Bullous Sweet's syndrome in a patient with ulcerative colitis: a rare case report.

Bullous Sweet's syndrome is an uncommon clinical presentation of classical Sweet's syndrome, often associated with various kinds of tumors, infections, and active inflammatory bowel diseases (IBDs), namely Crohn's disease and ulcerative colitis. Only a few cases of bullous Sweet's syndrome associated with ulcerative colitis are described in the literature. We report a case of a 62-year-old female patient with acute exacerbation of ulcerative colitis associated with infiltrating purple-erythematous skin plaques, which were partly vesicular, and oral ulcerative stomatitis. Biopsy was consistent with bullous Sweet's syndrome. Treatment with betamethasone sodium phosphate, starting at 5.5 mg, followed by gradual dose tapering for 12 weeks, resulted in improvement of the ulcerative colitis and disappearance of the cutaneous lesions. Bullous Sweet's syndrome most commonly occurs in the setting of hematologic malignancies, suggesting that physicians should perform long-term screening for early diagnosis of hematological and solid malignancies.

A 68-Year-Old Man With Skin Rash and a Pleural Effusion.

CASE PRESENTATION: A 68-year-old man developed an erythematous, papular, pruritic rash on his right thigh 1 month prior to presentation. It subsequently spread to his other extremities and trunk. He also endorsed fevers of > 38.3°C, night sweats, fatigue, shortness of breath, and a dry cough. He was prescribed triamcinolone 0.1% cream for his rash and azithromycin for presumed community-acquired pneumonia, with no improvement in symptoms. He had a history of relapsing polychondritis for which he was prescribed infliximab and low-dose prednisone. He had never smoked tobacco, did not use alcohol or illicit substances, and had no significant travel history.

Insights Into the Pathogenesis of Sweet's Syndrome.

Sweet's syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare inflammatory condition. It is considered to be the prototype disease of neutrophilic dermatoses, and presents with acute onset dermal neutrophilic lesions, leukocytosis, and pyrexia. Several variants have been described both clinically and histopathologically. Classifications include classic Sweet's syndrome, malignancy associated, and drug induced. The cellular and molecular mechanisms involved in Sweet's syndrome have been difficult to elucidate due to the large variety of conditions leading to a common clinical presentation. The exact pathogenesis of Sweet's syndrome is unclear; however, new discoveries have shed light on the role of inflammatory signaling, disease induction, and relationship with malignancy. These findings include an improved understanding of inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic contributions. Continued investigations into effective treatments and targeted therapy will benefit patients and improve our molecular understanding of inflammatory diseases, including Sweet's syndrome.

[The etiology, diagnosis, and treatment of neurological complications in Behçet disease and its related disorder Sweet disease].

Behçet disease, and its related disorder Sweet disease, are multisystem inflammatory conditions characterized by muco-cutaneous symptoms. When neuropsychiatric symptoms appear, the two conditions are referred to as neuro-Behçet disease and neuro-Sweet disease. While diagnosing these conditions according to their diagnostic criteria, muco-cutaneous symptoms must be observed; however, neuropsychiatric symptoms may precede muco-cutaneous symptoms. In these conditions the dysregulation of cytokines, following the onset of oral muco-cutaneous bacterial infection, may induce an abnormal chemotaxis of neutrophils causing ectopic encephalitis and meningitis. Thus, an initial treatment targeting neutrophils should be considered based on the diagnosis of neuro-neutrophilic disease when symptoms indicating neutrophil hyperactivity are observed, even without muco-cutaneous symptoms. In addition to human leukocyte antigen-B51 and -A26, genome-wide association analyses have identified new susceptibility loci on the genes of various immunological factors in Behçet disease. These findings may help elucidate disease pathogenesis and assist the development of diagnostic modalities and therapeutic agents for neuro-neutrophilic disease.

Necrotizing Sweet Syndrome of the Upper Extremity After Elective Hand Surgery.

