Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with agnogenic myeloid metaplasia

A 60-year-old man with a one-year history of agnogenic myeloid metaplasia was admitted to the hospital because of fever and a skin eruption. He had fever, anemia, hepatosplenomegaly, and a raised painful erythematous plaque in the face. The same kind of skin lesion developed thereafter at a venipuncture site in the left forearm. Bacterial cultures were negative. There was no response to antibiotic treatment. A biopsy specimen of the skin lesion revealed a dense dermal infiltration with mature neutrophils. A diagnosis of Sweet's syndrome was made. Fever and skin eruptions responded rapidly to prednisolone (PSL). Although the disease frequently recurred on rapid tapering of PSL, skin lesions cleared without scarring on a prolonged course of PSL. Four months after withdrawal of PSL, Sweet's syndrome recurred. A high dose PSL was given without benefit. He died of disseminated candidiasis.     

Case of granulocyte colony-stimulating factor-induced Sweet's syndrome.

A 33-year-old male was referred with a two-week history of fevers to 40 degrees C and painful, erythematous skin and oral mucosal eruptions that had failed to respond to multiple anti-infectious agents. He had a recent diagnosis of a "myeloproliferative disorder with myelodysplastic features" on bone marrow biopsy, with associated pancytopenia. Two weeks before admission, he had been treated with a course of granulocyte colony-stimulating factor (G-CSF) at a dose of 300 microg/day in an attempt to improve his neutropenia. After four days of treatment, the fever and lesions developed. Infectious evaluation was negative; however, biopsies of the skin and oral mucosal lesions revealed histology consistent with Sweet's syndrome. Intravenous methylprednisolone (30 mg/day) was started with prompt defervescence and resolution of the lesions within days. With the increasing use of G-CSF, Sweet's syndrome is becoming more commonly recognized as an adverse effect. This is the first case of G-CSF-induced Sweet's syndrome to demonstrate gingival involvement.     

Systemic manifestations of Sweet's syndrome: a case report

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.     

Lesions evoking Sweet's syndrome in acute leukemia: occurring during the stage of therapeutic aplasia

Acute febrile neutrophilic dermatosis, so called Sweet's Syndrome is a distinct dermatological disease defined by constant clinical and histological features: Eruption of painful, tender, raised erythematous plaques of face and neck with fever. Dense dermal infiltration with mature neutrophil polymorphs. Sweet's Syndrome may occur during the course of chronic or acute haematological diseases such as chronic myelogenous leukemia or acute non lymphoblastic leukemia. In all cases, the counts of Neutrophil Polymorphs were normal or above normal limits. We report a case of Sweet's Syndrome occurring during the aplastic period induced by the treatment of an acute myelo monocytic leukemia, and discuss the responsibility of white blood cells transfusion in genesis of typical histological aspect.     

Sweet's syndrome and malignancy

Acute febrile neutrophilic dermatosis (Sweet's syndrome) is characterized by pyrexia, neutrophilia, and the abrupt appearance of erythematous, painful, cutaneous plaques, primarily on the upper extremities, head, and neck. Histologically, the salient feature is a dense dermal infiltrate of neutrophils. Approximately 10 to 15 percent of published cases of Sweet's syndrome occurred in patients with cancer. This report reviews the 39 patients with malignancy-associated Sweet's syndrome described in the world literature and compares Sweet's syndrome in cancer patients with the idiopathic form of the disease. The most common associated malignancy was acute myelogenous leukemia. However, other myeloproliferative disorders, lymphoproliferative disorders, myelodysplastic syndrome, and carcinomas have been observed. Importantly, the diagnosis of Sweet's syndrome was often the presenting sign of a new or recurrent tumor. The presence of anemia, abnormal platelet counts, immature cells in the differential, and/or severe vesiculobullous or ulcerative cutaneous lesions is infrequent in idiopathic Sweet's syndrome and should alert physicians to the possibility of a more serious underlying disease. Extracutaneous manifestations may occur and most often involve the musculoskeletal system. Response to systemic steroids is dramatic in virtually all patients, regardless of the presence of malignancy.     

Ulcerative colitis and Sweet's syndrome: a case report and review of the literature.

A 47-year-old man with a history of ulcerative colitis on prednisone and azathioprine was admitted to the hospital with a four-day history of fever, skin rash, arthralgias and leukocytosis. A skin biopsy demonstrated neutrophilic infiltration of the dermis that was consistent with Sweet's syndrome. He improved after several days with an increase in his prednisone and azathioprine. Sweet's syndrome is a rare cutaneous manifestation of inflammatory bowel disease, with approximately 40 cases reported in the literature. In a previously reported case of a patient with ulcerative colitis-associated Sweet's syndrome who was on azathioprine at the time of the skin eruption, the azathioprine was stopped, raising the possibility of drug-induced Sweet's syndrome. In the present case, the azathioprine was actually increased with complete resolution of the skin manifestations. This would support the theory that immunosuppressive therapy is the mainstay of therapy for this condition. In conclusion, Sweet's syndrome is a neutrophilic dermatosis that is rarely associated with ulcerative colitis. It may occur while on immunosuppressive therapy and responds to an intensification of immunosuppression.     

