Neutrophilic pustulosis associated with chronic myeloid leukemia: a special form of Sweet's syndrome. Report of two cases.
Two subjects with Ph-positive chronic myeloid leukemia (CML) in whom pustular Sweet's syndrome was diagnosed are reported. The first patient was a 47-year-old woman who developed fever, painful ulcers of the oral mucosa and vagina and generalized pustulous skin lesions 2 years after the diagnosis of CML. Histologically, the skin lesions consisted of dense neutrophilic infiltrates with perifollicular disposition. The microbiologic studies were negative. The lesions showed a favorable response to corticosteroids, but fever recurred with every attempt of tapering prednisone; it finally disappeared with the addition of oral cyclophosphamide. The second patient was a 45-year-old man who developed fever and disseminated pustules with histologic features consistent with Sweet's syndrome and negative microbiologic studies at 2.5 years after diagnosis of CML. The picture showed a dramatic response to prednisone and did not recur after the drug was discontinued. In both patients, CML remained stable after resolution of Sweet's syndrome.
Feliu
E
E
Postgraduate School of Hematology Farreras Valenti, Barcelona, Spain.
Cervantes
F
F
Ferrando
J
J
Puig
S
S
Mascaro
J M
JM
Rozman
C
C
D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000150:complications*; D008297:Male; D008875:Middle Aged; D009504:Neutrophils / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D016463:Sweet Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D003520:Cyclophosphamide; D011241:Prednisone