Sweet syndrome: a rare feature of ANCA-associated vasculitis or unusual consequence of azathioprine-induced treatment

A U Arun Kumar,Austin G Stack,Mohamed E Elsayed,Husham Mohamed,Alaa A Ali,Ahmed Alghali,Wael Hussein,Catriona Hackett,Niamh Leonard

doi:10.1186/s13223-018-0265-6

A U Arun Kumar, Austin G Stack + Show 7 more

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BackgroundSweet syndrome is a rare skin condition characterised by fever, neutrophilia, and tender erythematous skin lesions and has been reported to occur in association with anti-neutrophil cytoplasmic antibodies (ANCA) as well as complicate treatment with azathioprine therapy. Azathioprine, a relatively safe immunosuppressive, is frequently used to maintain disease remission in the treatment of ANCA-associated vasculitis. The occurrence of Sweet syndrome in a patient with ANCA-positive vasculitis and following treatment with azathioprine prompted us to present this clinical case and share this unusually rare occurrence. In doing so, we also wish to discuss current understanding of the disease and plausible associations.Case presentationHerein, we discuss the case of a 54-year old white male, who presented with features of ANCA vasculitis with haemoptysis, arthralgia, abnormal kidney function with active urine sediment and a positive p-ANCA titre. Standard immunosuppressive treatment with corticosteroids and intravenous rituximab resulted in disease remission. Due to significant steroid side effects, his steroid treatment was gradually tapered and switched to azathioprine over a 6-month period. Two weeks following initiation of azathioprine, he developed a painful maculo-papular erythematous skin rash and fever. A skin biopsy confirmed classical features consistent with Sweet syndrome. Withdrawal of azathioprine and treatment with oral corticosteroids and colchicine therapy resulted in complete resolution of the rash, although he continued to have high titres of MPO positive ANCA.ConclusionSweet syndrome is a rare adverse reaction to azathioprine but has also been reported to occur in association with ANCA vasculitis. The temporal association with azathioprine in our case and the relatively rapid resolution of the skin vasculitis upon its withdrawal suggested a primarily drug-induced reaction rather than an associated feature of ANCA vasculitis.

Highlights

Sweet syndrome is a rare skin condition characterised by fever, neutrophilia, and tender erythematous skin lesions and has been reported to occur in association with anti-neutrophil cytoplasmic antibodies (ANCA) as well as complicate treatment with azathioprine therapy
Anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA-V) is a group of diseases characterised by inflammation and necrosis of small and medium sizedblood vessels [1]
AZA is known to cause many adverse reactions including a well described entity known as azathioprine hypersensitivity syndrome (AHS), which commonly presents as an acute febrile neutrophilic dermatosis

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We report a case of Sweets syndrome following exposure to AZA in a patient with pre-existing ANCA vasculitis. It is prudent to consider AZA-induced Sweet syndrome in patients presenting with fever and rash within weeks of initiating AZA therapy after excluding infection, malignancy and acute flare of vasculitis. The role of ANCA antibodies cannot be completely ignored, and prudence would suggest that patients presenting with Sweets syndrome should be comprehensively investigated for underlying ANCA vasculitis. Authors’ contributions All authors participated in the clinical management of the patient and/or in collection of clinical data, and in the preparation of the clinical case report. Author details 1 Division of Nephrology, Department of Medicine, University Hospital Limerick, St Nessans Rd, Limerick, Ireland. 3 Division of Dermatology, Department of Medicine, University Hospital Limerick, Limerick, Ireland.

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