Sweet's syndrome associated with monosomy 7 myelodysplastic syndrome

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a reactive skin process frequently associated with inflammatory and neoplastic diseases, but particularly with hematologic malignancies. It usually precedes the underlying disorders for months or even years. Much of the evidence for this is based on a small series of case reports and reviews of the literature. Recently, immunological theories have suggested that helper T cell type 1 is involved in the pathogenesis of Sweet's syndrome. This process causes stimulation of the cytokine cascade, which may be responsible for the local and systemic activation of neutrophils and histiocytes. Clinically, Sweet's syndrome is characterized by an acute eruption of painful erythematous or violaceous plaques or nodules with fever, malaise, neutrophilic leukocytosis, and an elevated erythrocyte sedimentation rate. Peripheral blood neutrophilia is frequent and is one of the diagnostic criteria. However, 53% of patients with Sweet's syndrome linked to hematologic malignancies do not present any neutrophilia but rather granulocytopenia. Abnormal functioning of neutrophils is possible in many diseases. We report a case of a middle-aged male patient presenting Sweet's syndrome and granulocytopenia due to myelodysplasia and an anomalous chromosome seven (7-) with poor prognosis.     

Sweet's syndrome associated with G-CSF

Summary. Sweet's syndrome (SS) developed in two patients with acute myeloid leukaemia (AML) treated with granulocyte colony stimulating factor (G-CSF) for febrile neutropenia due to AML chemotherapy. Fever, painful skin and conjunctival lesions developed and neutrophilic infiltration was detected at biopsy specimens. Neutrophilia was not detected. Skin lesions regressed within 1–2 weeks and conjunctival lesions within 4 weeks following the cessation of G-CSF. We conclude that SS may be a complication of G-CSF therapy and tender skin and/or conjunctival lesions developing during G-CSF therapy should suggest the possibility of SS.     

Sweet's syndrome associated with sideroblastic anaemia

Abstract Previous reports have noted an association between Sweet's syndrome (acute febrile neutrophilic dermatosis) and leukaemia, and less commonly other haematological abnormalities. We report a previously unrecognised association between Sweet's syndrome (SS) and sideroblastic anaemia (myelodysplastic syndrome—refractory anaemia with ring sideroblasts). Both patients were males and one had prominent extracutaneous features of SS. Bone marrow cytogenetic studies were normal in this latter patient and neither patient showed progression to leukaemia.     

Cronkhite-Canada syndrome associated with myelodysplastic syndrome

We report a case of Cronkhite-Canada syndrome (CCS) associated with myelodysplastic syndrome (MDS). A 54-year-old woman, diagnosed as MDS the prior year after evaluation of anemia, visited our hospital with the chief complaint of epigastric discomfort. She also had dysgeusia, alopecia, atrophic nail change, and pigmentation of the palm, all of which began several months ago. Blood tests revealed severe hypoalbuminemia. Colonoscopy (CS) showed numerous, dense, red polyps throughout the colon and rectum. Biopsy specimens showed stromal edema, infiltration of lymphocytes, and cystic dilatation of the crypt. Her clinical manifestations and histology were consistent with CCS. We prescribed corticosteroids, which dramatically improved her physical findings, laboratory data, and endoscopic findings. This is the first report of CCS in a patient with MDS.     

Successful reduced-intensity stem cell transplantation in a patient with myelodysplastic syndrome combined with Sweet's syndrome

A 53-year-old male with myelodysplastic syndrome developed Sweet's syndrome extensively over his left iliac and inguinal regions that was refractory to standard treatment with corticosteroids and chemotherapy, received a stem cell transplant from an HLA-matched unrelated donor, conditioned by reduced-intensity regimen. The patient achieved complete hematological remission, and the cutaneous lesions improved gradually and then disappeared completely despite the patient receiving granulocyte colony-stimulating factor after transplantation and developing acute graft-versus-host disease.     

Sweet's syndrome associated with propylthiouracil-induced antineutrophil cytoplasmic antibody

A 44-year-old woman had tender erythematous nodules in both the upper and lower extremities, headache, and fever during the course of propylthiouracil therapy for Graves' disease. Serologic tests showed high titers of antineutrophil cytoplasmic antibody (ANCA) against myeloperoxidase (MPO). A skin biopsy showed neutrophilic dermatitis consistent with Sweet's syndrome. After the cessation of propylthiouracil therapy and the administration of steroids, all her symptoms disappeared and the titer of antineutrophil cytoplasmic antibody against myeloperoxidase decreased. A causal relationship between propylthiouracil (PTU) therapy and Sweet's syndrome is suggested.     

Sweet's syndrome associated with subacute necrotizing lymphadenitis.

A 34-year-old man with Sweet's syndrome associated with subacute necrotizing lymphadenitis is reported. Histological examination of an erythematous, painful, cutaneous plaque revealed a dermal interstitial neutrophilic infiltrate. A biopsy specimen obtained from an inguinal lymph node showed granulomatous formation, consisting of histiocytes, with central necrosis in the paracortex and macrophages in the sinus. Although the causes of the two diseases remain obscure, this appears to be the first report of Sweet's syndrome associated with subacute necrotizing lymphadenitis.     

