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Case Note

Case Note: Painful Nodules on the Arms and Legs

History

A 53-year-old female, with no significant past medical history, presents for management of painful nodules on the arms and legs.

Initial evaluation

  • Tired-appearing female
  • No significant past medical history and not currently taking any medications
  • Presents for management ofpainful, juicy, indurated erythematous nodules on the arms and legs
    • Few scattered nodules on truck (without mucosal lesions)
  • Review of systems is notable for low-grade fever in association with the rash
  • Image 1
    Image 1

Enter your diagnosis

Diagnosis

  • A diagnosis of Sweet's syndrome is rendered

Initial Treatment

  • Skin biopsy performed to confirm diagnosis
  • Concern for an inflammatory condition or malignancy triggers diagnostic evaluation
    • Age-appropriate cancer screening, chest X-ray, and blood tests (complete blood count with differential, erythrocyte sedimentation rate, and rheumatologic serologies)
    • A colonoscopy to rule out inflammatory bowel disease is deferred due to lack ofgastrointestinal symptoms
  • Follow up in 1 week

Follow-up evaluation strategy

1-week follow-up evaluation:

  • Rash has not progressed but also shows no improvement
  • Blood tests indicate mild anemia and thrombocytopenia and slightly elevated erythrocyte sedimentation rate; the remainder of her tests and imaging are normal
  • The patient is initiated on systemic prednisone 1 mg/kg daily and given instructions to take vitamin D and calcium supplements
  • Follow up in 1 week

Subsequent 1-week follow-up evaluation:

  • Patient reports significant clinical improvement within 48 hours of prednisone use
  • Patient's prednisone is tapered over the next 6 weeks - with recurrence of symptoms
    • Symptoms remit following escalation of prednisone dose
    • Dapsone initiated and prednisone successfully tapered off
  • Patient remains stable on dapsone

Subsequent treatment steps:

  • Dapsone (as indicated in this case note) and colchicine, may be effective as first-line treatment in mild cases or as an adjunctive treatment when steroid-sparing agents are necessary
    • Dapsone: 100-200 mg/day
    • Colchicine: 0.6-1.2 mg once to twice daily (as tolerated due to the diarrhea)
  • In severe or refractory disease, cyclosporine A (5-10 mg/kg/day) may induce a response
  • Infliximab 5 mg/kg/day at Weeks 0, 2, and 6 or etanercept 50 mg twice weekly may be considered as rescue therapy in patients failing those treatments mentioned above
  • Other effective agents that may be used alone or in combination with other medications when needed for refractory cases:
    • Minocycline 100-200 mg/day; clofazamine 100 mg twice daily; thalidomide 100-400 mg/day (for 2-4 weeks, then tapered if the patient responds); mycophenolate mofetil 1-1.5 g twice daily (can be added to the combination of systemic steroids or cyclosporine and may benefit some refractory patients); and potassium iodide

General discussion

Indurated, often painful, erythematous nodules are the characteristic skin lesion that marks Sweet's syndrome. Skin lesions may be bullous or pseudocellulitic in appearance. Skin lesions of Sweet's syndrome are often accompanied by signs of systemic inflammation: Fever, malaise, arthralgias, and blood markers of inflammation such as leukocytosis or elevated erythrocyte sedimentation rate. Extracutaneous manifestations of Sweet's syndrome may occur.

The diagnostic criteria for Sweet's syndrome have been proposed in the literature, though remain somewhat non-specific. To meet diagnostic criteria, a patient must have both of the major criteria (abrupt onset of painful erythematous plaques/nodules; and neutrophilic dermal infiltration without leukocytoclastic vasculitis) and meet two of the five minor criteria (preceding fever/infection; accompanying fever, arthralgia, conjunctivitis, or underlying malignant lesion; leucocytosis; good response to systemic corticosteroids [not to antibiotics]; and increased erythrocyte sedimentation rate).

Sweet's syndrome may be idiopathic (estimated 70% of cases) or seen in association with other systemic illnesses, including malignancy, systemic inflammatory disease, infection, pregnancy, and pathergy. The relationship between Sweet's syndrome and its associated conditions is complex. Flares of Sweet's syndrome do not always occur in concordance with flares of the underlying disease. In some cases, a neutrophilic dermatosis may represent a flare of the underlying disease itself, as in rare cases of systemic lupus erythematosus. In other cases, Sweet's syndrome has a pattern of relapse and remittance that is dissociated from its underlying condition and requires distinct therapeutic considerations.

Sweet's syndrome is almost universally responsive to treatment with systemic corticosteroids; however, given the chronic intermittent nature of the disease, it may require alternative immunosuppression with steroid-sparing agents. Anti-neutrophilic agents often are effective in the management of Sweet's syndrome.

Further reading

Brinster NK, Nunley J, Periser R, Horvath B. Nonbullous neutrophilic lupus erythematosus: a newly recognized variant of cutaneous lupus erythematosus. J Am Acad Dermatol 2012;66:92-7.

Dabade TS, Davis MD. Diagnosis and treatment of the neutrophilic dermatoses (pyoderma gangrenosum, Sweet's). Derm Therapy 2011;24:273-84.

Schadt CR, Callen JP. Management of neutrophilic dermatoses. Derm Therapy 2012;25:158-72.

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