Autoimmune Clustering Sweet Syndrome, Hashimoto Thyroiditis, and Psoriasis
Acute febrile neutrophilic dermatosis (AFND; Sweet syndrome) is characterized by a constellation of symptoms and findings: fever, neutrophilia, and tender erythematous skin lesions that typically show an upper dermal infiltrate of mature neutrophils. Whereas some cases are idiopathic, others have been associated with a variety of disorders. In this report, we describe the occurrence of AFND with chronic lymphocytic thyroiditis (Hashimoto thyroiditis) and preexisting psoriasis. This is the first case report of the association of chronic lymphocytic thyroiditis with AFND from the United States and only the third reported in the world's literature. Because the coexistence of these disorders is rare, an underlying common pathogenic mechanism is a possibility. We postulate this to be CD4(+) T-cell dysfunction.
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ABSTRACT: To highlight the recent observations regarding not only research but also the clinical features and management of Sweet's syndrome.
Some of the new insights concerning Sweet's syndrome include: (1) bortezomib-induced Sweet's syndrome (some of which are the histiocytoid variant), (2) a rare extracutaneous manifestation of Sweet's syndrome with cardiovascular involvement including coronary artery occlusion, and (3) the possibility that photosensitivity may have a role in the pathogenesis of Sweet's syndrome.
Animal models of Sweet's syndrome and new associated medication have been observed. The definitive mechanism of pathogenesis still remains to be elucidated. Recent observations in paediatric patients suggest evaluation of dermatosis-related cardiac involvement in patients with post-Sweet's syndrome cutis laxa. Treatment advances include antitumour necrosis factor- alpha drugs; however, these medications have also been associated with inducing Sweet's syndrome. Nearly 50 years after the initial description of an acute febrile neutrophilic dermatosis by Dr Robert Douglas Sweet, the dermatosis remains a fascinating condition with regard to laboratory investigation, clinical manifestations and treatment.
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ABSTRACT: Sweet syndrome is a neutrophilic dermatosis with cutaneous tender lesions that can be associated with malignancies, infections, systemic inflammatory disorders, and medications. Although numerous studies have described Sweet syndrome, few studies have systematically investigated Sweet syndrome.
We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause.
A retrospective study was conducted to identify patients with Sweet syndrome evaluated at Mayo Clinic from 1992 to 2010.
Of 77 patients with Sweet syndrome (mean age of onset 57 years), 43 (56%) were male. Eighteen patients (23%) reported a preceding infection. A total of 41 (53%) patients were classified as having classic Sweet syndrome, 27 (35%) patients had malignancy-associated Sweet syndrome, and in 9 (12%) patients drug-induced Sweet syndrome was considered. In all, 21 patients had a hematologic malignancy or myeloproliferative/myelodysplastic disorder, whereas 6 patients had solid tumors. The mean hemoglobin level, in both male and female patients (P < .0443 and P < .0035, respectively), was significantly lower in malignancy-associated versus classic and drug-induced Sweet syndrome. Systemic corticosteroids were the most frequently used treatment (70%).
This is a retrospective study and represents patients from a single academic center.
Sweet syndrome is a distinctive disorder with certain clinical and histologic characteristics, which usually has a complete response to systemic corticosteroids. It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy.
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