Sweet's syndrome: Difference between revisions
 
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==Background==
==Background==
{{Skin anatomy background images}}
* Acute febrile neutrophilic dermatosis
* Acute febrile neutrophilic dermatosis
* Rare skin condition
* Rare skin condition
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* Classic Sweet syndrome affects women > men
* Classic Sweet syndrome affects women > men
* Unclear pathologenesis, but possibly factors include hypersensitivity reaction, cytokine dysregulation, or genetic susceptibility
* Unclear pathologenesis, but possibly factors include hypersensitivity reaction, cytokine dysregulation, or genetic susceptibility
===Etiologies===
* Classical Sweet syndrome, usually secondary to:
** Infection: Typically 1 to 3 weeks following [[upper respiratory infection]] or gastrointestinal infection
** Inflammatory bowel disease: [[Crohn's disease]] or [[ulcerative colitis]]
** [[Pregnancy]]
* Malignancy-associated Sweet syndrome
** Hematologic (~85%), most commonly [[acute myeloid leukemia]]
** Solid tumor (~15%), most commonly carcinoma of GU organs, breasts, GI tract
* Drug-induced Sweet syndrome, most commonly granulocyte-colony stimulating factor (G-CSF)
** Typically 2 weeks after drug exposure in patient without prior exposure
* [[Fever and rash]]


==Clinical Features==
==Clinical Features==
[[File:Sweet's Syndrome.png|400px|thumb|Sweet's Syndrome of index finger<ref>Cohen, P.R. Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2, 34 (2007). https://doi.org/10.1186/1750-1172-2-34</ref>]]
[[File:Sweet's Syndrome.png|400px|thumb|Sweet's Syndrome of index finger<ref>Cohen, P.R. Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2, 34 (2007). https://doi.org/10.1186/1750-1172-2-34</ref>]]
[[File:PMC1963326 1750-1172-2-34-10.png|thumb|Sweet's syndrome with lesions distributed in a sporotrichoid pattern.]]
[[File:Sweets.jpeg|thumb|Targetoid papules and plaques in Sweet's syndrome]]
* Cutaneous disease
* Cutaneous disease
** Painful, edematous, and erythematous papules, plaques, or nodules
** Painful, edematous, and erythematous papules, plaques, or nodules
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** Usually upper extremities > lower extremities, with asymmetrical distribution
** Usually upper extremities > lower extremities, with asymmetrical distribution
* Extracutaneous disease: neutrophilic infiltration of organ systems
* Extracutaneous disease: neutrophilic infiltration of organ systems
** Ocular infiltration (most common): conjuctivitis, scleritis, keratitis, iritis
** Ocular infiltration (most common): [[conjunctivitis]], [[scleritis]], keratitis, [[iritis]]
** Muscular system:  arthralgias, arthritis, myalgia
** Muscular system:  [[arthralgias]], [[arthritis]], [[myalgia]]
** Less commonly CNS, cardiovascular system, pulmonary system, liver, intestines, kidneys, bones
** Less commonly CNS, cardiovascular system, pulmonary system, liver, intestines, kidneys, bones
* [[Fever]] and leukocytosis
* [[Fever]] and [[leukocytosis]]


==Differential Diagnosis==
==Differential Diagnosis==
* Classical Sweet syndrome, usually secondary to:
*Infectious
** Infection: Typically 1 to 3 weeks following [[upper respiratory infection]] or gastrointestinal infection
**[[Adenovirus]]
** Inflammatory bowel disease: [[Crohn's disease]] or [[ulcerative colitis]]
**[[Arbovirus]]
** [[Pregnancy]]
**[[EBV]]
* Malignancy-associated Sweet syndrome
**[[Ehrlichiosis]]
** Hematologic (~85%), most commonly [[acute myeloid leukemia]]
**[[Enterovirus]]
** Solid tumor (~15%), most commonly carcinoma of GU organs, breasts, GI tract
**[[Fifths disease]]
* Drug-induced Sweet syndrome, most commonly granulocyte-colony stimulating factor (G-CSF)
**[[HHV 6]]
** Typically 2 weeks after drug exposure in patient without prior exposure
**[[Leptospirosis]]
* [[Fever and rash]]
**[[Lyme]]
**[[Meningococcemia]]
**[[Mycoplasma]]
**[[Parvovirus (B19)]]
**Primary [[HIV]]
**[[Psittacosis]]
**[[Rickettsia]]
**[[Rubella]]
**[[Rubeola]]
**Secondary [[syphilis]]
**[[Streptobacillus moniliformis]]
**[[Typhoid fever]]/[[typhus]]


*Noninfectious
**[[Acrodermatitis enteropathica]]
**[[Allergic reaction|Allergy]]
**[[Dermatomyositis]]
**[[Erythema marginatum]]
**[[Erythema multiforme]]
**[[Lupus]]
**[[Serum sickness]]
**[[Sweet's syndrome]]


==Evaluation==
==Evaluation==
===Workup===
* CBC
* CBC
** leukocytosis with neutrophilia
** leukocytosis with neutrophilia
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* CRP
* CRP


