Classical Sweet Syndrome Research Articles

Clinical Dilemma—Ecthyma Gangrenosum Versus Sweet Syndrome

Abstract Sweet syndrome is associated with malignancy, drugs, and certain infections; its pathogenesis is not well understood. We report a case of a 30-year-old woman with stage IV breast cancer, who presented with worsening respiratory failure and erythematous skin lesions. Biopsy of the skin lesion was consistent with the Sweet syndrome; however, the culture grew Pseudomonas aeruginosa. The lesions healed without scarring when treated with antibiotics and steroids. This is the first case report of classic Sweet syndrome in association with P. aeruginosa.

Classic Sweet Syndrome with Mucosal Involvement

Sweet syndrome is an uncommon disorder of unknown etiology and characterized by skin and systemic inflammation. Classic Sweet syndrome is not associated with malignancy or drug exposure and typically involves elderly females. A 67-year-old female with a past medical history of multiple sclerosis and chronic kidney disease presented with worsening erythema of bilateral lower extremities, and diffuse, tender pustular lesions in her extremities, eye lids, nares, oral commissures, and tongue. A biopsy revealed marked neutrophilic inflammation suggestive of Sweet syndrome and she was started on high dose methylprednisolone which immediately improved her condition. As with most progressive inflammatory diseases, early recognition and early treatment improves the prognosis. This case is unusual because of mucosal involvement at presentation. Classic Sweet syndrome can often be diagnosed by exclusion after failed treatment for either infectious or autoimmune disorders.

Sweet syndrome: A retrospective study of 64 cases and proposal of an algorithmic approach to improve investigation and management.

BackgroundSweet syndrome (SS) can be categorized as classical Sweet syndrome (CSS), malignancy‐associated Sweet syndrome (MASS) or drug‐induced Sweet syndrome (DISS). Appropriate categorization of patients with SS and identification of the associated trigger are essential to direct subsequent investigations and follow‐up, especially given that 21% of cases are malignancy‐associated. However, no published guidelines exist to guide this.ObjectiveTo analyse the categorization, management and outcomes of patients with SS in order to propose a structured approach for investigation and follow‐up.MethodsRetrospective data collection from the electronic records of patients diagnosed with SS between 1 January 2005 and 31 December 2018. Categorized and non‐categorized patients were compared, and the yield rate of investigations and duration of follow‐up were analysed.ResultsSixty‐four patients were included with CSS (77%), MASS (20%) and DISS (3%). Of these, 34 (53%) cases were not categorized by the assessing clinicians, three of which were subsequently diagnosed with a malignancy, up to 19 months later. There was no significant difference in investigations performed between categorized and non‐categorized patients and the yield rates were modest overall. Follow‐up averaged 10.5 (16.8) months; non‐categorized patients were followed‐up for significantly longer than categorized patients (15.0 (21.2) vs. 5.4 (6.8) months, p < 0.05).ConclusionThe lack of a structured way to approach patients with SS can lead to under‐ or over‐investigation, diagnostic delays of underlying conditions and unnecessary follow‐up. An algorithm is proposed to identify the likely trigger and manage patients accordingly. Larger prospective studies are required to confirm the optimal approach to investigate and follow‐up patients with SS.

Dapsone as corticosteroid-sparing therapy for Sweet syndrome

To the Editor: Sweet syndrome is a rare cutaneous condition that typically presents as a painful, pruritic, erythematous papular eruption with histologic evidence of dense neutrophilic infiltrates in the upper dermis.1 Classical Sweet syndrome is often associated with a preceding respiratory infection, although the condition can also be induced by hematologic malignancies, medications, and autoimmune diseases.2 While Sweet syndrome can spontaneously resolve, many patients undergo medical management.