Sweet's syndrome associated with propylthiouracil-induced antineutrophil cytoplasmic antibody

Abstract

A 44-year-old woman had tender erythematous nodules in both the upper and lower extremities, headache, and fever during the course of propylthiouracil therapy for Graves' disease. Serologic tests showed high titers of antineutrophil cytoplasmic antibody (ANCA) against myeloperoxidase (MPO). A skin biopsy showed neutrophilic dermatitis consistent with Sweet's syndrome. After the cessation of propylthiouracil therapy and the administration of steroids, all her symptoms disappeared and the titer of antineutrophil cytoplasmic antibody against myeloperoxidase decreased. A causal relationship between propylthiouracil (PTU) therapy and Sweet's syndrome is suggested.     

Interstitial pneumonitis associated with Sweet's syndrome in the elderly

Sweet's syndrome occurring during the course of interstitial pneumonitis in a 70-year-old woman was encountered. She was admitted because of dyspnea on exercise, dry cough and interstitial shadow on chest x-ray. Lung biopsy, together with other findings confirmed interstitial pneumonitis. Five days after admission, genital ulcer and aphtha on the oral mucosa were detected and exudative erythema appeared on her right shoulder, chest and back. Histological examination of the skin lesions demonstrated numerous nutrophilic infiltration in the upper dermis, indicating Sweet's syndrome. The skin eruption rapidly disappeared on treatment with colchicine. Although six months after admission interstitial pneumonitis caused respiratory failure, treatment with prednisolone and cyclophosphamide was effective. Serological and immunological tests demonstrated hyper-gammaglobulinemia and positive reaction for anti SS-A antibody. Pathological examination of the lip revealed numerous lymphocyte infiltrates around the duct of the minor salivary gland, suggesting Sj?gren's syndrome as the background disease of Sweet's syndrome and interstitial pneumonitis. This evidence indicating that even in elderly patients, skin lesions of Sweet's syndrome may reveal the background disease.     

Structural chromosomal abnormalities of 3q in myelodysplastic syndrome/acute myeloid leukaemia with Sweet's syndrome

Structural rearrangements in the long arm of chromosome 3, del(3)(q12q25) and t(3;5)(q21-25;q31-33), were observed in bone marrow cells from 2 patients with myeloid neoplastic disorders (myelodysplastic syndrome and acute myeloid leukaemia) and acute febrile neutrophil dermatosis (Sweet's syndrome). 3 of the 4 patients with leukaemia-associated Sweet's syndrome and acquired chromosome abnormalities known from the literature also had 3q changes, in 2 involving band 3q21.     

Sweet's syndrome associated with retinoic acid syndrome in a patient with promyelocytic leukemia

We report a case of Sweet's syndrome associated with retinoic acid syndrome in a patient with acute promyelocytic leukemia treated with all- trans retinoic acid (ATRA). Sweet's syndrome appeared on day 6 of ATRA therapy for promyelocytic leukemia. It was associated with a mild retinoic acid syndrome, an inflammatory syndrome occurring in 25% of patients treated with ATRA and characterized by features of capillary leakage with systemic inflammatory signs. The ATRA therapy was discontinued for 11 days and treatment with corticosteroids improved the systemic and cutaneous signs. Only 11 cases of Sweet's syndrome associated with ATRA have been previously reported in the literature, involving only the skin in eight cases, the skin and muscles in two cases, and the lung, kidney, fascia, and muscles in one case. Sweet's syndrome was followed by retinoic acid syndrome in one of these cases. The previously reported cases are reviewed, and the mechanisms of Sweet's and retinoic acid syndromes and the link between them are discussed.     

Pulmonary thromboembolism associated with myelodysplastic syndrome]

We present a 65-year-old female with myelodysplastic syndrome (MDS) who has attended our O.P.D. since 1983. In early December, 1990, dyspnea on effort developed which then progressed to dyspnea at rest at the end of December. She was admitted on January 8 with orthopnea. Chest X-ray films revealed loss of vascular shadows of the right lung. Blood gas analysis showed hypoxemia and hypocapnemia. Abnormalities in the coagulation-fibrinolytic system (increased TAT (thrombin-anti-thrombin III complex) and alpha 2-PIC (plasmin inhibitor complex)), possibly due to MDS, were detected. The diagnosis of pulmonary thromboembolism was made by pulmonary perfusion scintigram and pulmonary arteriography. After commencement of anticoagulation therapy on January 15, the subjective symptoms, blood gas analysis, pulmonary scintigram, and disorders of the coagulation-fibrinolytic system improved. The patient was discharged on March 5, 1991. The present case of myelodysplastic syndrome was associated with abnormalities of the coagulation-fibrinolytic system and was complicated by pulmonary thromboembolism.