Sweet's Syndrome


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  • Sweet’s syn patients may appear dramatically ill.
    Skin lesions may appear vesicle-like secondary to edema in the dermis, however, palpation confirms that they are solid nodules.
  • Both major plus 2 minor criteria are required.
  • Male=female incidence in malig-associated
  • If no alternative explanation for Sweet’s syn is found, it is reasonable to do a work-up for malignancy (especially looking for the most commonly associated ones above). Checking a CBC q6-12 months may be necessary as the syndrome can precede the diagnosis of hematologic malignancy by as long as a decade.
  • Other meds have only been described in a few case reports.
  • Cessation of causative medication or treatment of an underlying malignancy may be the only intervention necessary.
    Lesions of Sweet’s syndrome often become impetiginized so a course of antibiotics directed against Staph aureus often causes partial improvement in the dermatosis.
  • Sweet's Syndrome

    1. 1. Sweet’s SyndromeSweet’s Syndrome Allison DupontAllison Dupont AM ReportAM Report 1/17/061/17/06
    2. 2. DefinitionDefinition  Sweet’s syndrome (acute febrileSweet’s syndrome (acute febrile neutrophilic dermatosis) is characterizedneutrophilic dermatosis) is characterized by:by:  FeverFever  Peripheral neutrophiliaPeripheral neutrophilia  Painful red skin papules, nodules and/orPainful red skin papules, nodules and/or plaquesplaques  Neutrophilic infiltration of skin (particularlyNeutrophilic infiltration of skin (particularly the dermis)the dermis)
    3. 3. Clinical PresentationClinical Presentation  FeverFever (>38(>38˚C) can be intermittent and may precede skin˚C) can be intermittent and may precede skin manifestations by days to weeks.manifestations by days to weeks.  Systemic symptomsSystemic symptoms may include headache, myalgia,may include headache, myalgia, arthralgia, and general malaise.arthralgia, and general malaise.  Cutaneous lesionsCutaneous lesions consist of erythematous to violaceousconsist of erythematous to violaceous tender papules which may coalesce to form plaques.tender papules which may coalesce to form plaques.  The plaques are not pruritic.The plaques are not pruritic.  Most often found on the face, neck and upper extremitiesMost often found on the face, neck and upper extremities (especially the dorsum of the hands), but can occur anywhere.(especially the dorsum of the hands), but can occur anywhere.  Lesions on the lower extremities may resemble erythemaLesions on the lower extremities may resemble erythema nodosum.nodosum.
    4. 4. Clinical PresentationClinical Presentation  Oral ulcersOral ulcers (more common in patients(more common in patients with Sweet’s syndrome and a hematologicwith Sweet’s syndrome and a hematologic malignancy.malignancy.  Ocular involvementOcular involvement (uncommon in(uncommon in malignancy-associated and drug-inducedmalignancy-associated and drug-induced Sweet’s syndrome).Sweet’s syndrome).  Conjunctivitis, episcleritisConjunctivitis, episcleritis
    5. 5. Clinical PresentationClinical Presentation  Involvement of internal organs may occurInvolvement of internal organs may occur leading to:leading to:  AlveolitisAlveolitis  Sterile osteomyelitisSterile osteomyelitis  Involvement of liver, pancreas, and/orInvolvement of liver, pancreas, and/or kidneyskidneys  Neurologic and psychiatric changesNeurologic and psychiatric changes
    6. 6. Laboratory FindingsLaboratory Findings  Lab findings are nonspecific.Lab findings are nonspecific.  Majority will have peripheral neutrophilia.Majority will have peripheral neutrophilia.  Other possible lab abnormalities include:Other possible lab abnormalities include:  Elevated sedimentation rateElevated sedimentation rate  Elevated C-reactive proteinElevated C-reactive protein  LeukocytosisLeukocytosis  Consider evaluation of hepatic and renalConsider evaluation of hepatic and renal function.function.