EP11 An unusual presentation of Sweet syndrome

Abstract Background Sweet syndrome (acute febrile neutrophilic dermatosis) is a rare inflammatory disorder affecting individuals between the ages of 30 and 60. It is characterised by painful, edematous, and erythematous skin papules, plaques, or nodules. Fever and leukocytosis (neutrophilia) frequently accompany the skin lesions. A majority of cases are idiopathic but there is a significant proportion attributed to malignancy or medications. Our case is peculiar because of an unusual presentation of a 12-month history of undifferentiated inflammatory arthritis before manifestation as Sweet syndrome. The mainstay of treatment is corticosteroids, although spontaneous resolution has been reported. Treatment of an underlying medical cause may lead to an improvement in Sweet syndrome. Methods A 57-year old British male presented with a 1-year history of intermittent joint pain and swellings in both upper and lower limbs. He had been treated with short courses of oral corticosteroids for undifferentiated inflammatory arthritis. As part of his extensive investigations whilst as a private outpatient, a knee MRI raised suspicion of malignancy, leading him to be referred to haematology and having a bone marrow examination. He was admitted with fever, polyarthritis and skin rash in form of papules around elbows and pustules on tip of some fingers. He was started on empirical antibiotics and reviewed by the Dermatology and Rheumatology teams. A skin biopsy was performed. He continued to have temperature spikes throughout admission and inflammatory markers remained elevated despite being on antibiotics. Results Full blood count showed leucopenia and Iron deficiency anaemia MCV 78 Iron 6 Transferin saturation 15% .Electrolytes, renal and liver function were normal. CRP was elevated at 190 mg/L. Acute infection screen including blood cultures and viral screen were negative. Immunology tests including Anticcp, ANA, ENA, and ANCA were all unremarkable. Echocardiogram was normal. The bone marrow biopsy showed hypercellular marrow with reactive appearances. Full body CT showed no focal signs of infection, lymphadenopathy or malignancy, with the positive findings being only a mildly enlarged spleen. Skin biopsy was consistent with a neutrophilic dermatosis such as Sweet syndrome. Conclusion This case highlights the atypical presentation of classical Sweet syndrome with no obvious underlying cause and no rise in neutrophil count. It also raised our awareness that splenomegaly can be part of Sweet syndrome and does not necessarily mean an underlying haematological disorder. The patient showed remarkable improvement with resolution of synovitis, fever and the rash once restarted on corticosteroids. He has been discharged from haematology as his blood count and CRP have normalised. Although arthralgia and arthritis have been reported in Sweet syndrome, we believe that this the first case in the literature where Sweet syndrome has manifested as undifferentiated inflammatory arthritis. Disclosures A.A.M. Mohamednour None. D. Teo None. V. Rastu None. K. Sunmboye None.

Afebrile and Leukopenic Sweet's Syndrome Following Partial Ileectomy in an Elderly Woman

Afebrile and Leukopenic Sweet's Syndrome Following Partial Ileectomy in an Elderly Woman

抗γ干扰素自身抗体阳性伴Sweet综合征的马尔尼菲篮状菌病九例临床分析

Objective To report 9 HIV-negative patients with talaromycosis marneffei (TSM) complicated by Sweet syndrome, and to analyze the relationship of the anti-interferon-γ (anti-IFN-γ) autoantibody with TSM complicated by Sweet syndrome. Methods HIV-negative patients with TSM complicated by Sweet syndrome were collected from the First Affiliated Hospital of Guangxi Medical University between 2013 and 2018. Their clinical and laboratory data were analyzed retrospectively. Meanwhile, 19 HIV-positive patients with TSM and 107 health checkup examinees served as controls. Anti-IFN-γ autoantibody was detected in peripheral blood samples of the patients and controls. Results A total of 9 HIV-negative patients with TSM (5 males and 4 females) were included in this study, and the age of onset ranged from 38 to 60 years. The 9 patients all presented with disseminated infections, manifesting as long-term irregular fever, multiple lymph node enlargement, cough, emaciation and anemia. All of the 9 patients met the diagnostic criteria for classical Sweet syndrome, and microbiological examination of Sweet syndrome lesions was negative. Besides Talaromyces marneffei, 6 patients also were infected with nontuberculous mycobacteria, 4 with varicella-zoster virus, and 2 with Salmonella. All the 9 HIV-negative patients with TSM were positive for anti-IFN-γ autoantibody, while the 107 healthy controls and 19 HIV-positive patients with TSM were negative for anti-IFN-γ autoantibody. Conclusion Anti-IFN-γ autoantibody may be associated with HIV-negative TSM complicated by Sweet syndrome. Key words: Sweet syndrome; Interferon-gamma; HIV; Mycoses; Talaromycosis marneffei

Recurrent Idiopathic Sweet Syndrome - Case Report and Literature Review.

