Sweet's syndrome masquerading as facial cellulitis

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a cutaneous condition that typically occurs as tender red plaques or nodules. However, atypical presentations may occur and, in our case, Sweet's syndrome masqueraded as facial cellulitis and soft tissue infections of the extremities in a sporotrichoid pattern. Despite treatment with broad-spectrum antibiotics, the cutaneous lesions progressed. Results of skin biopsy specimens of the facial plaque and a nodule on the right upper extremity were diagnostic of Sweet's syndrome. Simultaneous to diagnosis, the patient also was found to have acute myelogenous leukemia (AML).     

Sweet's syndrome without granulocytosis

Background Sweet's syndrome (SS), acute febrile neutrophilic dermatosis, has been linked to hematologic malignancies and presents with characteristic edematous dermal plaques. Peripheral blood neutrophilia is frequently seen in association with SS and is one of the diagnostic criteria.
Objective To report the clinical, laboratory, and hematologic data of four patients with myeloid leukemia who developed SS after chemotherapy. Three of these patients were neutropenic.
Methods A retrospective study of four patients with SS and hematologic malignancies was undertaken. Three patients had de novo acute myelogenous leukemia and one was in the acute blast crisis of chronic myelogenous leukemia.
Results Sweet's syndrome was not originally suspected in these patients because of the low peripheral white blood cell counts caused by chemotherapy. All of the patients presented with fevers, arthralgias, and an eruption. They had been treated with antibiotics because of a presumed infection. Once the correct diagnosis was made and oral prednisolone was started, a rapid response followed.
Conclusions Sweet's syndrome should be considered in the differential diagnosis when acute myeloid leukemic patients develop skin lesions and unexplained fevers regardless of the peripheral blood counts.     

Sweet's syndrome revealing relapsing polychondritis

Sweet's syndrome is extremely rare in relapsing polychondritis. We report a rare case of Sweet's syndrome revealing relapsing polychondritis. A 77-year-old man presented with fever, associated with respiratory symptoms and non pruritic, painful, erythematous, papules and plaques mainly on the arms and legs. Skin biopsy of the arm found a neutrophilic dermal infiltrate consistent with Sweet's syndrome. A month and a half after admission, the patient presented with ear chondritis and dysphonia. Biopsy of the ear cartilage found a significant perichondral inflammatory cells infiltrate. The diagnosis of relapsing polychondritis was established and a steroid treatment was instituted with a complete regression of the cutaneous signs and chondritis.     

Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with hairy-cell leukemia

A case of hairy-cell leukemia that started with cutaneous lesions similar to those of acute febrile neutrophilic dermatosis (Sweet's syndrome) is reported. The patient had leukopenia and a recurrent eruption for a year prior to the diagnosis of the hematologic disorder. Bone marrow examination eventually demonstrated characteristic "hairy" cells with tartrate-resistant, acid-phosphatase activity. Biopsy of a cutaneous lesion suggested an abscess showing a dense neutrophilic dermal infiltrate with perivascular predilection. The findings of IgA, IgM, C3, and fibrinogen in vessel walls by immunofluorescence and vascular disruption by electron microscopy contribute additional evidence for an immunologic determinant in this instance of Sweet's syndrome. It is known that neutrophilic infiltrates of Sweet's syndrome may have a variety of clinical presentations. This is the second case of hairy-cell leukemia with which Sweet's syndrome was associated.     

大疱性Sweet综合征合并克罗恩病一例

患者,男,65岁.因腹泻1个月,全身红斑、大疱、血疱5天伴发热入院.皮肤组织病理示:表皮角化过度,局部表皮缺失,表皮下疱形成,真皮乳头高度水肿,真皮浅中层多数中性粒细胞浸润.结肠组织病理特征符合克罗恩病改变.结合临床及病理表现诊断为大疱性Sweet综合征合并克罗恩病.治疗:给予甲泼尼龙40 mg/d,发热逐渐消退,皮疹...     

A case of Sweet's syndrome developed after the treatment of herpes simplex infection in a metastatic breast cancer patient

BACKGROUND: Sweet's syndrome or acute febrile neutrophilic dermatosis is associated with several systemic diseases such as malignancies and infectious diseases. METHODS: We present a 34-year-old woman with Sweet's syndrome associated with both herpes infection and metastatic disease. RESULTS: Skin biopsy showed neutrophilic infiltrates in the dermis confirming the diagnosis of Sweet's syndrome. CONCLUSIONS: To our knowledge, this is the second case of Sweet's syndrome associated with herpes simplex infection in the literature. Further observations are required to determine the relationship between Sweet's syndrome and herpetic infection.     

