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Optic nerve involvement with panuveitis in Sweet syndrome.
Ann-Marie Lobo1, Rebecca Stacy, Dean Cestari
1Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts 02114, USA. Ann-Marie_Lobo@meei.harvard.edu
Ocular Immunology and Inflammation
|May 21, 2011
Summary
Neuro-Sweet syndrome, a systemic inflammatory condition, can affect the optic nerve, leading to vision problems. Early diagnosis and treatment are crucial for managing this rare but serious disease.
Area of Science:
- Ophthalmology
- Dermatology
- Rheumatology
Background:
- Sweet syndrome is a rare neutrophilic dermatosis characterized by fever, neutrophilia, and tender, erythematous papules and plaques.
- Ocular involvement in Sweet syndrome is uncommon but can lead to significant visual impairment.
Observation:
- A 49-year-old male presented with bilateral panuveitis and optic disc edema.
- He also exhibited a papular rash, fever, malaise, and arthralgias, consistent with neuro-Sweet syndrome.
- Laboratory findings included aseptic meningitis and neutrophilic leukocytosis.
Findings:
- Skin biopsy confirmed an intense neutrophilic infiltrate, supporting the diagnosis of Sweet syndrome.
- The patient required ongoing corticosteroid therapy despite immunomodulatory treatment with infliximab.
- Optic nerve involvement, presenting as optic disc edema, was a key feature in this case.
Implications:
- This case highlights the importance of considering optic nerve involvement in patients diagnosed with neuro-Sweet syndrome.
- Comprehensive screening for underlying causes, such as autoimmune diseases or malignancy, is essential for effective management.
- Prompt recognition and targeted therapy can help prevent irreversible visual loss.
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