Can Sweet’s syndrome affect the eyes?

Help for Sweet's Syndrome, UK 5 Comments

Links checked 07/04/19.

Sweet’s syndrome can affect the eyes in up to 72% of patients (Bilgin et al, 2015:54). Usually, only one eye will be affected, but it’s possible for both eyes to be affected at the same time (Zaouak et al, 2017; Zhuang and Li, 2011).

What are the symptoms?

Initial symptoms often include:

  • Eyes feeling sore.
  • Eye pain, e.g sharp or stabbing pain.
  • Blurry vision.

Other symptoms:

  • Eye redness.
  • Dry eyes.
  • Eye watering.
  • Itching.
  • ‘Sticky’ or gunky eyes.
  • Sensitivity to light.

On rare occasions, eye symptoms may worsen causing severe inflammation, tissue damage, ulceration, and vision loss. In December 2017, the first case of permanent vision loss in the left eye was reported in a 40-year-old man with Sweet’s syndrome and probable myelodysplastic syndrome or myeloproliferative neoplasm (Jang et al, 2017).

What are the commonest eye problems?

Conjunctivitis, episcleritis, limbal nodules and iridocyclitis are the some of the commonest eye problems (Baartman et al, 2014:193).

What other eye problems can Sweet’s syndrome cause?

Other eye problems include:

  • Blepharitis (Cohen, 2007).
  • Choroiditis (Baartman et al, 2014:193).
  • Conjunctival erythematous lesions with tissue biopsy showing neutrophilic inflammation (Cohen, 2007).
  • Conjunctival haemorrhage.
  • Conjunctival nodules (Zhuang and Li, 2011).
  • Dacryoadenitis (Cohen, 2007).
  • Glaucoma (Bilgin et al, 2015:54; Cohen, 2007). This is rare in Sweet’s syndrome, and can also be caused by steroid medication.
  • Iritis (Cohen, 2007).
  • Ocular congestion.
  • Optic nerve involvement (Bilgin et al, 2015:54; Jang et al, 2017; Koay et al, 2013; Lobo et al, 2011).
  • Periocular cellulitis (cellulitis around the eyes), swelling around the eyes, lesions on the eyelid or around the eyes, or eye and eyelid inflammation (Bilgin et al, 2015:54; Cohen, 2007; Khatri and Taha, 2007; Morgan and Callen, 2001).
  • Peripheral ulcerative keratitis (Bilgin et al, 2015; Carbonell et al, 2011; Cohen, 2007). This is rare in Sweet’s syndrome, but can cause severe inflammation and tissue damage, potentially leading to visual impairment and blindness.
  • Raccoon eyes or periorbital ecchymosis.
  • Retinal vasculitis (Baartman et al, 2014; Cohen, 2007). This is rare in Sweet’s syndrome, but more common in the similar condition Behcet’s syndrome. It can potentially lead to retinal ischaemia, visual impairment and blindness.
  • Scleritis (Bilgin et al, 2015; Carbonell et al, 2011; Cohen, 2007; Zaouak et al, 2017).
  • Uveitis and panuveitis (Bancu et al, 2016; Cohen, 2007; Lobo et al, 2011; Matsumiya et al, 2012; Stevenson and Hannay, 2016). This is rare in Sweet’s syndrome, but uveitis is the most common eye condition in Behcet’s syndrome.

How are eye problems caused by Sweet’s syndrome diagnosed?

  • A doctor will ask you about your symptoms.
  • Your eyes will be examined.
  • Sometimes other investigations are needed, e.g. CT scan (computerized tomography), or an eye biopsy.

How are eye problems treated?

Treatment includes systemic steroids and/or eye drops (usually steroid eye drops) depending on which part of the eye is affected or the severity of the eye condition. However, other medications may be given.

If the eyes are dry, lubricant eye drops, otherwise known as ‘artificial tears’ can be beneficial.

Further information.

