Sweet's syndrome associated with cellulitis

A 38-year-old woman developed an acute, painful cutaneous eruption on her lower legs, neck, chest and fingers while receiving a number of oral and intravenous antibiotics for left leg cellulitis caused by trauma. Clinically and histopathologically, she was diagnosed with Sweet's syndrome with typical pseudovesicular skin changes in conjunction with erythema nodosum-like lesions on her lower legs. She responded quickly to oral prednisolone. We propose Staphylococcal cellulitis as a cause of Sweet's syndrome.     

累及皮下组织的Sweet综合征

报告1例Sweet综合征.患者女,28岁.发热6d,躯干及四肢结节3d.皮肤科检查:躯干、四肢及臀部多发直径1～3 cm的红色水肿性结节,压痛明显.皮损组织病理检查:真皮上部毛细血管内皮细胞明显肿胀,血管周围大量淋巴细胞及中性粒细胞浸润,真皮深部及邻近皮下脂肪中性粒细胞呈团块状浸润,可见明显核碎裂.诊断:Sweet综合...     

Sweet's syndrome: a spectrum of unusual clinical presentations and associations

BACKGROUND: Sweet's syndrome (SS) is the prototypic neutrophilic dermatosis. First described in 1964, the characterization of new clinical associations, unique histopathological findings and clinical variants have stimulated much interest and discussion recently. However, the prevalence of these unusual variants and clinical associations within a single cohort of patients, has not been described. OBJECTIVES: To describe and evaluate the prevalence of unusual clinical and histopathological features, as well as the clinical associations of SS seen in patients from the National Skin Centre, Singapore. METHODS: This is a retrospective study of all consecutive cases of SS seen at our centre over a 5.5-year period (June 1999-December 2004). Data on associated systemic diseases was obtained from the medical records and matched with information from the National Cancer Registry, Singapore. Patients not actively followed up for more than 3 months were contacted for their updated health status. RESULTS: Thirty-seven patients were identified. Ten (27%) had non-idiopathic SS. These were associated with haematological disorders, connective tissue disorders, infections or a drug. Twenty-nine patients (78%) had at least one atypical clinical or histopathological feature. Atypical clinical features included bullous lesions, SS with hand involvement or neutrophilic dermatoses of the hands and the concomitant existence of subcutaneous SS with pyoderma gangrenosum. SS was the presenting feature in three patients with infections caused by atypical organisms, including Mycobacterium chelonae, Penicillium species and Salmonella type D. Unique histopathological variants included subcutaneous SS and lesions containing an admixture of mature and immature neutrophils. Subcutaneous neutrophilic inflammation seemed to be more common in patients with an underlying haematological disorder. This group of patients also had a lower mean haemoglobin level. CONCLUSIONS: Unusual clinical and histopathological variants of SS described in the literature are similarly encountered in our cohort of patients, with some features being more common than others. We highlight and discuss some unique clinical and histopathological observations seen in our patients with SS.     

皮下型Sweet综合征

报告1例皮下型Sweet综合征.患者女,58岁.眼睑、四肢红色结节伴发热1周.皮肤科检查:左上眼睑一直径2 cm的红色结节,边缘隆起似假水疱;前臂伸侧、小腿伸侧及腕、肘、膝、踝部可见十余处直径1～3 cm的红色结节,有压痛,无破渍,无渗出.皮损组织病理检查:表皮、真皮大致正常,脂肪小叶大量中性粒细胞浸润,可见白细胞碎裂.诊断:皮下型Sweet综合征.     

Sweet's syndrome associated with skin methicillin-resistant Staphylococcus epidermidis infection

A 53-year-old Chinese man presented with fever, a painful exudative plaque around his left eye and subsequent multiple tender plaques and nodules on his bilateral face, neck and back. He had a 12-year history of refractory anemia, a subtype of myelodysplastic syndrome. Repeated bacterial cultures of exudates from the lesion of the left zygomatic area showed growth of methicillin-resistant Staphylococcus epidermidis. A biopsy of a plaque on the back showed edema of the papillary dermis and marked neutrophilic infiltrates in the upper dermis. The patient was diagnosed with Sweet's syndrome (SS), and responded well to combination therapy of amoxycilline/sulbactam and corticosteroids. To our knowledge, this is the first reported case of SS associated with skin S. epidermidis infection.     

Sweet's syndrome associated with systemic lupus erythematosus: A case report and review of the literature

A 35‐year‐old Japanese female patient with systemic lupus erythematosus (SLE) presented with fever, erythematous papules and nodules, and polyarthralgia. Skin biopsy of a nodule was compatible with Sweet's syndrome. The papules/nodules were well treated with an oral glucocorticoid. Thirty cases of Sweet's syndrome associated with lupus erythematosus (LE) have been reported in the published work. The mean age was 34.2 years. They showed a higher male ratio (male : female, 1:2) compared with patients with SLE (1:9) and Sweet's syndrome (1:3.7). Sweet's syndrome may occur as a manifestation of LE, and a moderate dose of an oral glucocorticoid will result in a good response.     