Propylthiouracil-induced anti-neutrophil cytoplasmic antibodies and agranulocytosis together with granulocyte colony-stimulating factor induced Sweet's syndrome in a patient with Graves' disease

Propylthiouracil (PTU) is an antithyroid drug which is known to cause drug-induced vasculitis. PTU is implicated in 80-90% of cases of anti-neutrophil cytoplasm circulating antibody (ANCA)-associated vasculitis caused by anti-thyroid drugs which induce ANCA production. Sweet's syndrome is characterized by fever, leucocytosis, neutrophilia and the sudden onset of painful skin lesions. The pathology of the disease is still unclear. Cytokine dysregulation including interleukin-6 and endogenous granulocyte colony-stimulating factor (G-CSF) are thought to play a role in the pathogenesis of Sweet's syndrome. PTU and G-CSF are known to cause Sweet's syndrome and other neutrophilic dermatosis. The presence of ANCA can have a diagnostic value in Sweet's syndrome. Systemic corticosteroids are the first-line therapy for both diseases. Here we report a female patient with Graves' disease who developed ANCA and Sweet's syndrome after using PTU and G-CSF.     

Sweet's syndrome and sarcoidosis

In this review we summarize a number of cases of Sweet's syndrome (SS) associated with sarcoidosis that have been reported in the English literature. In all of the cases, the two disorders were diagnosed simultaneously. Patients with both disorders were younger and had a higher rate of fever than patients with SS alone. In this group of patients, we found a trend toward less skin involvement of the face and trunk, more involvement of the upper limbs, and more atypical skin lesions, particularly papules. The association of the two disorders seems to be more related to a subset of acute sarcoidosis (Lofgren's syndrome). All of the patients in this group had a benign course and self-limiting disease. Thus, SS in association with sarcoidosis could be considered a favorable prognostic factor. Although SS has a high rate of recurrence, no recurrence occurred in this group of patients during follow-up.     

Appearance of Sweet's syndrome in a patient with myelodysplastic syndrome (MDS) without relation to the hematological findings of MDS

Sweet's syndrome is known often to associate with non-lymphocytic leukemia (ANLL); however, there have been very few reports of Sweet's syndrome associated with myelodysplastic syndrome (MDS). It was reported that improvement and exacerbation of these two syndromes occurred simultaneously. We present here a 49-year-old male with Sweet's syndrome developed in RAEB in T. He complained of fever and infiltrative eruptions on the trunk and legs. At the time of admission to Tsukuba University Hospital, the peripheral blood showed leukocytopenia (WBC 2,000/microliter: Blast 9%, PMN 51%) and anemia (Hb 6.5 g/dl). Pseudo-Pelger anomaly of neutrophils was found on the blood smear. From the hematological findings and the result of skin biopsy, the patient was diagnosed as having MDS (RAEB in T) complicated by Sweet's syndrome. Prednisolone was effective to improve his fever and eruptions. However, when treated with low-dose Ara-C and when transformed into acute myelogenous leukemia, there was no correlation between the condition of Sweet's syndrome and the percentages of blasts in the marrow. We suggest that eruptions of Sweet's syndrome associated with MDS are not always a good index of exacerbation of MDS.     

A case of Sweet's syndrome in a patient with liver cirrhosis caused by chronic hepatitis B

Sweet's syndrome (SS), also known as acute febrile neutrophilic dermatosis, is characterized by the sudden onset of painful erythematous skin lesions together with fever and neutrophilia. SS can be associated with several disorders, such as malignancy, autoimmune disease, and infections. However, SS associated with liver cirrhosis is uncommon. We report a case of SS in a patient who was diagnosed with liver cirrhosis caused by chronic hepatitis B.     

Sweet's syndrome and accelerated phase of chronic myelogenous leukemia

 Sweet's syndrome is a neutrophilic dermatosis characterized clinically by raised, erythematous, tender lesions on the face, neck, upper thorax and extremities. Several diseases have been associated with this entity. We report a case of Sweet's syndrome associated with chronic myelogenous leukemia: a 48-year-old woman who developed recurrent skin lesions 3 years after the diagnosis of chronic myelogenous leukemia. Progression to an accelerated phase of the disease was detected 3 months after the beginning of the skin lesions. This case shows the convenience of evaluation and closer follow-up of patients with chronic myelogenous leukemia who develop skin lesions, especially if these lesions are recurrent. Received: 29 September 1999 / Accepted: 29 February 2000     

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 myelodysplasia: possible role of cytokines in the pathogenesis of the disease

Summary. The clinical course of a 56-year-old female patient with Sweet's syndrome (SS) preceded by a myelodysplastic syndrome (MDS) is described. During the acute phase of the disease with high remittent fever, painful skin lesions and maximal leucocytosis IL-6 and G-CSF serum levels were extremely high, while TNF-alpha was only slightly elevated and gamma-interferon and IL1-β were not increased. On clinical improvement IL-6 serum levels rapidly fell, whereas G-CSF values already slightly elevated before the manifestation of the disease slowly declined.
High G-CSF levels triggered by a yet unknown factor could explain the leucocytosis, neutrophilic dermatosis and skin lesions in SS, while IL-6 probably induced the associated clinical symptoms of fever and pain.     

