Acute febrile neutrophilic dermatosis (Sweet's syndrome) in association with myelodysplastic syndromes: a report of three cases and a review of the literature

Summary. Three patients with acute febrile neurophilic dermatosis (AFND, Sweet's syndrome) in association with myelodysplastic syndromes (MDS) are described and, in addition, another 10 published cases are reviewed. In the reviewed patients the male/female ratio is 2:1. The great majority of the patients had decreased or normal leucocyte counts at the time the syndrome developed. Chromosome analysis was available in eight cases; in three the karyotype was normal while five patients had a clonal abnormality. No clinical or laboratory parameters seemed to predict the response to steroids; all but one of the treated patients responded promptly. Moreover, three patients recovered spontaneously. Shortly after the appearance of Sweet's syndrome nine patients developed acute leukaemia and one patient died with hypoplasia. Development of Sweet's syndrome in association with MDS may not be an uncommon clinical finding. The occasional MDS patient developing AFND will often progress into acute leukaemia and thus the occurrence of AFND warrants a bone marrow examination and close follow-up of the patient.