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Sweet described the entity that now bears his name, termed by him acute febrile neutrophilic dermatosis. In his initial description, lesions were frequently associated with infection, particularly an upper respiratory infection. Subsequently, associations with malignancy (particularly myeloid hematologic malignancy) and with a variety of drugs were noted. Some, including this author, believe Sweet syndrome represents one end of a spectrum of neutrophilic dermatoses that includes atypical pyoderma gangrenosum in the middle and classic pyoderma gangrenosum on the other end. Some researchers have identified vascular inflammation in classic Sweet syndrome (Arch Dermatol 2002; 138:345).
These authors summarized their clinical experience with 77 patients seen over 18 years at a large tertiary care facility. They excluded 36 patients, some of whom were given alternate diagnoses (vasculitis, 7; pyoderma gangrenosum, 2; and erythema nodosum, 1). Twenty-seven patients had an associated malignancy, 21 hematologic. Complete response to therapy occurred in 82% of patients; systemic corticosteroids were the most frequently administered agent, with dapsone given to those who had a recurrence with prednisone. Only one patient had no response, which might imply that the disease was acute and nonrelapsing in most patients.
Rochet NM et al. Sweet syndrome: Clinical presentation, associations, and response to treatment in 77 patients. J Am Acad Dermatol 2013 Jul 23; [e-pub ahead of print]. (http://dx.doi.org/10.1016/j.jaad.2013.06.023)
Comment
In this large group of patients, the incidence of hematologic malignancy parallels my experience. In my practice, however, many of the patients with hematologic malignancy — particularly myelofibrosis — have recurrent or chronic disease, and some have chronic disease requiring the use of steroid-sparing agents. Although these researchers report use of Curth's criteria for assessing the relationship to cancer, they do not discuss concurrent onset or parallel course of the solid tumors with Sweet syndrome. They eliminated patients with vascular inflammation, which may align with the original classification scheme, but current concepts of “pustular vasculitis of the dorsal hands” and atypical pyoderma gangrenosum question the notion that vascular inflammation rules out a Sweet syndrome diagnosis. The authors note the importance of conducting an extensive workup for malignancy in patients with Sweet syndrome and anemia, but they do not specify what such a workup might entail.