Sweet's Syndrome: Systemic Signs and Symptoms and Associated Disorders

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Objective

To characterize the findings associated with acute febrile neutrophilic dermatosis (Sweet's syndrome [SS]) and the response of SS to treatment.

Design

We retrospectively reviewed 48 cases of SS encountered at the Mayo Clinic between 1980 and 1992.

Material and Methods

Histopathologic specimens and medical records were studied to determine initial manifestations, patterns of involvement, systemic signs and symptoms (including mucosal, musculoskeletal, hematologic, pulmonary, hepatic, and renal findings), and conditions associated with SS.

Results

In patients with SS, the typical manifestations are the acute onset of tender, erythematous or violaceous nodules or plaques in association with fever, leukocytosis, and dermal neutrophilia. In our study group, the cutaneous lesions most frequently involved the arms and legs. Of our 48 patients, 26 (54%) had a hematopoietic, plasma cell, or malignant disorder, and many of these patients had associated anemia, especially the male patients. No single laboratory finding specifically indicated an association with serious systemic disease. Most patients were treated with a tapering dose of prednisone, which yielded a good response.

Conclusion

Clinical acumen and appropriate laboratory tests are the main requirements for detection of hematologic disorders, internal malignant diseases, or other systemic conditions associated with SS.

Section snippets

METHODS

We retrospectively reviewed the Mayo Clinic medical records for 1980 through 1992 to find diagnoses of definite or possible SS. For the 63 cases identified, all historical data and histologic slides were thoroughly reviewed.

Fifteen of the 63 cases were excluded for the following reasons: (1) no lesions were present at the time of initial assessment at the Mayo Clinic (8 cases), (2) the histopathologic findings did not support the diagnosis (6 cases), and (3) few clinical features existed to

Results

The study group consisted of 28 women and 20 men. The age range was 26 to 79 years (mean ages, 56 in women and 58 in men).

The predominant lesions at the time of initial examination were multiple tender or painful erythematous or violaceous plaques (58%) or nodules (27%) of acute onset (Fig. 1). Vesicles or bullae, papules, urticarial lesions, pustules, ulcers, purpura, or hemorrhagic lesions (Fig. 2) were also noted. These less typical lesions were found in 26 of our patients, 14 of whom had a

DISCUSSION

Since the first description of SS, it has been recognized in numerous clinical settings. The spectrum of associated conditions reported in the world literature includes infections, hematopoietic or lymphoid disorders and malignant diseases, solid tumors, paraproteinemias, rheumatologic disease, inflammatory bowel disease, pregnancy, drug associations, and other systemic disorders.2, 3, 4, 5, 6, 7, 8, 9 Pathergy has also been previously noted in patients with SS.4, 9, 10 The patients in our

CONCLUSION

SS has been reported to occur in conjunction with infections, hematopoietic, lymphoid, and plasma cell disorders, malignant disease, and many other systemic conditions.2, 3, 4, 5, 6, 7, 8, 9 In light of the currently available information about SS, thorough assessment of patients with new-onset SS is imperative, especially when it occurs in combination with oral lesions, anemia, or a platelet count abnormality. Such an assessment should include a complete history and physical examination,

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