Abstract
Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a rare dermatosis characterized by painful papules and plaques accompanied by cutaneous infiltration with neutrophilic granulocytes. Bullous changes are observed in some cases. We report about a patient with osteomyelofibrosis who developed fever accompanied by painful plaques and confluent papules on both arms and thighs. The course of the disease was complicated by blistering and pulmonary infiltrates. After the diagnosis of bullous Sweet's syndrome was established, systemic therapy with glucocorticoids was successful in treating skin lesions and dyspnea.
Keywords:
Dermatoses; Extracutaneous manifestation; Glucocorticoids; Lung; Osteomyelofibrosis.
MeSH terms
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Aged
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Biopsy
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Bone Diseases, Developmental / diagnosis
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Bone Diseases, Developmental / drug therapy
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Bone Diseases, Developmental / pathology
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Combined Modality Therapy
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Diagnosis, Differential
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Erythrocyte Transfusion
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Glucocorticoids / therapeutic use
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Granulocyte Colony-Stimulating Factor / therapeutic use
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Humans
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Lung Diseases / diagnosis*
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Lung Diseases / drug therapy
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Lung Diseases / pathology
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Male
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Nitriles
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Primary Myelofibrosis / diagnosis
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Primary Myelofibrosis / drug therapy
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Primary Myelofibrosis / pathology
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Pyrazoles / therapeutic use
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Pyrimidines
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Skin / pathology
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Skin Diseases, Vesiculobullous / diagnosis*
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Skin Diseases, Vesiculobullous / drug therapy
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Skin Diseases, Vesiculobullous / pathology
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Sweet Syndrome / diagnosis*
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Sweet Syndrome / drug therapy
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Sweet Syndrome / pathology
Substances
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Glucocorticoids
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Nitriles
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Pyrazoles
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Pyrimidines
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Granulocyte Colony-Stimulating Factor
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ruxolitinib