The syndrome was first described in 1964 by Robert Douglas Sweet. It was also known as Gomm–Button disease in honour of the first two patients Sweet diagnosed with the condition.[3][4][5]
Signs and symptoms
Pustular lesions with central necrosis on the left leg of a patient with Sweet's syndrome associated with Crohn's disease.Punch biopsy of a skin lesion showing neutrophilic infiltration in the dermis, with no evidence of vasculitis (same patient with Crohn's disease).
Acute, tender, erythematous plaques, nodes, pseudovesicles and, occasionally, blisters with an annular or arciform pattern occur on the head, neck, legs, and arms, particularly the back of the hands and fingers. The trunk is rarely involved. Fever (50%); arthralgia or arthritis (62%); eye involvement, most frequently conjunctivitis or iridocyclitis (38%); and oral aphthae (13%) are associated features.[citation needed]
Cause
SS can be classified based upon the clinical setting in which it occurs: classical or idiopathic SS, malignancy-associated SS, and drug-induced SS.[5]
Systemic diseases
SS is a reactive phenomenon and should be considered a cutaneous marker of systemic disease.
hematologic malignancies, solid tumors, pregnancy) is found in up to 50% of cases. Attacks of SS may precede the hematologic diagnosis by 3 months to 6 years, so that close evaluation of patients in the “idiopathic” group is required.[citation needed
]
There is now good evidence that treatment with hematopoietic growth factors—including granulocyte colony-stimulating factor (G-CSF), which is used to treat neutropenia, and granulocyte-macrophage colony-stimulating factor—can cause SS.[citation needed] Lesions typically occur when the patient has leukocytosis and neutrophilia but not when the patient is neutropenic. However, G-CSF may cause SS in neutropenic patients because of the induction of stem cell proliferation, the differentiation of neutrophils, and the prolongation of neutrophil survival.[citation needed]
Associations
Although it may occur in the absence of other known disease, SS is often associated with
Pregnancy associated Sweet syndrome typically present in first or second trimester. It may recur with subsequent pregnancy, but there seems to be no risk to the fetus.[citation needed]
A genetic association has been suggested,[6] but no specific genetic link has been identified.
In SS, inflammatory lesions are known to occur not only on the skin but also in various organs. When complications such as encephalitis or meningitis occur, it is referred to as neuro-Sweet disease.
corticosteroids often leads to favorable outcomes, and the frequencies of human leukocyte antigen (HLA) types B54 and Cw1 are notably high, indicating their association with the condition. However, it is suggested that multiple risk factors, including these, are involved in the onset of the disease. While the more frequent HLA type associated with a related disorder, neuro-Behçet's disease, is different (B51), it is believed to form a spectrum of disorders with other shared predisposing factors.[8]
] Recurrences are common and affect up to one third of patients.
Laboratory studies
Studies show a moderate neutrophilia (less than 50%), elevated
immunoglobulins within the vessel walls; red blood cell extravasation;inflammatory infiltration of vascular walls) are absent in early lesions.Perivasculitis occurs secondarily, because of cytokines released by the lesional neutrophils. True transmural vasculitis is not an expected finding histopathologically in SS.[citation needed
]
Definition
Sweet described a disease with four features: fever; leukocytosis; acute, tender, red plaques; and a
neutrophils. This led to the name acute febrile neutrophilic dermatosis. Larger series of patients showed that fever and neutrophilia are not consistently present.[citation needed] The diagnosis is based on the two constant features, a typical eruption and the characteristic histologic features;[citation needed
] thus the eponym "Sweet's syndrome" is used.
Treatment
Systemic
corticosteroids such as (prednisone) can produce rapid improvement and are the “gold standard” for treatment.[citation needed
] The temperature, white blood cell count, and eruption improve within 72 hours. The skin lesions clear within 3 to 9 days. Abnormal laboratory values rapidly return to normal. There are, however, frequent recurrences. Corticosteroids are tapered within 2 to 6 weeks to zero.Resolution of the eruption is occasionally followed by milia and scarring. The disease clears spontaneously in some patients. Topical and/or intralesional corticosteroids may be effective as either monotherapy or adjuvant therapy.
Oral potassium iodide or colchicine may induce rapid resolution.Patients who have a potential systemic infection or in whom corticosteroids are contraindicated can use these agents as a first-line therapy.
In one study,
indomethacin
, 150 mg per day, was given for the first week, and 100 mg per day was given for 2 additional weeks. Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours, and eruptions cleared between 7 and 14 days.
Patients whose cutaneous lesions continued to develop were successfully treated with prednisone (1 mg/kg per day). No patient had a relapse after discontinuation of indomethacin.
Other alternatives to corticosteroid treatment include
cyclosporine. All of these drugs influence migration and other functions of neutrophils.[citation needed
]
See also
Chloroma
List of cutaneous conditions
Periodic fever syndrome, also known as autoinflammatory diseases or autoinflammatory syndromes
^Hisanaga K, Iwasaki Y, Itoyama Y, the neuro-Sweet disease study group (2005). “Neuro-Sweet disease": Clinical manifestations and criteria for diagnosis. Neurology. 64: 1756-1761.
^Hisanaga K (2022). “Neuro-Behçet disease, neuro-Sweet disease, and spectrum disorders.” Intern Med. 61: 447-450.
