Sweet Syndrome

At a glance

Acute febrile neutrophilic dermatosis is characterized by the abrupt onset of fever; arthritis; raised painful plaques on the limbs, face, and neck; neutrophilic leukocytosis; and dense dermal infiltration with mature neutrophilic polymorphs. Associated diseases are frequent.

Synonyms

Acute Febrile Neutrophilic Dermatosis; Sweet Disease; Gomm-Button Disease.

History

First described by Robert Douglas Sweet, an English dermatologist, in 1964.

Classification

Previously four groups of Sweet Syndrome were recognized:

• Classic/Idiopathic: This medical disorder appears spontaneously for no known reasons. It is often called Idiopathic Sweet Syndrome.

• Parainflammatory: A dysregulation in cytokine proteins, produced by immunological cells, are involved in inflammatory response and have been reported to play a role in the development of this medical condition. It is seen in individuals affected with Ulcerative Colitis Disease and Crohn’s Disease.

• Paraneoplastic: This medical condition has also been, less frequently however, associated with underlying cancer. The most usual form of malignancy associated with Sweet Syndrome are hematological, particularly leukemic. It has also been associated with breast cancer.

• Pregnancy-Associated: Sweet Syndrome can develop typically during the first or second trimester. It does not appear to increase fetal risk but it may recur with subsequent pregnancies.

It is now better divided into three more adapted situations consisting of:

• Classic: Sporadic manifestation is the most frequent mode of presentation of this medical condition.

• Malignant: Hematological malignancy are associated with this syndrome.

• Drug-Associated: An association with granulocyte-colony stimulating factor medication has been established with Sweet Syndrome. This type of medication is used to stimulate the production of neutrophils.

Incidence

Uncommon. Several hundreds of cases have been described. The malignant form represents 20% of cases. Pediatric cases account for 8% of all case reports. Female predilection in the classic and drug induced but not in malignant form. It is estimated that Classical Sweet Syndrome in adults affects women more than men by as much as 15:1 ratio.

Pathophysiology

Unclear. It is postulated that the pathogenesis of Sweet Syndrome is driven by helper T cells through the production of several cytokines, such as interleukin-1, interleukin-2, and interferon-gamma, as well as granulocyte colony-stimulating factor (G-CSF). It appears to be in some cases a response to systemic factors (hematological disease, infection, or drug exposure to granulocyte colony-stimulating factor, minocycline, Bactrim, lithium, furosemide, hydralazine, carbamazepine, and levonorgestrel/ethinyl estradiol). There is a neutrophil mediation, associated neutrophilia, and response to medications that impact neutrophil activity. Drugs implicated include G-CSF, all-trans-retinoic acid, trimethoprim-sulfamethoxazole, and azathioprine.

Diagnosis

Diagnostic criteria for classic Sweet Syndrome have been established and the diagnosis is made when the two major criteria are associated with at least two of the minor criteria:

• Major ...