MOFFITT AM REPORT PEARLS 10/24/16: Sweet Syndrome!

Hi Everyone! Thanks to Kenny for presenting a SWEET case of a young woman with multiple prior malignancies presenting with monoarticular swelling and erythema, possibly concerning for Sweet syndrome! There were 30148592 other things we could have talked about in this case, but we’ll focus on Sweet syndrome for today.

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Top pearls:

  • Sweet syndrome is acute febrile neutrophilic dermatosis
  • Characterized by abrupt onset painful palpable skin lesions
  • 3 types: Classical, Malignancy-associated, and Drug-associated
  • Treatment is corticosteroids (topical or systemic based on severity)

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For those who want more info:

Abrupt appearance of painful papules, plaques, or nodules, with fever and leukocytosis

Inflammation of eyes, MSK system, and internal organs may occur

According to some authors, there are 3 types:

  • Classical Sweet syndrome (idiopathic), majority of cases
    • 1-3 weeks after URI or GI infection
    • IBD-associated
    • Pregnancy-associated
  • Malignancy-associated Sweet syndrome
  • Drug-induced Sweet syndrome

Dx criteria.jpg

The first two criteria (major criteria) are necessary for the diagnosis, in addition to two of the remaining (minor) criteria.

Criteria for drug-induced Sweet syndrome are similar but involve establishing a temporal relationship between the culprit drug and symptom onset/resolution.

Malignancy-associated Sweet syndrome is more likely with hematologic malignancies than with solid tumors (approx. 85% vs 15% of cases). AML is the most common associated malignancy.

The list of drugs associated with Sweet syndrome is long, but commonly used drugs include furosemide, hydralazine, diazepam, TMP-SMX, nitrofurantoin, carbamazepine, abacavir, PTU, azathioprine, and G-CSF, among several others.

Epidemiology: Adults aged 30-60 is most common age group, 80% women for classical Sweet syndrome, 50-60% women for malignancy-associated Sweet syndrome.

Skin lesions most common on upper extremities, head, neck, trunk, and back.

Treatment is based on severity but is always steroids:

  • Mild (<5% BSA involvement and mild systemic sx): high potency topical steroids or intralesional steroid injections
  • Moderate-severe: Systemic steroids. Alternative therapies include colchicine, dapsone, or potassium iodide
  • Refractory cases: IV steroid pulse or other immunomodulation therapy

Some pictures of classic skin findings (Remember, these are painful! Itching is rare.):

*Photos from VisualDx and Uptodate

Evernote: https://www.evernote.com/shard/s272/sh/6353e1c7-6108-4392-9c2d-eacaeab3e773/22a51b3817e52af36a89c90839e155ed

Have a great day everyone!

SamMy

 

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