Long-term suppression of chronic Sweet's syndrome with colchicine∗

Abstract

Sweet's syndrome is generally self-limited and responds promptly to oral corticosteroid therapy. A middle-aged Thai woman presented with widespread tender, erythematous, edematous plaques on the trunk and extremities. A biopsy specimen demonstrated papillary dermal edema and a nodular and diffuse neutrophilic infiltrate without vasculitis; a diagnosis of Sweet's syndrome was made. The lesions responded promptly to oral prednisone. New skin lesions were noted during repeated attempts to taper the dose of prednisone. Complete blood cell counts during multiple episodes demonstrated an elevated neutrophil count without a left shift. Mild anemia was noted, but bone marrow examination findings were normal. Dapsone resulted in a good response initially, but the lesions recurred after several months, despite doses of 100 mg/d. The patient was treated with colchicine, 0.6 mg twice daily with good control of her skin lesions. Crops of lesions continued to occur as faint pink macules, but never evolved. Over the next 10 years, the patient required almost continuous colchicine treatment, with recurrence of tender skin lesions noted soon after discontinuation of the medication. Thirteen years after the onset of her skin lesions, the patient reports that her general health is good, and she has not experienced any systemic disease. Skin lesions continue to occur with regularity, but are now milder with little tenderness. She is no longer taking colchicine. Recurrences of Sweet's syndrome are common, but our patient's history of nearly unremitting skin disease over many years is unusual. The lesions were symptomatic, and the patient sought treatment. Colchicine provided effective long-term relief. Diarrhea and cramping were noted during the first few days of therapy, but the drug was well tolerated during the remainder of a prolonged course of therapy. Roughly 10% of cases of Sweet's syndrome are associated with leukemia, and the diagnosis of leukemia may be delayed up to a year after the initial presentation of Sweet's syndrome. Patients with leukemia are more likely to be anemic.1Bourke JF Keohane S Long CC Kemmett D Davies M Zaki I et al.Sweet's syndrome and malignancy in the U.K.Br J Dermatol. 1997; 147: 609-613Crossref Scopus (95) Google Scholar Although our patient was anemic at the time of her initial presentation, a bone marrow examination was unremarkable, and she has remained well. 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The views expressed are those of the authors and are not to be construed as official or as representing those of the Army Medical Department or the Department of Defense. Conflict of interest: None identified.