Particular histological features of a case of Sweet's syndrome induced by G-CSF

BACKGROUND: Sweet's syndrome may occur during medullar aplasia, especially after treatment with exogenous growth factors such as G-CSF. In this context, Sweet's syndrome presents particular histological features that we detail in this observation. CASE REPORT: A 50 year-old man was treated for multiple myeloma with a mobilizating chemotherapy prior to autologous stem cell transplantation. Four days after the onset of G-CSF, he presented with a febrile generalized eruption of erythematous infiltrated lesions. Histological examination of a skin biopsy showed a neutrophilic infiltrate associated with atypical xanthomized histiocytes and vascular hyperplasia with marked endothelial turgescence. Treatment with a short cause of oral corticosteroids was efficient. DISCUSSION: The classical histological features of Sweet's syndrome consist in a dermal neutrophilic infiltrate with edema. In our patient, we noticed the presence of atypical histiocytes among the dermal neutrophilic infiltrate. These histiocytes are described in maculo-papular eruptions induced by G-CSF, and should not be confused with a malignant infiltrate associated with a hemopathy. Vascular hyperplasia may be related to the angiogenic properties of G-CSF. Knowledge of these histological features would enable clinicians and histologists to recognize the appropriate diagnosis.     

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito Santo)

Sweet's syndrome or acute febrile neutrophilic dermatosis is rare in Brazil. It is clinically characterized by painful erythematous nodules, papules or plaques that occur mainly on the neck and upper limbs. Its cause may be unknown (idiopathic form) or it may be associated with malignancies, usually hematologic, or drugs. The authors describe 16 cases of the syndrome. The median age was 36 years, and all patients were white and presented solid lesions, predominantly on the upper limbs and trunk. Histopathological examination of the dermis of all patients revealed predominance of moderate to intense, superficial and deep, diffuse inflammatory infiltrate, mainly consisting of polymorphonuclear neutrophils, with leukocytoclasia. It also revealed changes in the epidermis and hypodermis (neutrophilic hypodermitis), but with no signs of vasculitis in most patients, which was not considered an important finding for diagnosis. Presence of exocytosis of neutrophils was common, favoring the diagnosis of Sweet's syndrome when accompanied by diffuse interstitial neutrophilic dermatitis.     

Sweet's syndrome with neurologic manifestations in a patient with esophageal adenocarcinoma: case report and review of the literature

Background  Sweet's syndrome, also known as febrile neutrophilic dermatosis, can occur in patients with an underlying malignancy and can present with extracutaneous manifestations, including neurologic symptoms.
Methods  This report describes a 62-year-old man with adenocarcinoma of the esophagus who developed Sweet's syndrome and whose postoperative course was complicated by encephalitis.
Results  A diagnosis of Sweet's syndrome with neurologic manifestations was made, and the patient was treated with oral corticosteroids. His symptoms improved markedly within 12 h.
Conclusion  Neurologic symptoms in Sweet's syndrome are infrequently reported and have not been described previously in a patient with adenocarcinoma of the esophagus.     

A case of Sweet's syndrome with early gastric cancer

The case of a 49-year-old man with Sweet's syndrome (acute febrile neutrophilic dermatosis) is described, who had coincidently gastric cancer. The therapeutic effectiveness of colchicine is also presented as one treatment for Sweet's syndrome, because rapid regression of the lesions was noticed.     

A NEUTROPHILIC REACTION OF SWEET''S SYNDROME TYPE ASSOCIATED WITH THE ORAL CONTRACEPTIVE

A case is presented of a Sweet's syndrome-like eruption in association with the oral contraceptive. A 46 year old caucasian woman developed recurrent episodes of erythematous tender plaques on her trunk six weeks after commencement of the oral contraceptive (OC). Her condition clinically and histologically resembled Sweet's dermatosis. On cessation of the OC there was complete resolution of her lesions and she remains well 12 months later. This is the first report, to our knowledge, of a neutrophilic reaction to the oral contraceptive, and we believe that drugs may be implicated in the aetiology of atypical neutrophilic reactions simulating Sweet's syndrome in patients who are otherwise well.     

Sweet综合征临床研究进展

Sweet综合征又称急性发热性嗜中性粒细胞皮病,是一种少见的特发性疾病,近年来其临床研究有些新的进展.在分类上,发现一种局限性亚型,称为"手部嗜中性皮病",在临床表现上则发现更多不同于典型表现的皮疹,而组织学上皮损的炎性浸润方式及深度有所扩展,诊断及系统评估方法亦更加完善.     

Acute febrile neutrophilic dermatosis--a marker of malignancy?

A retrospective survey during a 2-year period disclosed 18 patients with acute febrile neutrophilic dermatosis (Sweet's syndrome). An associated lympho- or myeloproliferative malignancy was found in 6 patients. Attacks of Sweet's syndrome preceded the diagnosis of neoplasia in 4 patients (3 months to 6 years). Some differences in symptoms and signs were found in the group of patients with associated malignancy compared with the group without, that is, male predominance, mucosal symptoms, anemia, and frequent recurrence of skin symptoms. The onset of Sweet's syndrome indicates an acute infectious disease, and the patients are frequently referred to departments of internal medicine and infectious diseases. In addition, the skin lesions may mimic those which often accompany a generalized infection (erythema multiforme, erythema nodosum, vasculitis, pustular eruptions and urticaria). Since Sweet's syndrome may precede the possibly associated malignant disease, the initial diagnosis of the syndrome is important and should be made with confidence with increasing awareness of the characteristic symptoms.     

