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
This young girl with painful skin lesions, mainly on her face
(coalescent) and arm, suffers from Sweet syndrome.
Acute Febrile Neutrophilic Dermatosis; Sweet Disease;
There are four groups of Sweet Syndrome:
- 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
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
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
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
Behçet Syndrome: Characterized by