Sweet syndrome


Sweet’s syndrome is an acute febrile neutrophilic dermatosis.

A reactive pustular dermatosis.

Characterized by a rash, fever, and systemic symptoms.

An uncommon inflammatory disorder characterized by the abrupt onset of painful skin lesions.

Categorized as drug induced, malignancy associated and idiopathic.

Underlying pathogenesis is probably multifactorial with a component related to cytokine dysregulation.

Requires two major criteria: the abrupt occurrence of edematous erythematous-to-violaceous cutaneous lesions and a biopsy specimen that shows superficial edema and a neutrophilic infiltrate, without vasculitis, and at least two minor criteria- such as an associated underlying condition or exposure, fever, leukocytosis, and a rapid response to systemic steroids.

Most patients, about 70%, have the classical type, which is an idiopathic disorder.

Those most commonly affected are middle aged women.

Its presentation ranges from occurring after a mild respiratory illness, to a more aggressive neutrophilic process, which may be associated with inflammatory diseases or malignancies, especially hematologic malignancies.

Also seen in solid organ cancers, upper respiratory or UGI infections, hepatitis C, HIV, inflammatory bowel disease, mycobacteria, fungi, or bacteria, pregnancy, autoimmune disorders,  in those who received vaccinations or other drugs such as G-CSF, all- trans retinoic acid, following CABG, and pregnancy.

Associated with the wide variety of drugs, most notably granulocyte-colony stimulating factor, developing within two weeks of exposure.

10-20% of patients have a malignancy, usually a hematologic process:most commonly AML and myelodysplastic syndrome.

Characterized by pathergy.

15% of adult cases associated with hematologic neoplasms.

Characteristically associated with a tender edematous reddened plaque, with or without pustules, that develop on the head, neck and upper torso.

Differential diagnosis for pustular rash with fever includes: cutaneous infections, Behçet’s disease , extra intestinal manifestation of Crohn’s disease, and cutaneous drug reaction.

May present with conjunctivitis.

Rash may become generalized.

Patients may be ill with malaise, fever, neutrophilic, elevated sedimentation rate, and an elevated C reactive protein.

Female predominance.

Usually preceded by an infection.

Associated with an abrupt onset, with histopathologic evidence of a neutrophilic infiltrate.

May be associated with an underlying autoimmune process or malignancy.

Can be associated with inflammatory bowel disease.

Malignancy associated syndrome has no sexual predilection.

Malignancy associated syndrome most commonly associated with hematological neoplasms.

Retinoic acid, hypomethylating agents associated in the presence of APL and myelodysplastic syndrome.

May be associated with drugs including G-CSF and all trans retinoic acid.

Anecdotal reports of association with minocycline, hydralazine and trimethoprin-sulfamethoxazole.

Prompt response to systemic corticosteroids.

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