Behcet’s Syndrome Society (2018) Behcet’s Medical Factsheets (online). Accessed 07/04/19.

Ngan, V. (2002) Behcet Disease (online). Accessed 07/04/19.

NHS (2018) Dry Eyes (online). Accessed 07/04/19.

References.

Baartman, B., Kosari, P., Warren, C., Ali, S., Jorizzo, J., Sato, M. and Kurup, S. (2014) Sight-Threatening Ocular Manifestations of Acute Febrile Neutrophilic Dermatosis (Sweet’s Syndrome). Dermatology, Mar;228:193–197 (Karger).   

Bancu, L., Ureche, C., Craciun, N., and Marian, D. (2016) A case of Sweet’s syndrome associated with uveitis in a young male with ulcerative colitis. Romanian Journal of Morphology and Embryology, 57(3):1145-1147 (PubMed). In this case, the uveitis may be associated with the Sweet’s syndrome or ulcerative colitis.

Bilgin, A., Tavas P., Turkoglu, E., Ilhan, H., Toru, S. and Apaydin, K. (2015) An uncommon ocular manifestation of Sweet syndrome: peripheral ulcerative keratitis and nodular scleritis. Arquivos Brasileiros de Oftalmologia, Jan-Feb;78(1):53-5 (PDF).

Carbonell, D., Agdeppa, M. and Mejia, M. (2011) Peripheral Ulcerative Keratitis in Sweet’s Syndrome. Phillipine Journal of Ophthalmology, Jan-Jun; 36(1):46-49 (online).

Cohen, P. (2007) Extracutaneous Manifestations: Table 4. In Sweet’s syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis, Orphanet Journal of Rare Diseases (BMC).

Jang, Y., Jang, K., Kim, N., Khwarg, S. and  Choung, H. (2017) Blindness in Sweet’s Syndrome. Korean Journal of Ophthalmology, Dec;31(6):570-571 (PMC).

Khatri, M. and Taha. M. (2007) Sweet’s syndrome associated with myelodysplastic syndrome presenting as periorbital cellulitis. International Journal of Dermatology, May;46(5):496-9 (PubMed).

Koay, C., Chew, F., Chong, K. and Subrayan (2013) Compressive Optic Neuropathy. Indian Journal of Ophthalmology, Mar;61(3):140-141 (PMC).

Lobo, A., Stacy, R., Cestari, D., Stone, J., Jakobiec, F. and Sobrin, L. (2011) Optic nerve involvement with panuveitis in Sweet syndrome. Ocular Immunology and Inflammation, Jun;19(3):167-70 (PubMed).

Matsumiya, W., Kusuhara, S., Yamada, Y., Azumi, A. and Negi, A. (2012) Sweet’s syndrome with panuveitis resembling Behçet’s disease. Japanese Journal of Ophthalmology, May;56(3):268-72 (PubMed).

Morgan, K. and Callen, J. (2001) Sweet’s syndrome in acute myelogenous leukemia presenting as periorbital cellulitis with an infiltrate of leukemic cells. Journal of the American Academy of Dermatology, Oct;45(4):590-5 (PubMed).

Stevenson, R. and Hannay, J. (2016). Sweet’s syndrome: a rare extraintestinal manifestation of ulcerative colitis. BMJ Case Reports, May 11 (online).

Zaouak, A., Jrad, M., Bacha, T., Salah, M., Brahim, E., Hammami, E. and Fenniche, S. (2017) Sweet syndrome associated with bilateral nodular scleritis. Skinmed, Dec 1;15(6):491-493 (PubMed).

Zhuang, Y. and Li, Y. (2011) Bilateral Conjunctival Nodules in Sweet’s Syndrome. Journal of Clinical Experimental Ophthalmology; 2:146 (online).

2012-present, Sweet’s Syndrome UK

5 thoughts on “Can Sweet’s syndrome affect the eyes?”