Sweet's syndrome associated with Behçet's disease

A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with Behçet's disease occurring in a 48 year old woman is reported. She fulfilled the diagnostic criteria for both Sweet's syndrome and Behçet's disease. There have been several reports of this association. Herpes simplex virus was isolated from a genital ulcer in our patient. The possible role of herpes simplex virus in this interesting association of Behçet's disease and Sweet's syndrome is discussed and a review of the literature is made.     

Clinical features and outcomes of Sweet's syndrome associated with non‐tuberculous mycobacterial infection and other associated diseases

Sweet's syndrome (SS) is associated with various diseases including non‐tuberculous mycobacterial infection (NTM). Recent reports have shown that SS associated with NTM is increasing. Clinical features of SS associated with NTM may be different from SS associated with other associated diseases. The aim of the present study was to compare clinical parameters and treatment outcomes of SS associated with NTM and other associated diseases. Patients from January 2004 to April 2014 diagnosed with SS were retrospectively enrolled. Clinical variables were compared between SS patients with and without NTM infection. There were 51 SS patients during the study period; 36 patients (70.59%) had NTM. Clinical variables between the NTM and other associated diseases were comparable: age, sex, and pattern and locations of skin lesions. Five laboratory factors were significantly different between the groups including white blood cell counts (NTM 25 800 vs 12 850 cells/mm³), lymphocyte percentages (13.0% vs 18.7%), monocytes (3.0% vs 7.2%), blood urea nitrogen (BUN) (11.7 vs 8.1 mg/dL) and serum creatinine (Cr) (1.0 vs 0.7 mg/dL). The presence of markedly high white blood cell counts, a low percentage of mononuclear cells and high BUN/Cr levels in SS may be a clinical clue to recognize the association with NTM infections; particularly in dissemination.     

Treatment of Sweet's syndrome in pregnancy

Pregnancy‐associated Sweet's syndrome is a rare occurrence (2%), with good prognosis, spontaneous resolution after delivery, and not increased infant morbidity and mortality. However, differential diagnosis is not easy for physician not familiar with skin lesions. Systemic involvement, even though unusual, might occur in nearly every organ of the body, including pericardium, myocardium, and placenta, as well as one report of early fetal miscarriage, questioning the possibility of risks underestimation. We present two further cases, one occurred in a 31‐year‐old woman at 26 weeks of gestation and the other on a 26‐year‐old woman at 24 weeks of gestation, primigravidae. Both presented with tender papules and nodules on their face and upper body parts. Laboratory examinations and skin biopsy histology were pathognomonic. Monitoring of general maternal and fetal conditions showed no signs of sufferance, but the decision to accelerate skin symptoms release, being time to delivery quite distant, challenge the treatment options. There are no recommended treatments for Sweet syndrome and the choice is very limited during pregnancy. A short course of oral steroids was very effective, with lesions healing in few days, no relapses or fetal complications. When pregnant patients exhibit fever, neutrophilia, arthralgia or myalgia, and tender erythematous plaques or nodules, Sweet syndrome should be considered. The trained dermatologist is in the leading position to address the differential diagnosis, reassure the patient, and avoid complications, even if they are rare.     

Drug-induced Sweet's syndrome in acne caused by different tetracyclines: case report and review of the literature

Sweet's syndrome was first described in 1964. It is characterized by an acute onset of non-pruritic, painful reddish nodules on the head and neck, chest and/or the upper limbs, mostly accompanied by fever, general malaise and leucocytosis. Histopathological examination shows a diffuse dermal neutrophilic infiltrate. The pathogenesis is still not fully understood, and different diseases have been shown to be associated with this syndrome. However, although still very rare, there is an increase of reports on Sweet's syndrome induced by drugs. We describe a 30-year-old man who experienced acute neutrophilic dermatosis after systemic treatment with minocycline. Additionally, there is a strong possibility that the same patient developed a drug-induced Sweet's syndrome after oral administration of tetracycline and doxycycline.     

Sweet's syndrome following surgery: Cutaneous trauma as a possible aetiological co‐factor in neutrophilic dermatoses

A 47‐year‐old man presented with an acute, cutaneous eruption of exquisitely painful papules at the operative site 4 weeks after a right tibial osteotomy. Initially this was managed as a postoperative wound infection; however the exacerbation and spread of the cutaneous eruption prompted further investigation. Histopathology and clinical findings were consistent with the development of Sweet's syndrome and resolution was obtained after the initiation of dapsone. We propose that surgery may produce an acute inflammatory response in a similar manner to pathergy reactions, which play an aetiological role in other neutrophilic dermatoses, such as pyoderma gangrenosum. We conclude that there may be greater overlap between these neutrophilic dermatoses than previously appreciated.     

