Rapid-onset febrile dermatosis

Severe Sweet's Syndrome with Elevated Cutaneous Interleukin-1β after Azathioprine Exposure: Case Report and Review of the Literature

“…When SS occurs in such patients, IBD is the first suspected cause of the syndrome, given the well-reported association [10,15]. However, in the last few years several papers reporting a possible association between AZA and SS have been published (table 2) [4,5,6,7,8,10,11,12,13,14,15,16,17]. Interestingly, two of the cases reporting AZA-associated SS had an underlying disease other than IBD, and rechallenge of patients with AZA reproduced SS lesions in all 6 cases rechallenged, both facts providing support for a potential role of AZA as a trigger/co-factor in certain cases of SS [4,7].…”

“…The majority thereof had biopsy or clinical features consistent with a neutrophilic dermatosis. Interestingly, withdrawal of AZA and rechallenge with the drug indicated that at least in some of the above-mentioned cases [4,6,7,11,12,14], a direct association between AZA and SS existed. In another case, on the other hand, the AZA dose was increased and the rash eventually resolved [41].…”

“…A very recent review of the literature up to the 30th of May 2014 identified 16 reported cases of AZA-induced SS (table 2) [4,5,6,7,8,10,11,12,13,14,15,16,17]. Analysis of these 16 reported cases shows an age distribution upon onset ranging from 9 to 89 years, an association of AZA-induced SS with IBD in 13 of 16 cases (81%), and an onset within the first month of AZA therapy (with an average time to onset of 33 days, range 7-330) in 15 of 16 patients (94%) (table 2).…”

“…More recently azathioprine (AZA), the nitroimidazole of 6-mercaptopurine, which is widely used as a corticosteroid-sparing agent in a variety of autoimmune inflammatory diseases, has been increasingly reported as a potential cause of SS [4,5,6,7,8,9,10,11,12,13,14]. Here we report a severe case of SS associated with AZA therapy, review the literature concerning similar cases reported to date and analyze the expression of the proinflammatory cytokine interleukin-1β (IL-1β) in our patient's skin lesions.…”

The cutaneous and systemic manifestations of azathioprine hypersensitivity syndrome

“…Most of the cases reported as neutrophilic dermatoses were from recent case reports in which a biopsy had been performed. [1][2][3][4][5][6]9,14 Several earlier case reports had more detailed descriptions of skin findings suggestive of neutrophilic dermatoses, but were not biopsy confirmed. [15][16][17] If the clinical cases suggestive of neutrophilic dermatoses were included with confirmed cases, then 88% (29/33) of patients presenting with cutaneous findings would have a neutrophilic dermatosis.…”