Major criteria
===Diagnosis===
====Major criteria====
* Abrupt onset painful erythematous plaques or nodules
* Abrupt onset painful erythematous plaques or nodules
* Histopathological evidence of neutrophilic infiltrate without evidence of leukocyoclastic vasculitis
* Histopathological evidence of neutrophilic infiltrate without evidence of leukocyoclastic vasculitis


Minor criteria
====Minor criteria====
* Fever
* [[Fever]]
* Associated malignancy, IBD, pregnancy, preceding URI, preceding GI infection
* Associated malignancy, [[IBD]], [[pregnancy]], preceding [[URI]], preceding GI infection
* Improvement with treatment with systemic steroids
* Improvement with treatment with systemic steroids
* Abnormal labs (3+ of following): ESR > 20, elevated CRP, leukocytosis, neutrophilic predominance
* Abnormal labs (3+ of following): ESR > 20, elevated CRP, leukocytosis, neutrophilic predominance


==Management==
==Management==
* Systemic glucocorticoids - prednisone 0.5 - 1.0 mg/kg per day with taper after symptom improvement
* Systemic glucocorticoids - [[prednisone]] 0.5 - 1.0 mg/kg per day with taper after symptom improvement
* Topical and intralesional corticosteroids - limited data, mostly used as adjunct, for mild disease (<5% BSA), or absence of systemic symptoms
* [[topical corticosteroids|Topical and intralesional corticosteroids]] - limited data, mostly used as adjunct, for mild disease (<5% BSA), or absence of systemic symptoms
* Alternatives agents include colchicine, dapsone, potassium iodide if steroids contraindicated
* Alternatives agents include colchicine, dapsone, potassium iodide if steroids contraindicated
* Referral to dermatology for biopsy
* Referral to dermatology for biopsy
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==External Links==
==External Links==
 
*https://dermnetnz.org/topics/acute-febrile-neutrophilic-dermatosis/


==References==
==References==
<references/>
<references/>
https://dermnetnz.org/topics/acute-febrile-neutrophilic-dermatosis/
[[Category:Dermatology]]

Latest revision as of 18:15, 11 December 2024

Background

Normal dermal anatomy.
  • Acute febrile neutrophilic dermatosis
  • Rare skin condition
  • Usually affects age 30-60, but can happen in any age group
  • Classic Sweet syndrome affects women > men
  • Unclear pathologenesis, but possibly factors include hypersensitivity reaction, cytokine dysregulation, or genetic susceptibility

Etiologies

  • Classical Sweet syndrome, usually secondary to:
  • Malignancy-associated Sweet syndrome
    • Hematologic (~85%), most commonly acute myeloid leukemia
    • Solid tumor (~15%), most commonly carcinoma of GU organs, breasts, GI tract
  • Drug-induced Sweet syndrome, most commonly granulocyte-colony stimulating factor (G-CSF)
    • Typically 2 weeks after drug exposure in patient without prior exposure
  • Fever and rash

Clinical Features

Sweet's Syndrome of index finger[1]
Sweet's syndrome with lesions distributed in a sporotrichoid pattern.
Targetoid papules and plaques in Sweet's syndrome
  • Cutaneous disease
    • Painful, edematous, and erythematous papules, plaques, or nodules
    • Often exhibit a mamillated surface with significant superficial dermal edema leading to pseudovesicular quality
    • Usually upper extremities > lower extremities, with asymmetrical distribution
  • Extracutaneous disease: neutrophilic infiltration of organ systems
  • Fever and leukocytosis

Differential Diagnosis

Evaluation

Workup

  • CBC
    • leukocytosis with neutrophilia
    • anemia (more common in malignancy or drug associated)
    • platelet abnormalities (more common in malignancy or drug associated)
  • Complete metabolic panel
  • Urinalysis
  • ESR
  • CRP

Diagnosis

Major criteria

  • Abrupt onset painful erythematous plaques or nodules
  • Histopathological evidence of neutrophilic infiltrate without evidence of leukocyoclastic vasculitis

Minor criteria

  • Fever
  • Associated malignancy, IBD, pregnancy, preceding URI, preceding GI infection
  • Improvement with treatment with systemic steroids
  • Abnormal labs (3+ of following): ESR > 20, elevated CRP, leukocytosis, neutrophilic predominance

Management

  • Systemic glucocorticoids - prednisone 0.5 - 1.0 mg/kg per day with taper after symptom improvement
  • Topical and intralesional corticosteroids - limited data, mostly used as adjunct, for mild disease (<5% BSA), or absence of systemic symptoms
  • Alternatives agents include colchicine, dapsone, potassium iodide if steroids contraindicated
  • Referral to dermatology for biopsy
  • Evaluation for malignancy (if clinically appropriate)
    • If other concerning symptoms and no other explanation for diagnosis

Disposition

  • Usually outpatient with close dermatology follow up

See Also

External Links

References

  1. ↑ Cohen, P.R. Sweet's syndrome – a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2, 34 (2007). https://doi.org/10.1186/1750-1172-2-34
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