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Sweet syndrome
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Acute and febrile dermatosis accompanied by neutrophilia. Rare: pediatric cases represent only 8 % of all cases, and they generally involve children aged less than 3 years. There are classic, malignant and drug-induced forms.
In the classical form , the skin eruption is often preceded by a respiratory or another infectious disease.
The diagnosis can be made when 2 major criteria are associated to 2 of the 4 minor criteria.
These diagnostic criteria are:
1) the onset of painful erythematous plaques (nodules, papules).
2) at skin biopsy: a dense neutrophilic infiltrate in the dermis without signs of vasculitis
1) body temperature > 38°C
2) association of the lesions to a local triggering phenomenon (pathergy)
3) a quick response to corticosteroid therapy
4) neutrophilic leukocytosis.
The skin symptomatology can be accompanied be arthralgia, myalgia and other extracutaneous manifestations: sterile osteomyelitis, aseptic meningitis, hepatitis, pancreatitis, renal, pulmonary or cardiac involvement.
The pathergy means that new papules or pustules appear in areas that have suffered a skin trauma shortly before (point of puncture of an IV line, for example).
In the malignant form, it appears to be a paraneoplastic syndrome that may precede or accompany the cancer (hematologic or digestive). In case of associated pulmonary involvement, there seems to be an increased risk for malignant hematological pathology (myelodysplasia).
In the drug-induced form, the causative factor is a treatment with: granulocytes growth stimulating factor, transretinoid acid, trimethoprim-sulfamethoxazole, azathioprine.
Anesthetic implications:
differential diagnosis: sepsis; corticosteroid therapy; check for extracutaneous involvements.
References:
- Arakaki R, Shofner JD, Kroshinsky D.
An infant with pulmonary-cutaneous Sweet syndrome.
J Pediatr 2012; 161: 959-61.
Updated: September 2018