  2. Ocular manifestations of dermal paraneoplastic syndromes, 2020. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7394149/

    – Ocular involvement in Sweet’s syndrome is ranged from 4% to 72%. Mainly conjunctivitis or episcleritis.
    – Also, scleritis, subconjunctival haemorrhage, glaucoma (inflammatory glaucoma), orbital as well as periorbital and eyelid inflammation, eruptions, dacryoadenitis, limbal nodules, peripheral ulcerative keratitis, iritis, choroiditis, retinal vasculitis and occlusive vasculitis.
    – Severe bilateral chemosis (swelling of the conjunctiva) as the first sign of recurrent Sweet’s syndrome in a patient with stable skin lesions and rapid improvement after steroid therapy started.
    – Case of bilateral panuveitis and papillitis with secondary macular oedema in the left eye in neuro-Sweet’s syndrome. This variant of Sweet’s syndrome usually presents with CNS involvement (meningitis or encephalitis).
    – In half of cases, just one eye is affected.
    – Eye symptoms usually appear at the same time or several days after skin lesions. However, there is a case of Sweet’s syndrome in which the patient developed nodular scleritis and peripheral ulcerative keratitis during the dermatologically inactive period (no skin lesions) of the disease. Treatment with fluorometholone 0.1%, ofloxacin 0.3% and artificial tear eye drops 5 times a day together with oral fluocortolone worked well.

    Like

    Reply

  4. Acute Exaggerated Meibomitis as Initial Manifestation of Sweet Syndrome, 2020. https://www.aaojournal.org/article/S0161-6420(20)30418-8/fulltext

    MEIBOMITIS OR POSTERIOR BLEPHARITIS, AN EYE SYMPTOM OF SWEET’S SYNDROME.

    Meibomitis is inflammation of the meibomian glands – glands in eyelids near the eyelashes. The glands normally produce free flowing oil, but when the gland stops working properly, the oil thickens and blocks the glands.

    – A 56-year-old man developed meibomitis – acute onset of inflammation and obstruction of meibomian glands in both eyes.
    – 2-day history of fever, skin lesions, and diarrhoea that developed at the same time as his eye symptoms.
    – Skin biopsy showed Sweet’s syndrome.
    – Symptoms vastly improved with systemic corticosteroids, doxycycline, and ophthalmic treatment.

    Like

    Reply

  5. Oral tacrolimus for ocular involvement in pediatric neutrophilic dermatoses, 2022. https://pubmed.ncbi.nlm.nih.gov/35242966/

    “Neutrophilic dermatoses (ND), including pyoderma gangrenosum (PG) and SWEET SYNDROME (SS), are rare in children. One study showed that only 2% of PG cases occurred in patients younger than 18 years old. While both disorders are associated with extracutaneous features, ocular (EYE) involvement is uncommon and often a diagnostic challenge. One French case series found ocular involvement in less than 15% of the 27 pediatric patients with PG or SS. Ocular manifestations include conjunctivitis, iritis, scleritis, and retinal vasculitis. Periorbital inflammation and eyelid lesions are also considered ocular manifestations, as eyelid lesions can lead to decreased visual acuity and occasionally vision loss. Most of the current literature describes ocular involvement in adults and highlights the risk of misdiagnosis as cellulitis or other bacterial infections, chalazion, or malignancy, resulting in inappropriate treatment. Early recognition and timely initiation of immunosuppressive therapy are imperative to prevent ocular damage and unnecessary antimicrobial exposure.”

    CASE 1: 15 year old girl. History of juvenile idiopathic arthritis and dermatitis herpetiformis. Sweet’s syndrome – persistent leg ulcer and conjunctivitis.
    CASE 2: 12 year old girl. History of juvenile idiopathic arthritis, presumed septic arthritis, “culture-negative cutaneous abscesses (including on the upper eyelids), and 1 episode of left eye periorbital cellulitis, all refractory to systemic antimicrobials.” PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum, cystic acne).

    Like

    Reply

Leave a comment