve with both prednisolone acetate 0.125% and oral prednisone 60 mg daily. Medications were tapered over 2 months; however, once a dose of 30 mg of prednisone was reached, he developed new skin lesions and the scleritis recurred. Increasing the prednisone to 40 mg daily resulted in partial improvement of the skin lesions and resolution of the scleritis. The skin lesions and scleritis have remained inactive for 16 months on prednisone 15 mg daily and prednisolone acetate 0.125% one drop every other day.

COMMENT

Most articles in the medical literature on Sweet’s syndrome describe ocular involvement as benign.2 There are only two case reports in the ophthalmic literature describing potentially vision threatening ocular complications associated with this disease.5 6 We believe this is the first report of a patient with Sweet’s syndrome presenting with scleritis. A physician seeing a patient with ocular inflammation, skin lesions, and fever must consider the diagnosis of Sweet’s syndrome. This condition should not be missed, because it may be associated with an underlying haematological malignancy or solid tumour.

References

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