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Febrile neutrophilic dermatosis
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Febrile neutrophilic dermatosis
Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
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.
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]
SS can be classified based upon the clinical setting in which it occurs: classical or idiopathic SS, malignancy-associated SS, and drug-induced SS.
SS is a reactive phenomenon and should be considered a cutaneous marker of systemic disease. Careful systemic evaluation is indicated, especially when cutaneous lesions are severe or hematologic values are abnormal. Approximately 20% of cases are associated with malignancy, predominantly hematological, especially acute myelogenous leukemia (AML). An underlying condition (streptococcal infection, inflammatory bowel disease, nonlymphocytic leukemia and other 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]
Although it may occur in the absence of other known disease, SS is often associated with hematologic disease (including leukemia, most commonly acute myelogenous leukemia), solid cancers (especially genitourinary, gastrointestinal and breast cancer) and immunologic diseases including rheumatoid arthritis, and inflammatory bowel disease, Behçet's syndrome.
Pregnancy associated Sweet syndrome typically presents in the first or second trimester. It may recur with subsequent pregnancies, but there seems to be no risk to the fetus.[citation needed]
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Febrile neutrophilic dermatosis
Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a skin disease characterized by the sudden onset of fever, an elevated white blood cell count, and tender, red, well-demarcated papules and plaques that show dense infiltrates by neutrophil granulocytes on histologic examination.
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.
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]
SS can be classified based upon the clinical setting in which it occurs: classical or idiopathic SS, malignancy-associated SS, and drug-induced SS.
SS is a reactive phenomenon and should be considered a cutaneous marker of systemic disease. Careful systemic evaluation is indicated, especially when cutaneous lesions are severe or hematologic values are abnormal. Approximately 20% of cases are associated with malignancy, predominantly hematological, especially acute myelogenous leukemia (AML). An underlying condition (streptococcal infection, inflammatory bowel disease, nonlymphocytic leukemia and other 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]
Although it may occur in the absence of other known disease, SS is often associated with hematologic disease (including leukemia, most commonly acute myelogenous leukemia), solid cancers (especially genitourinary, gastrointestinal and breast cancer) and immunologic diseases including rheumatoid arthritis, and inflammatory bowel disease, Behçet's syndrome.
Pregnancy associated Sweet syndrome typically presents in the first or second trimester. It may recur with subsequent pregnancies, but there seems to be no risk to the fetus.[citation needed]
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