Introduction. Sweet syndrome (SS), also denominated as acute febrile neutrophilic dermatosis, is a rare disease characterized by the sudden onset of painful, erythematous, firm skin lesions (papules, plaques, and nodules) which show, upon histologic examination, the presence of a diffuse infiltrate of mature neutrophils. The cutaneous manifestation typically involves the face, neck, trunk, and upper limbs and is associated with fever, general malaise, arthralgia. Case report. A female patient, 60 years old, attended the Dermatology Clinic due to the appearance of violaceous erythematous-oedematous infiltrated plaques, located on the face, neck, upper limbs, trunk and knees. The onset of the cutaneous manifestation had occurred 2 months prior, accompanied by pain, chills, flares of fever and arthralgia. The onset coincided with the surgical treatment of an umbilical hernia. From the medical history we note that the patient was diagnosed in 2014 with histiocytoid SS. She followed a treatment with methylprednisolone, with positive response, but had many relapses after the discontinuation of treatment. In 2017, due to a new episode, the histopathological examination was repeated, which revealed classical SS. She received treatment with Disulone and Colchicine. She had not been administered any treatment throughout the previous year. Laboratory tests revealed leukocytosis with neutrophils, increased ESR, elevated C4, hyperglycemia. The current histopathological examination revealed lymphocytic SS. Under treatment with methylprednisolone 32mg/day, the evolution was favorable. Discussions. The first case of SS was described by Robert Douglas Sweet in 1964. As known aetiological factors there have been described gastrointestinal and urinary tract infections, pregnancy, inflammatory bowel disease, drugs or malignancies. There have been described cases of SS that appeared after surgical treatment, as in our case, which registered a new outbreak following the umbilical hernia treatment. The histopathological variants of SS described in the literature are: subcutaneous, eosinophilic, histiocytoid, lymphocytic type. The first line-therapy consists in systemic corticosteroids, which induce a fast remission of lesions and general symptoms. Recurrence may occur in approximately 50% of patients and is common in idiopathic or paraneoplastic cases. Conclusions. In addition to the neutrophilic infiltrate that is typical for Sweet syndrome, different types of histological manifestations have been described in the literature: subcutaneous, eosinophilic, histiocytoid, lymphocytic. In our case, we noted that the histological profile changed over time, from a histiocytoid SS recorded in 2014, to a classical SS in 2017, followed by the appearance of lymphocytic SS in 2019. Due to the fact that SS can be associated with a numerous other disorders, our patient requires regular monitoring with a view to eliminate them, and potentially to make a diagnosis and initiate early specific treatment.

3157 Sweet's Syndrome: A Rare Cutaneous Manifestation of IBD

INTRODUCTION: Sweet's syndrome (SS) is one of the rare cutaneous presentations associated with Crohn's disease. SS is characterized by a sudden onset of painful, edematous, and erythematous papules, plaques or nodules on the skin along with fever and neutrophilia. Here we present a case of SS in a patient with Crohn's disease. CASE DESCRIPTION/METHODS: A 29-year-old gentleman with a past medical history of Crohn's disease that was well controlled on mesalamine and infliximab infusions presented with a one week history of numerous erythematous papules on the chest and back and a few pustules around hair follicles on the upper torso. At the time of presentation, he was febrile and had elevated WBCs with a neutrophilic predominance. ESR was 63 and CRP was 11.2. Based on the presentation, he was diagnosed with Varicella infection and treated with Valacyclovir. However, he continued to be febrile with worsening new eruptions. Labs including VZV &amp; HSV PCR, viral cultures, fungal cultures, hepatitis panel, HIV and RPR were negative so the patient underwent a skin biopsy to investigate the etiology of the rash. Biopsy showed diffuse infiltration of the epidermis and dermis by a neutrophil-rich inflammatory cell infiltrate without any signs of leukocytoclastic vasculitis. This was consistent with the histopathological findings seen in Sweet's Syndrome. His antivirals were discontinued and he was started on a high dose prednisone taper with a resultant dramatic improvement in signs and symptoms. Over the next 7 days his rash resolved with normalization of white count and inflammatory markers. DISCUSSION: SS can be clinically classified into malignancy associated SS, drug induced SS or classic (idiopathic) SS most commonly associated with infections, inflammatory diseases or pregnancy. Classic SS usually appears in the active phase of inflammatory bowel disease. Our patient had uncontrolled CD for two years, but he had not had any extraintestinal manifestations during this time. Moreover, Infliximab has been used to treat steroid non-responsive SS. Our patient developed the syndrome even though he was on infliximab. Sweet's Syndrome should be one of the differentials when a patient with IBD presents with painful pustular rash and fever.