Sweet's syndrome in a child

INTRODUCTION: Sweet's syndrome was described for the first time in 1964. It is usually described in adults and remains rare in children. We report a case in a 23 month-old infant. OBSERVATION: A 23 month-old boy presented with diffuse papular, edematous annular plaques surrounded by vesicles and bullas. Laboratory examinations revealed neutrophilic polynuclear hyperleukocytosis, anemia and an inflammatory syndrome. The myelogram was rich and the abdominal sonography normal. Oral corticosteroids (2 mg/kg/d) led to spectacular improvement. After 19 days' treatment, the boy developed cervical adenopathies and hepatomegaly. The second myelogram was normal. Evolution under corticosteroids was good. DISCUSSION: Sweet's syndrome is exceptional in infants. The frequent association with a malignant blood disease should prompt appropriate investigations and prolonged surveillance. Systemic corticosteroid therapy is the reference.     

A continuum of neutrophilic disease occurring in a patient with ulcerative colitis

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders, Aiypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological) dyscrasias but not. to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum,; Sweet's syndrome, erythema elevatum diutinum and. subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.     

Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.     

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--like neutrophilic lobular panniculitis associated with all-trans-retinoic acid chemotherapy in a patient with acute promyelocytic leukemia

Sweet's syndrome-like (Sweet's-like) neutrophilic panniculitis is usually idiopathic, but is frequently associated with hematologic, inflammatory, and immunologic disease. Drug-related cases of Sweet's syndrome have been reported. Of relevance, chemotherapy with the retinoid all-trans-retinoic acid (ATRA) infrequently induces Sweet's-like neutrophilic panniculitis that occurs concomitantly with neutrophilic differentiation. The pathologic features are limited to the adipose tissue or include both the dermis and the subcutaneous fat; the neutrophilic infiltrate can be observed in the lobules, the septae, or both. We present this case because of the unusual subcutaneous neutrophilic infiltrate consistent with Sweet's-like neutrophilic lobular panniculitis in a patient with acute promyelocytic leukemia receiving ATRA chemotherapy. This case highlights the important connection between ATRA and Sweet's syndrome.     

A case of recurrent Sweet's syndrome in an 80-year-old man: a clue to an underlying malignancy

An 80-year-old man presented with multiple erythematous papules on the trunk and extremities of a few weeks' duration. He had no past medical or family history of skin diseases or any other medical diseases. A biopsy showed a perivascular lymphohistiocytic infiltrate and sparse neutrophils with several atypical lymphocytes in the deeper dermis. With an initial diagnosis of T-cell pseudolymphoma or unspecified neutrophilic dermatosis, he showed a brisk response to an intramuscular injection of triamcinolone acetonide (40 mg/mL). After 1 month, his skin lesion recurred. Steroid was given with a good clinical response. One month later, however, his skin lesion relapsed. At this time, he presented with disseminated pustulopapular lesions on the trunk and extremities. Examination revealed multiple, variable-sized, erythematous plaques with central pustules on the extremities (Fig. 1). The mucous membranes were not involved. He had no pain or tenderness. He had no systemic symptoms. Laboratory tests showed a hemoglobin level of 10.3 g/dL, a leukocyte level of 6,900/mm(3), with an increased proportion of segmented nuclear neutrophils (83%), and an elevated C-reactive protein. A skin biopsy revealed a dense perivascular and interstitial infiltrate composed of neutrophils with marked dermal edema (Fig. 2). Sweet's syndrome was the final diagnosis and he was treated with oral prednisolone (30-40 mg/day) and dapsone (50 mg/day) for 2 months. As this 80-year-old patient had a recurrent history of similar skin lesions and anemia, an underlying hematologic malignancy was suspected. A bone marrow biopsy showed typical myelodysplastic syndrome (MDS). The hemoglobin level was decreased to 5.3 g/dL during a follow-up period of 5 months. The skin lesions recurred despite oral steroids and dapsone. The patient received only symptomatic treatment, such as a transfusion, for the underlying malignancy MDS.     

Sweet's syndrome in subacute cutaneous lupus erythematosus

A 67-year-old man being treated with prednisone, 15 mg/day, for subacute cutaneous lupus erythematosus developed widespread, tender, erythematous plaques, arthralgias, and fever. Clinically, the cutaneous lesions mimicked lupus erythematosus. The diagnosis of acute febrile neutrophilic dermatosis, or Sweet's syndrome, was confirmed by histologic examination of three biopsy specimens, which revealed prominent dermal edema, extensive infiltration with intact and karyorrhectic polymorphonuclear leukocytes, and perivascular and appendiceal lymphocytes in the absence of leukocytoclastic angitis.