Sweet's syndrome in a patient with Crohn's disease.

Crohn's disease is rarely associated with Sweet's syndrome. We report a 32-year old woman who presented with diarrhea, fever and disseminated erythematous plaques on the arms and the trunk. After colonoscopy with biopsies, Crohn's disease was diagnosed. Skin biopsy showed a dense infiltration of neutrophilic polymorphonuclear leukocytes, establishing also the diagnosis of Sweet's syndrome. Crohn's disease is one of several systemic diseases that may underlie Sweet's syndrome. Treatment with methylprednisolone resulted in a rapid improvement of both gastro-intestinal symptoms and skin lesions.     

Sweet's syndrome during the chronic phase of chronic myeloid leukaemia

We report the case of a 52 year-old male in the chronic phase of chronic myeloid leukaemia, with Philadelphia chromosome due to t(9;22) in the karyotype. He was treated with courses of busulfan and hydroxyurea. Fourteen months after initial presentation, the patient developed fever, non-productive cough, maculonodular violaceous painful skin lesions and bilateral pulmonary infiltrates visible on a chest roentgenogram. Laboratory data, repeated bone marrow aspiration and biopsy and karyotype analysis showed findings similar to those of the initial diagnosis. A biopsy taken from one of the trunk lesions was consistent with Sweet's syndrome. Oral methylprednisolone therapy was initiated at doses of 64 mg daily, and the skin lesions and fever were rapidly resolved. When we reduced the steroid dose, skin lesions and fever recurred. Two further courses of steroid therapy were given with similar results. Finally we treated him with naproxen (750 mg daily for 1 month) with a rapid and stable response. This drug should be considered as an alternative treatment for patients with Sweet's syndrome not responding to corticosteroids or for immunocompromised hosts.     

Sweet's syndrome in chronic lymphocytic leukemia associated with neutropenic fever and granulocyte colony stimulation factor

Granulocyte colony stimulation factor (G-CSF) is commonly used in the treatment of chemotherapy-induced myelosuppression. We report the case of a 62-year-old man with chronic lymphocytic leukemia who presented with neutropenic fever and sepsis. After treatment with G-CSF he developed Sweet's syndrome. Sweet's syndrome is a rare disorder but has been associated with cancer recurrence as well as administration of G-CSF. We present clinical and pathologic images that highlight the salient features of this entity.     

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.     

Recurrent tenosynovitis in Sweet's syndrome

SIR, An uncommon association of inflammatory polyarthritis isacute febrile neutrophilic dermatosis or Sweet's syndrome. Thishas the four cardinal features of fever, peripheral neutrophilicleucocytosis, painful plaque-forming inflammatory papules anda diffuse dermal neutrophilic infiltrate without vasculitis[1, 2]. There may be ocular, central nervous system, pulmonary,hepatic and renal involvement as well as oro-genital ulceration.Up to 62% of cases [3, 4] have musculo-skeletal manifestationsincluding myalgia, arthralgia and arthritis. Of the cases ofarthritis described in the literature [4–6], there noreports of associated tenosynovitis. The cause of Sweet's syndromeis unknown but it has an increased incidence in a number ofconditions, particularly infections, haematological     

All-trans-retinoic acid-induced myositis: a description of two patients

All-trans-retinoic acid (ATRA) induces complete clinical remissions in a high proportion of patients with acute promyelocytic leukemia and has become the standard induction therapy. Its use as a single agent results in short-lived remissions; thus, cytotoxic drugs are used for "consolidation" therapy. Side effects reported during treatment with ATRA include retinoic acid syndrome and Sweet's syndrome. Sweet's syndrome has been associated with acute myelogenous leukemia at presentation, but only two cases of Sweet's syndrome involving the musculoskeletal system in patients treated with ATRA have been described. We describe two additional patients with acute promyelocytic leukemia who had unexplained fever and myalgias (cutaneous lesions in one patient) during induction therapy with ATRA. Radiologic findings were similar to those in previously reported ATRA-associated Sweet's syndrome of the musculoskeletal system. The clinical course was characterized by a rapid resolution of the symptoms during treatment with dexamethasone. Recognition of the syndrome is important, especially considering the rapid resolution of symptoms after early institution of therapy with corticosteroids.