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
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.