Sweet's syndrome in a patient with relapsing polychondritis and myelodysplastic syndrome

Abstract

A 71-year-old Japanese man noticed some painful lesions on his ear and nose with fever in July 2010. He was diagnosed with relapsing polychondritis (RP) and oral prednisolone was commenced at 20 mg/day. While the amount of prednisolone was gradually reduced, he was diagnosed with disseminated non-tuberculous mycobacterial infection by Mycobacterium intracellular in March 2012 and three-drug combination therapy was started. Despite this therapy, fever and skin eruptions appeared and he was referred to our Department of Dermatology in August 2013. He presented asymptomatic nodules on his neck, upper limbs, and trunk. Cultures of the skin lesions for aerobic and anaerobic bacteria, fungi, and mycobacteria were all negative. Histological findings of the nodules demonstrated diffuse neutrophilic infiltration with nuclear dust in the dermis without vasculitis. Thus, he was diagnosed with Sweet's syndrome. Soon after the diagnosis, a bone marrow aspiration revealed myelodysplastic syndrome (MDS). After the dose of prednisolone was increased, the skin eruptions improved temporarily. However, after that he had recurrent flares of multiple invasive erythematous plaques and nodules on his face, neck, trunk, and extremities and he died following respiratory dysfunction in January 2014. Although three different types of skin eruptions occurred during only four months, histological findings of all the biopsies showed dense neutrophilic dermal infiltration which is typical of Sweet's syndrome. We report here a case of Sweet's syndrome in a patient with RP and MDS.