    7. 7. PathologyPathology  Sweet’s syndrome characteristicallySweet’s syndrome characteristically involves dense neutrophilic infiltration ofinvolves dense neutrophilic infiltration of the dermis +/- dermal edema.the dermis +/- dermal edema.  Neutrophil karyorrhexis is commonlyNeutrophil karyorrhexis is commonly seen.seen.  There is no involvement of the vasculatureThere is no involvement of the vasculature of the skin and no necrosis (in contast toof the skin and no necrosis (in contast to pyoderma gangrenosum).pyoderma gangrenosum).
    8. 8. DiagnosisDiagnosis Major CriteriaMajor Criteria  Abrupt onset of painful erythematousAbrupt onset of painful erythematous plaques or nodules.plaques or nodules.  Histopathologic evidence of a denseHistopathologic evidence of a dense dermal neutrophilic infiltratedermal neutrophilic infiltrate withoutwithout vasculitisvasculitis.. Minor CriteriaMinor Criteria  Fever (>38Fever (>38˚C)˚C)  Association with an underlyingAssociation with an underlying hematological/visceral malignancy,hematological/visceral malignancy, inflammatory disease, or pregnancyinflammatory disease, or pregnancy oror preceded by an upper respiratory orpreceded by an upper respiratory or GI infection or vaccination.GI infection or vaccination.  Excellent response to treatment withExcellent response to treatment with systemic corticosteroids.systemic corticosteroids.  Peripheral neutrophilia (>70%Peripheral neutrophilia (>70% neutrophils)neutrophils)
    9. 9. Associated ConditionsAssociated Conditions  Sweet’s syndrome is associated with anSweet’s syndrome is associated with an underlying disease or condition in up to 50% ofunderlying disease or condition in up to 50% of patients.patients.  Sweet’s syndrome may be the presenting signSweet’s syndrome may be the presenting sign and the underlying disease may not becomeand the underlying disease may not become apparent for several years after Sweet’sapparent for several years after Sweet’s syndrome occurs.syndrome occurs.  There is a female predominance except in theThere is a female predominance except in the case of malignancy-associated Sweet’scase of malignancy-associated Sweet’s syndrome.syndrome.
    10. 10. Associated ConditionsAssociated Conditions 1.1. MalignancyMalignancy  Approximately 21% of patients with Sweet’sApproximately 21% of patients with Sweet’s syndrome have a malignancy.syndrome have a malignancy.  15% hematological (most commonly AML)15% hematological (most commonly AML)  6% solid tumors (most commonly carcinomas of6% solid tumors (most commonly carcinomas of the GU tract, GI tract, or breast)the GU tract, GI tract, or breast) 2.2. InfectionsInfections  Mostly of the upper respiratory or GI tractMostly of the upper respiratory or GI tract  Streptococcus, mycobacterium, Yersinia,Streptococcus, mycobacterium, Yersinia, Salmonella, ShigellaSalmonella, Shigella
    11. 11. Associated ConditionsAssociated Conditions 3.3. Inflammatory bowel diseaseInflammatory bowel disease  Sweet’s syndrome may occur alone or in combination withSweet’s syndrome may occur alone or in combination with pyoderma gangrenosum.pyoderma gangrenosum. 3.3. PregnancyPregnancy 4.4. Other conditions with a possible association:Other conditions with a possible association: -Sarcoidosis-Sarcoidosis -Rheumatoid arthritis-Rheumatoid arthritis -Thyroid disease (Grave’s-Thyroid disease (Grave’s disease and Hashimoto’s thyroiditis)disease and Hashimoto’s thyroiditis)
    12. 12. Drug-induced Sweet’s syndromeDrug-induced Sweet’s syndrome  Criteria slightly different than classicalCriteria slightly different than classical syndrome.syndrome.  Temporal relationship between drugTemporal relationship between drug ingestion/injection and clinicalingestion/injection and clinical presentation.presentation.  Resolution of lesions/symptoms afterResolution of lesions/symptoms after withdrawal of drug or treatment withwithdrawal of drug or treatment with corticosteroids.corticosteroids.
