SWEET SYNDROME (ACUTE FEBRILE NEUTROPHILI C DERMATOSIS) OCCURRING DURING THE TRANSFORMATION FROM A MYELODYSPLASTIC SYNDROME TO ERYTHROLEUKEMIA

Citation
S. Grune et al., SWEET SYNDROME (ACUTE FEBRILE NEUTROPHILI C DERMATOSIS) OCCURRING DURING THE TRANSFORMATION FROM A MYELODYSPLASTIC SYNDROME TO ERYTHROLEUKEMIA, Deutsche Medizinische Wochenschrift, 121(30), 1996, pp. 939-942
Citations number
18
Categorie Soggetti
Medicine, General & Internal
Volume
121
Issue
30
Year of publication
1996
Pages
939 - 942
Database
ISI
SICI code
Abstract
History and clinical findings: Two months after being diagnosed as hav ing refractory anaemia with an excess of blasts in transformation (RAE B-T), a 62-year-old man presented in the emergency room with fever (40 degrees C) for two weeks and scattered deep-red macular indolent effl orescences over the chest, back, face and thighs. Other than splenomeg aly there were no significant findings on physical examination. Invest igations: Erythrocyte sedimentation rate was increased to 38 mm in the first hour. Haemoglobin concentration and platelet count were at the lower limits of normal, white cell count within the normal range. Diff erential count: 60 erythroblasts per 100 leukocytes and 33.5 blast cel ls. Two skin biopsies revealed massive oedema in the upper corium and focal erythrocyte extravasations. There were perivascular and perifoll icular inflammatory infiltrates in the deeper layers and elastosis of the cerium. There was no leucocytoclastic vasculitis. These findings e stablished the diagnosis of Sweet syndrome and erythroleukaemia. Treat ment and course: The erythroleukaemia was treated symptomatically and the skin changes gradually receded under prednisone, 1 mg/kg, but new spots occurred when the prednisone dose was halved. Candida oesophagit is occurred as a complication of the erythroleukaemia. Chest radiogram showed diffuse infiltrates in both upper lobes of the lung. Despite i ntensive antimycotic and antibiotic treatment the patient died 10 days later from pulmonary aspergillosis. Conclusion: This case report desc ribes the rare occurence of Sweet syndrome during the transformation f rom a myelodysplastic Syndrome to erythroleukaemia.