Sweet's syndrome in an Australian hospital: a retrospective analysis

A retrospective analysis was performed of patients meeting the clinicopathological diagnosis of Sweet's syndrome identified over a 10-year period, in a metropolitan hospital. Nine patients met the inclusion criteria. Two patients had preceding streptococcal infection, two had associated inflammatory bowel disease, two had idiopathic Sweet's syndrome, and three had an underlying malignancy, one of whom was receiving granulocyte colony-stimulating factor. Although only three patients were found to have an elevated white cell count, erythrocyte sedimentation rate or C-reactive protein was elevated in all patients when measured. There was a wide spectrum of clinical presentations, with variations in lesion distribution and the presence/absence of systemic symptoms such as fever and arthralgia. Fever was observed in two-thirds of cases. Seven patients responded to oral corticosteroids, while one patient cleared spontaneously and one patient was lost to follow up. As erythrocyte sedimentation rate or C-reactive protein was elevated in all cases when measured, it may be a useful diagnostic tool.     

Azathioprine‐induced Sweet's syndrome: A case series and review of the literature

We present three patients with azathioprine‐induced Sweet's syndrome (AISS) who attended our tertiary institution within a 12‐month period. Established associations exist between Sweet's syndrome and some medications; however, to date links to azathioprine are tentative. While there are case reports of AISS, most have occurred in patients with inflammatory bowel disease (IBD), an underlying predisposition for Sweet's syndrome. Our case series adds to the evidence that the entity of AISS truly exists independent of confounding factors such as concurrent IBD.     

儿童Sweet综合征

报告1例儿童Sweet综合征。患儿男,7岁。因四肢疼痛性斑块伴间断性发热2个月就诊。皮肤科检查:四肢数十个大小不等、境界清楚的浸润性红斑或结节,部分红斑边缘隆起呈环状;左小腿见一10 cmx20 cm暗紫红色斑块,表面呈颗粒状,轻度水肿,浸润感明显,皮损触痛明显。血常规检查显示白细胞计数14.3×10~9/L.,中性粒细胞比例0.62,C反应蛋白29.0 mg/L,血沉67 mm/1h。皮损组织病理示真皮浅中层轻度水肿和弥漫性中性粒细胞浸润,血管壁未见纤维蛋白样坏死。结合临床、实验室检查和组织病理检查符合Sweet综合征诊断。     

Colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis

Neutrophilic dermatoses (ND) are characterized by epidermal and dermal infiltrates of polymorphonuclear leukocytes (PMN), without any infectious or other detectable agents as a cause. We describe a case of ND, which very rapidly improved upon colchicine treatment. The patient was a 67-year-old female with an 8-month history of dusky-red, tender, swollen plaques and nodules with superimposed vesicles and bullae on the buttocks, hands, and ankles associated with rheumatoid arthritis. The diagnosis of ND was made on the basis of the clinical and histopathological features of the skin lesions. She was treated with 0.6 mg of oral colchicine twice daily. Within one week, the erythematous plaques and arthralgia began to recede and disappeared within four weeks. In our case, colchicine seemed to improve the signs and symptoms of ND and appeared to be more effective than the other drugs we had used.     

Sweet syndrome associated with malignancies: A retrospective analysis of 25 patients from West China hospital

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is a rare inflammatory disorder characterized by sudden onset of variably tender brightly erythematous‐to‐violaceous papules, plaques, or nodules. To clarify the frequency, clinical course, therapeutic response, and outcome of Sweet syndrome with malignancy. We conducted a retrospective review of patients diagnosed with SS between 2009 and 2019 with a diagnosis of Sweet syndrome in West China hospital. Records were analyzed for variables, clinical features, etiological factors, laboratory results, and treatment outcomes. The 25 cases under study consisted of 14 (56%) females and 11 (44%) males. Mean age was 52.2 years (range: 4‐76 years). Nodules were the commonest lesion presentation (48%), involving mostly the lower limbs (52%). Pain of lesion was identified as the most common symptom (88%), followed by fever (76%) and cough (44%). Among the patients, 52% exhibited the classic form, 48% showed the malignancy‐associated form, and 4% exhibited the drug‐induced form in the setting of malignancy. Acute myeloid leukemia was the most common malignancy, observed in 5 of 12 patients (41.6%). Anemia (P = .002) was associated with malignancy (Chi‐square test).     

Complete response of deep neutrophilic dermatosis associated with myelodysplastic syndrome to 5-azacytidine

Cutaneous manifestations of myelodysplastic syndromes (MDS) may predict disease progression and a poorer prognosis. We describe a patient in whom a deep neutrophilic dermatosis preceded evolution of disease from refractory anaemia to RAEB (refractory anaemia with excess blasts) and resolved completely on treating the disease with 5-azacytidine. The dermatological response was accompanied by complete bone marrow remission and trilineage haematological improvement. We suggest that 5-azacytidine should be considered in the treatment of immune mediated cutaneous manifestations of MDS.