Sweet Syndrome

A syndrome 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. Evolution can be favorable or recurrent. Associated diseases are frequent.

(Figure)

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

There are four groups of Sweet Syndrome:

• Classic/Idiopathic
• Parainflammatory
• Paraneoplastic
• Pregnancy Associated

The malignant form represents 20% of cases. Pediatric cases account for 8%.

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

Hundreds of cases have been described.

Unclear; Sweet Syndrome seems 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. Tumor necrosis factor is thought to be implicated, as well as type 1 helper T cells.

Clinically evocated by abrupt fever associated with nodules followed by headache; myalgias and arthralgias are common.

Skin lesions characteristically affect primarily the face and the extremities in an asymmetric distribution: reddish blue or violaceous papules, plaques that can coalesce into circinate or arcuate plaques, nodules, subepidermal edema pustules. Ulcers and bullae are more common in malignancy-associated disease. A pathergy phenomenon is often associated. Lesions could be mucosal (conjunctivitis), pulmonary (chronic cough or pulmonary infiltrates on chest radiographs), renal (proteinuria, hematuria, and decreased creatinine clearance), skeletal (sterile chronic recurrent multifocal osteomyelitis), or central nervous system (CNS) (cerebrospinal fluid pleocytosis). Other clinical features can include splenomegaly, storage liver disease, thrombocytopenia, and anemia. Associated diseases are frequent: malignancies (myelodysplasia, chronic myelogenous leukemia, acute myeloid leukemia, lymphoma, genitourinary cancers) and systemic disorders (Crohn Disease, Ulcerative Colitis, Sjögren Syndrome, Behçet Disease, Lupus Erythematosus, Rheumatoid Arthritis, and undifferentiated connective-tissue disease).

Evaluate pulmonary function (clinical, chest radiographs, pulmonary function test if necessary); renal function (clinical, echography); and hepatic function (clinical, biochemical, coagulation, echography). Preoperative laboratory investigations should include full blood count, electrolytes, serum glutamic-oxaloacetic transaminase (SGOT), serum glutamic-pyruvic transaminase (SGPT), urea, and creatinine. Evaluate associated diseases.

Tolerance of anemia has to be evaluated preoperatively and should require transfusion. Regional anesthesia is not contraindicated but platelet count should be obtained preoperatively and pathergy, i.e., all associated allergic morbid manifestations, should be considered.

Perioperative fluid regimen and anesthetic drugs dosages should be adapted to renal function. Preoperative stress doses of steroid should be given if necessary. Avoid drugs that trigger Sweet Syndrome.

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