By Disease Name > Sweet's Syndrome

Sweet's Syndrome

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•AKA acute febrile neutrophilic dermatosis
•drug induced Sweet’s = G-CSF (most common)

 

clinical:

•fever (>100.4;  variable)
•leukocytosis (WBC 10-20;  neutrophils >6000 cells/µl)
•abrupt onset

 

cutaneous:

•the surface of the lesion is mamillated or may contain pustules  (“relief of a mountain range”)
•often pseudovesicular secondary to intense edema of the papillary dermis
•arms, neck, and face most commonly involved
•asymmetrically distributed (vs. EM)

 

Sweet’s is a reactive phenomenon and should be considered a cutaneous marker of systemic disease:

•~20% of cases associated with malignancy mainly hematopoietic malignancy (esp. AML)
•80% of patients are women with preceding URI
•pregnancy

 

Treatment:

•just as onset is abrupt, there is prompt and dramatic relief of both cutaneous and systemic symptoms with PO glucocorticoids or potassium iodide (or SSKI)
•colchicine may also give rapid relief

 

Histology:

•epidermis not involved
•category = nodular dermatitis: neutrophils predominate
•leukocytoclasia, yet no evidence of vasculitis (i.e. dust but no fibrin)
•Sweets, EED, and granuloma faciale are all on same spectrum

 

hmtoggle_plus1Histologic ddx:
•EED (but LCV)
•granuloma faciale (but LCV)
•pyoderma gangenosum
•leukemia cutis (immature neutrophils)
•neutrophilic eccrine hidradenitis (neutrophils around eccrine glands;  usually in patients with AML receiving induction chemotherapy)
•halogenoderma*
•abscess/ cellulitis (but positive cultures)*
•bowel bypass syndrome*

*last three diagnoses are clinically pustular

 

hmtoggle_plus1Todd's thoughts (if you care):
•Sweets and AML:  are the neutrophils in AML-associated Sweets atypical (i.e. malignant) and therefore this is just a distinctive form of leukemia cutis?  OR: AML, like all cancer, requires both induction and promotion/proliferation to occur, and just as induction can arise from one of many different mutations, promotion can be due to any number of factors. In the patients with AML who develop Sweet’s syndrome, perhaps the nature of the “promoter” is more global and therefore affects normal granulocytes to migrate to the skin ???