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Síndrome de Sweet | Acute febrile neutrophilic dermatosis

AKA Acute febrile neutrophilic dermatosis
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Dermatoses Neutrofílica
 
Etiologia
  • Idiopathic (50%)
  • Infectious
    • Post-infectious (group A Strep)
  • Malignancies(10-20%)
    • Paraneoplastic (AML) - Associação com doença hematológica (leucemias)
    • IgA gammopathies
    • Timoma (caso clínico)
    • More likely associated with malignancy in bullous forms
  • Drugs
    • Tempo de latência ~1 semana
    • Mainly granulocyte growth factors (ex. GM-CSF)
    • all-trans-retinoic acid
    • Antineoplastics (bortezomib, FLT3 inhibitors, immune checkpoint inhibitors, imatinib, decitabine, azacitidine)
    • Antibiotics (TMP-SMX, minocycline, quinolones)
    • Abacavir, azathioprine, furosemide, hydralazine, IFN alpha, NSAIDs
  • Pregnancy → no risk to fetus
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Apresentação clínica
  • Skin
    • Placas eritematosas edematosas
    • Erythematous, juicy dermal plaques
    • May pseudovesiculate, be bullous and ulcerate
    • Typically affects face/neck/upper extremities
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  • Systemic
    • Fever, leucocytosis, arthralgias, myalgias and ocular involvement are common
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Course: 5-12 weeks, but 30% may relapse
 
Histologia
  • Dense perivascular neutrophilic infiltrate, subepidermal edema and leukocytoclasia
  • Location: superficial dermis
  • Usually without vasculitis
  • Varians
    • Histiocytoid
      • Histologia mostra células mononucleadas (habitualmente linfócitos ou histiócitos), mas que marcam para MPO indicando que são neutrófilos imaturos
      • DDx Leukemia Cutis
    • Lymphocytic
    • Eosinophilic
 
Diagnóstico
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DDx
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Treatment
  • Treat underlying disease or withdrawal of drug
  • 1st line: systemic corticosteroids
    • Oral prednisolone 0,5-1mg/kg for 2-6 weeks
    • Prolonged low dose prednisone for 2-3 months may be necessary
    • However, may be contraindicated in the case of systemic infection
  • NSAIDS
  • Cyclosporin