Sweet syndrome, or acute febrile neutrophilic dermatosis, is a systemic disease process mainly characterized by hyperpyrexia and skin lesions. A newly described entity, necrotizing Sweet syndrome, is a severe and locally aggressive dermatological condition that clinically and histopathologically resembles a necrotizing soft tissue infection. It is characterized by pathergy, a nonspecific inflammatory response to cutaneous trauma resulting in a propagation of the disease. In contrast to a necrotizing infection, this condition responds to systemic steroids. A high clinical suspicion is required in order to distinguish a necrotizing polymicrobial infection from noninfectious necrotizing Sweet syndrome. We present a case following elective hand surgery.

Disease-related and drug-induced skin manifestations in inflammatory bowel disease.

INTRODUCTION: Skin manifestations are common in patients with inflammatory bowel diseases (IBD) and can be part of a concomitant illness with a shared genetic background, an extra-intestinal manifestation of the disease, or a drug side-effect. Areas covered: We provide a practical overview of the epidemiology, pathogenesis, diagnosis, therapeutic approach and prognosis of the most frequent disease-related and drug-induced cutaneous manifestations in IBD, illustrated by cases encountered in our clinical practice. Among the most frequently encountered IBD-related lesions are erythema nodosum, pyoderma gangrenosum and Sweet's syndrome. Common skin manifestations with a strong association to TNF antagonists are local injection site reactions, psoriasiform lesions, cutaneous infections, vasculitides and lupus-like syndromes. In addition, we discuss the relation of thiopurines and TNF antagonists with the risk of skin cancer. Expert commentary: We hope this review will help caretakers involved in the management of IBD patients to recognize the lesions and to manage them in close collaboration with a dedicated dermatologist.

Síndrome de Sweet: revisión de la literatura a propósito de un caso / Sweet syndrome: review of the literature about a case

Sweet (SS) syndrome is a neutrophilic dermatosis, characterized by a rapid onset of painful erythematous-purplish papules, nodules or plaques, accompanied by fever and associated with a dermal neutrophil infiltrate. CASE REPORT: Male, 69 years old, with a history of mild plaque psoriasis, arterial hypertension, gastroesophageal reflux and chronic sinusitis. He consulted for a 24-hour period characterized by headache and pain in the scalp. The examination highlights fever (38.5°), with slight erythema, sensitivity to palpation and some psoriasis plaques on the scalp. 48 hours later, it presents extensive painful erythematouspurplish plaques, present only on his right face and scalp. Biopsy was informed as Sweet Syndrome. He completed 14 days on prednisone, with excellent clinical evolution. DISCUSSION Sweet syndrome is classified into three categories: classic or idiopathic (most common), drug induced and associated with malignancy. In addition, other conditions have been described which may be related to: infections, autoimmune diseases, pregnancy. The management is focused on investigating an underlying cause and the use of corticosteroids, as a first line therapy. We report a case of idiopathic Sweet syndrome with atypical presentation, which was initially interpreted as facial cellulitis, with a rapid response to systemic corticosteroids. (AU)

Sweet syndrome: long-term follow-up of 138 patients.

BACKGROUND: Several studies support a strong association of Sweet syndrome (SS) with malignancy. However, only a few studies analysing the clinical features of malignancy-associated SS have been published in recent years. AIM: To retrospectively study the clinical features of SS that could predict the development of associated malignancies and to analyse the development of malignant neoplasia during long-term follow-up of patients with SS. METHODS: Clinical features of the patients diagnosed with SS syndrome between 1987 and 2013 at Bellvitge Hospital (Barcelona, Spain) were retrospectively analysed. RESULTS: In total, 138 patients were included in the study (66 male, 72 female, mean ± SD age 51.24 ± 14.11 years). SS was associated with haematological malignancy in 31 cases, infection in 23, inflammatory bowel disease in 12, inflammatory systemic disease in 8, and solid tumours in 4. It was drug-induced in 5 cases and idiopathic in 54. In four patients, an underlying haematological disease that was considered related to SS was diagnosed between 4 and 16 months after SS presentation. Variables significantly associated with malignancy in multivariate logistic regression analysis were age (OR = 1.08 for each increasing year, P = 0.01), anaemia (OR = 9.38, P = 0.001), thrombocytopenia (OR = 16.10, P < 0.01) and absence of arthralgia (OR = 11.13, P < 0.01). CONCLUSIONS: Patients with older age, anaemia or thrombocytopenia, and without arthralgia are more likely to have malignancy-associated SS. We recommend that patients with SS without clear aetiology should be followed up for at least 16 months to exclude a possible underlying haematological malignancy.

Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A French Multicenter Study of 17 Cases and Literature Review.

A few reports suggest combination of ANCA-associated vasculitis (AAV) and neutrophilic dermatoses (ND). We aimed to describe the main characteristics of patients presenting with both AAV and ND in a French cohort and through a systematic literature review, and to discuss the possible common pathogenic process involved. We conducted a retrospective study of patients with both conditions. Patients were selected via the French Internal Medicine Society (SNFMI) and the French Vasculitis Study Group (FVSG). A literature review focusing on a combination of both conditions, concentrated only on publications with well-established diagnoses and individual detailed data. Seventeen patients diagnosed with AAV and ND were identified in this cohort. Twelve patients had granulomatosis with polyangiitis (GPA), 4 had microscopic polyangiitis (MPA) and one had eosinophilic GPA (EGPA). Eight patients, all with GPA, displayed pyoderma gangrenosum (PG). Sweet's syndrome was observed in 6 patients (4 with MPA, one with GPA and one with EGPA) and erythema elevatum diutinum in the other three (2 with GPA and 1 with MPA). The literature review identified 33 additional patients with both conditions, including 26 with GPA. Altogether, of the 50 patients (17 from our study and 33 from the literature review), 33 (66%) patients presented with PG associated with GPA in 29 cases (89%). Corticosteroids were the first-line treatment in conjunction with an immunosuppressive agent in most cases. Outcomes were good and a total of 15 patients experienced a relapse. Patients who relapsed were more likely to have ear, nose and throat manifestation than patients who did not [12/15 (80%) relapsing patients vs. 15/35 (43%) non-relapsing patients; p = 0.03)]. In our stud, the most frequent association concerned GPA and PG. ND should be considered and specifically researched within the spectrum of cutaneous manifestations observed in AAV.

Sweet Syndrome: A Review and Update.

Sweet syndrome is the most representative entity of febrile neutrophilic dermatoses. It typically presents in patients with pirexya, neutrophilia, painful tender erytomatous papules, nodules and plaques often distributed asymmetrically. Frequent sites include the face, neck and upper extremities. Affected sites show a characteristical neutrophilic infiltrate in the upper dermis. Its etiology remains elucidated, but it seems that can be mediated by a hypersensitivity reaction in which cytokines, followed by infiltration of neutrophils, may be involved. Systemic corticosteroids are the first-line of treatment in most cases. We present a concise review of the pathogenesis, classification, diagnosis and treatment update of this entity.

Síndrome de Sweet idiopático: reporte de caso / Sweet syndrome idiopathic: case report

El síndrome de Sweet (SS) es el prototipo de las dermatosis neutrofílicas, un grupo de enfermedades cutáneas de carácter reactivo caracterizadas histopatológicamente por la presencia de un infiltrado de neutrófilos. Es un marcador de diversas enfermedades internas, entre las que destacan las infecciones, la enfermedad inflamatoria intestinal, las conectivopatías autoinmunitarias y variadas neoplasias malignas, especialmente las de origen hematológico. Se presenta el caso de una mujer de 39 años, con diagnóstico de SS, tras sospecha clínica y biopsia de lesiones cutáneas.

Sweet's syndrome (SS) is the prototype of neutrophilic dermatosis, a group of skin diseases reactive in nature and characterized histopathologically by the presence of an infiltrate of neutrophils. It is a marker of various internal diseases, among which are infections, inflammatory bowel disease, autoimmune connectivopathies and various malignancies, especially those oj hematologic origin. We present a case of a 39 year old woman, diagnosed with SS after clinical suspicion and biopsy of skin lesions.