    13. 13. Drug-induced Sweet’s syndromeDrug-induced Sweet’s syndrome  G-CSF is responsible for the majority ofG-CSF is responsible for the majority of cases.cases.  Other possible causes: furosemide,Other possible causes: furosemide, lithium, hydralazine, trimethoprim-lithium, hydralazine, trimethoprim- sulfamethoxazole, and oral contraceptives.sulfamethoxazole, and oral contraceptives.
    14. 14. PathogenesisPathogenesis  Etiology of Sweet’s syndrome is unknown.Etiology of Sweet’s syndrome is unknown.  Presumed to be due to a hypersensitivityPresumed to be due to a hypersensitivity reaction to an eliciting antigen whichreaction to an eliciting antigen which leads to stimulation of cytokine release.leads to stimulation of cytokine release.  Cytokines precipitate neutrophilCytokines precipitate neutrophil activation and infiltration.activation and infiltration.  Response to treatment with corticosteroidsResponse to treatment with corticosteroids supports this etiology.supports this etiology.
    15. 15. PathogenesisPathogenesis  The source of the eliciting antigen may beThe source of the eliciting antigen may be diverse, including bacterial, viral ordiverse, including bacterial, viral or tumoral antigens.tumoral antigens.
    16. 16. TreatmentTreatment  Gold standard:Gold standard: Systemic corticosteroidsSystemic corticosteroids  Start at 1 mg/kg/day prednisone with longStart at 1 mg/kg/day prednisone with long taper (4-6 weeks) to 10 mg/day.taper (4-6 weeks) to 10 mg/day.  Many patients require several months ofMany patients require several months of 10-30 mg/day to suppress recurrences.10-30 mg/day to suppress recurrences.  Localized Sweet’s syndrome canLocalized Sweet’s syndrome can sometimes be treated with high-potencysometimes be treated with high-potency topical corticosteroidstopical corticosteroids..
    17. 17. TreatmentTreatment Other first-line agents include:Other first-line agents include: 1.1. Oral potassium iodideOral potassium iodide -Systemic symptoms resolve in 1-2 days.-Systemic symptoms resolve in 1-2 days. -Dermatitis resolves in 3-5 days.-Dermatitis resolves in 3-5 days. 2.2. ColchicineColchicine -Systemic symptoms resolve in 2-3 days.-Systemic symptoms resolve in 2-3 days. -Dermatitis resolves in 2-5 days.-Dermatitis resolves in 2-5 days.
    18. 18. Alternative therapiesAlternative therapies  IndomethacinIndomethacin  ClofazimineClofazimine  CyclosporineCyclosporine  DapsoneDapsone
    19. 19. ReferencesReferences Burall, Barbara M.D. “Sweet’s syndrome (acute febrile neutrophilic dermatosis)”.Burall, Barbara M.D. “Sweet’s syndrome (acute febrile neutrophilic dermatosis)”. DermatologyDermatology Online JournalOnline Journal 5(1):8.5(1):8. Cohen, Philip R. MD, Kurzrock, Razelle MD. “Sweet’s syndrome revisited: a review of diseaseCohen, Philip R. MD, Kurzrock, Razelle MD. “Sweet’s syndrome revisited: a review of disease concepts”.concepts”. International Journal of DermatologyInternational Journal of Dermatology. Volume 42, Issue 10. October 2003.. Volume 42, Issue 10. October 2003. Cohen, Philip R. “Sweet’s syndrome”.Cohen, Philip R. “Sweet’s syndrome”. OrphanetOrphanet. October. October 2003.2003. Federman et al. “Cutaneous manifestations of malignancy”.Federman et al. “Cutaneous manifestations of malignancy”. Postgraduate Medicine OnlinePostgraduate Medicine Online. January. January 2005.2005. Joe, Edwin K. MD. “Sweet’s syndrome”.Joe, Edwin K. MD. “Sweet’s syndrome”. Dermatology Online JournalDermatology Online Journal 9(4):28.9(4):28. Moschella, Samuel L. MD. “Neutrophilic dermatoses”.Moschella, Samuel L. MD. “Neutrophilic dermatoses”. UpToDateUpToDate.. ““Sweet’s Syndrome”.Sweet’s Syndrome”. Dermis.netDermis.net..