Pulmonary and central nervous system involvement in Sweet's syndrome: a very rare case report

Sweet's syndrome is a multisystem inflammatory disorder characterized by painful, erythematous plaques and aseptic neutrophilic infiltration of various organs. The absence of vasculitis is a histological criterion for diagnosis, but recent reports suggest that vasculitis can occur in Sweet's syndrome. Involvement of the central nervous system and the pulmonary system is very rare. In this case study we describe a chronic alcoholic man with Sweet's syndrome associated with acute-onset encephalitis and severe pulmonary involvement. The patient's symptoms responded dramatically to steroid treatment, and notably, a skin biopsy of his lesions showed vasculitis.     

Non responding pneumonia with skin lesions

Sweet's syndrome (acute febrile neutrophilic dermatitis) is characterised by classical skin lesions accompanied by fever and malaise. Systemic involvement may be present and lung involvement in Sweet's syndrome has been reported in the form of bilateral pulmonary infiltrates, bronchiolitis obliterans organising pneumonia and pleural effusion. There are dense papillary neutrophilic infiltrates on histopathology. We present a case of Sweets' syndrome with left lower lobe consolidation and persistent fever which was non-responsive to antibiotics but showed clinical improvement with clearing of radiological opacities on oral steroid therapy.     

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.     

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.     

All-trans retinoic acid-induced erythema nodosum in acute promyelocytic leukemia

A 24-year-old woman with acute promyelocytic leukemia was treated with all-trans retinoic acid (ATRA) as a remission induction therapy. After pneumonia in the neutropenic period was successfully treated with antibiotic treatment, there was recurrence of high fever alone, followed by the appearance of erythema nodosum with pain in her upper limbs on day 25 of ATRA therapy. Skin biopsy neither revealed infiltration of leukemic cells nor suggested Sweet's syndrome. We considered the eruptions to be associated with ATRA, and prednisolone (30 mg/day for 5 days) was administered. Although the administration of ATRA was continued until complete remission of the leukemia, the erythema nodosum rapidly disappeared following short-term steroid therapy and no recurrence was observed. ATRA-induced erythema nodosum is rare, however it should be recognized as a possible adverse effect in ATRA therapy.     

Pulmonary involvement in acute febrile neutrophilic dermatosis (Sweet's syndrome)]

We encountered a 55-year-old man with pulmonary involvement in acute febrile neutrophilic dermatosis (Sweet's syndrome). He had been treated with steroids for Sweet's syndrome for 2 years, and on September 17, 1998 presented with a cough and a fever of 38.9 degrees C. Physical examination revealed fine crackles at the bases of both lungs. Chest radiography and computed tomography demonstrated reticular and nodular infiltrates in both lungs. Treatment with a variety of antibiotic agents and an antifungal agent was not effective. Sputum culture was sterile and bronchial washings were negative for infectious pathogens. Transbronchial biopsy revealed a mild chronic interstitial infiltrate and an inflammatory exudate in bronchiolo-alveolar tissue. The pulmonary lesions and cutaneous lesions were resolved by intradermal injections of triamcinolone acetonide in addition to oral prednisolone. Although the apparent neutrophilic infiltrates cited by earlier reports were not observed in transbronchial biopsy specimens, the clinical course in this case suggested that our patient had Sweet's syndrome with pulmonary involvement.     

IgG4-related skin lesions in a patient with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis

We describe a 60-year-old man with IgG4-related chronic sclerosing dacryoadenitis and sialoadenitis associated with lymphoplasmacytic and eosinophilic infiltration in erythematous nodules. Physical examination revealed left eye extrusion and small itchy nodules on the scalp and neck. The serum IgG level was 1,570 mg/dL, IgG4 463 mg/dL (29.5%), and IgE 4,554 IU/mL. Lacrimal gland biopsy disclosed prominent infiltrates of IgG4-positive plasma cells and scattered eosinophilic infiltrates with fibrosis, consistent with IgG4-related disease. A skin biopsy of a cutaneous nodule demonstrated that the infiltrated plasma cells around arterioles or venules in the deep dermis and subcutaneous fat tissue were strongly positive for IgG4. Although the swollen lacrimal and parotid gland and itchy subcutaneous erythematous nodules improved rapidly with oral prednisolone at a dose of 20 mg per day, the skin, lacrimal, and parotid lesions deteriorated simultaneously during steroid tapering and improved after increasing the dosage. As skin lesions are easy to biopsy, further study of the skin manifestations of IgG4-related disease will be important in further clarifying the clinical spectrum, pathophysiology and response to therapy of this disorder.     

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.     

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 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 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     

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.     

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.     

Sweet's syndrome followed by retinoic acid syndrome during the treatment of acute promyelocytic leukemia with all-trans retinoic acid.

We present here the case of a 49-year-old female with acute promyelocytic leukemia (APL) who, after first developing all-trans retinoic acid (ATRA)-related Sweet's syndrome, was later diagnosed as having retinoic acid (RA) syndrome. Preceding the RA syndrome diagnosis, she developed a fever as well as erythematous nodules on her upper arms. These symptoms were observed on day 18 of treatment with ATRA. Ten days later, she began to develop respiratory distress. There was no indication of infection, and her condition did not improve with empiric therapy. At this time, the diagnosis of RA syndrome was made, resulting in the initiation of steroid pulse therapy, and within 24 hours her elevated fever and respiratory distress improved markedly. In addition, the erythematous nodules gradually began disappearing. A skin biopsy revealed a dense dermal infiltrate consisting of neutrophils.     

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.     

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.     

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.     

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.     

A case of relapse with drug-susceptible M. leprae after multidrug therapy

A male born in 1930 was diagnosed as smear-positive borderline leprosy in 1971, and was treated with dapsone and/or sulfamethoxypyridazine from 1972 to 1980 with clinical improvement. However, new skin lesions with smears strongly positive appeared in August 1980, and he was diagnosed as having downgraded to lepromatous (LL) leprosy, but the bacilli recovered from the skin biopsy were fully susceptible to both dapsone and rifampin by mouse foot pad technique. Between 1981 and 1983, the patient was treated with 24 months of rifampin 600 mg and dapsone 100 mg daily, supplemented with prothionamide 500 mg daily during the initial 3 months, and his skin lesions gradually improved during treatment with the combined regimen. Afterward, the patient was kept under surveillance without treatment. From 1984 to 1986, his skin smears were negative, and no bacilli could be found from a skin biopsy taken in 1985. Then in 1987, 52 months after stopping treatment, new skin lesions appeared with a high concentration of Mycobacterium leprae (2 x 10(6)/mg tissue). The drug-susceptibility test again demonstrated that the organisms were fully susceptible to both dapsone and rifampin. Apparently the relapse was due to remultiplication of drug-susceptible persisters.