Síndrome de Sweet. Descripción de un caso idiopático / Sweet syndrome. Idiopathic case report

El síndrome de Sweet (SS) es el prototipo de las dermatosis neutrofílicas, un grupo de enfermedades cutáneas de carácter reactivo caracterizadas histopatológicamente por la presencia de un infiltrado de neutrófilos. Es un marcador de diversas enfermedades internas, entre las que destacan las infecciones, la enfermedad inflamatoria intestinal, las conectivopatías autoinmunitarias y variadas neoplasias malignas, especialmente las de origen hematológico. Se presenta el caso de una mujer de 39 años, con diagnóstico de SS, tras sospecha clínica y biopsia de lesiones cutáneas.

Sweet's syndrome (SS) is the prototype of neutrophilic dermatosis, a group of skin diseases reactive in nature and characterized histopathologically by the presence of an infiltrate of neutrophils. It is a marker of various internal diseases, among which are infections, inflammatory bowel disease, autoimmune connectivopathies and various malignancies, especially those of hematologic origin. We present a case of a 39 year old woman, diagnosed with SS after clinical suspicion and biopsy of skin lesions.

Insight into Sweet's syndrome and associated-malignancy: a review of the current literature.

Sweet's syndrome (acute febrile neutrophilic dermatosis) is an infrequent skin disease characterized by sudden onset of fever, leucocytosis and erythematous plaques or nodules infiltrated by neutrophils. There are three main clinical settings in which Sweet's syndrome has been described: classical or idiopathic Sweet's syndrome, malignancy-associated Sweet's syndrome and drug-induced Sweet's syndrome. Classical Sweet's is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. Approximately 21% of patients have an associated malignancy, most commonly hematological disease. The syndrome may occur as a paraneoplastic accompaniment to established cancer or may be a first sign of malignancy or its recurrence. The incidence is said to be increasing in recent years due to the frequent use of growth factors in cancer patients. Several anticancer agents including all-trans-retinoic acid proteosome inhibitors, hypomethylating agents, tyrosine kinase inhibitors and lenalidomide are potential harbingers of Sweet's syndrome. Unfortunately, little is known about the pathophysiology of Sweet's syndrome and there are no established guidelines for treatment of malignancy-associated Sweet's syndrome. Systemic corticosteroids are the mainstay of treatment. Sweet's syndrome caused by anticancer agents sometimes involves withdrawal or temporary discontinuation of anticancer agents, use of systemic corticosteroids and/or rechallenge with either with the same anticancer agents or different agents. This report provides insights into the pathophysiology, clinical presentation, diagnostic work, differential diagnosis and management of malignancy-associated Sweet's syndrome published in reported cases.

Sweet's syndrome: a revisit for hematologists and oncologists.

The diagnosis of Sweet's syndrome rests on a combination of clinical symptoms and characteristic physical and pathologic features. Patients typically have fever and tender erythematous skin lesions (papules, nodules, or plaques). Neutrophilia, high levels of serum inflammatory markers, and diffuse mature neutrophil infiltration localized to the upper dermis are the most important findings. Sweet's syndrome was first described by Robert Sweet in 1964, whose sentinel paper described 8 women with fever, leukocytosis, and erythematous plaques infiltrated by neutrophils. Subsequently, extracutaneous sites were included in the diagnosis. This review of publications between 1964 and April 2012 found 1683 reports of Sweet's syndrome of which only 8 were published between 1964 and 1969, after which the number of the papers grew by decades to 59, 228, 459 and 692. With more articles, there are more reports of malignancy-associated Sweet's syndrome. This may reflect the awareness by physicians of the disease and of the drugs which may cause it. There is considerable overlap in the constitutional findings of Sweet's syndrome and malignant disorders. It is crucial that the possibility of Sweet's syndrome be included in a hematologist or oncologist's differential diagnosis of fever and skin lesions.