2068 Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome)

INTRODUCTION: Sweet syndrome is associated with various GI conditions such as acute gastroenteritis, inflammatory bowel disease and colon cancer. We describe a unique case of a young patient with Crohn’s disease who developed Sweet syndrome while being on infliximab therapy. CASE DESCRIPTION/METHODS: A 29 yo M with history of sacroiliitis and fistulizing ileocolonic Crohn's disease on maintenance oral mesalamine and IV infliximab presented with sudden onset of painful papulonodular rash along with high grade fever. The rash involved his face, trunk and upper extremities. He had significant leukocytosis with predominant neutrophilia, elevated ESR and CRP. Infliximab antibody was negative. Skin biopsy showed a dense neutrophilic infiltrate without evidence of vasculitis. Work up for an occult malignancy including a peripheral blood smear, recent colonoscopy and CT scan of the body was unremarkable. Based on the overall presentation he was diagnosed with Sweet syndrome and started on oral prednisone 1 mg/kg body weight daily. Patient showed a rapid response to steroid treatment and the rash completely resolved within a couple of weeks. Differential for his presentation included infliximab induced pustular psoriasis but the biopsy findings were not typical for the condition and there was no recurrence of the rash on continuation of IV infliximab. DISCUSSION: Sweet syndrome is characterized sudden onset of papulonodular skin rash and fever. It is of three types; classical, malignancy associated and drug induced. All types can be encountered in the gastroenterology practice and it is therefore important to have good understanding of the condition. Classical sweet syndrome can be seen in patients with inflammatory bowel disease and acute gastroenteritis. Among solid tumor cancers, sweet syndrome can be seen with colon cancer where as various medications prescribed in the GI practice including azathioprine, norfloxacin and furosemide can cause it too. In the appropriate setting a skin biopsy showing dense neutrophilic infiltrate without leukocytoclastic vasculitis can be pathognomonic. Patients usually have an excellent response to systemic steroids. In recalcitrant cases, other immunosuppressive medications such as methotrexate, adalimumab and infliximab have been used successfully. Our case presentation is unique since patient developed Sweet syndrome while being on IV infliximab therapy. In patients with recurrent Sweet syndrome malignancy should strongly be considered.

DIFFUSE ALVEOLAR HEMORRHAGE AS A RARE COMPLICATION OF SWEET SYNDROME

SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Sweet’s syndrome (SS) is a rare inflammatory condition, characterized by fever, neutrophilia and skin lesions in the form of papules, nodules, plaques and rarely bullae. It typically occurs in middle age female, with around 70% of cases being idiopathic. While the disease may involve many organ systems, involvement of pulmonary system is rare (1,2). Systemic corticosteroids is the treatment of choice with favorable clinical outcomes. We present a case of sweet syndrome with pulmonary involvement presenting as diffuse alveolar hemorrhage. CASE PRESENTATION: Our patient is a 62 year old female who presented with Acute Hypoxemic respiratory failure secondary to Diffuse Alveolar Hemorrhage (DAH). An extensive Rheumatologic workup failed to indicate a possible etiology, until the skin biopsy returned positive for an intense neutrophilic infiltration of the epidermal layers of skin, consistent with Sweet Syndrome. A Lung biopsy was also negative for any vasculitis changes, while being suggestive of a diagnosis of Sweet Syndrome. She responded well to intravenous and oral corticosteroids, with full resolution of her respiratory and dermatological lesions. She was maintained on oral Prednisone in the outpatient setting with good clinical response. The patient then returned to our emergency department for new skin lesions. Initial physical exam was significant for purpuric papules on the dorsum of the left hand with ecchymosis and scattered bullae. The rest of the physical exam, laboratory investigations and imaging were within normal limits. The patient then developed hemoptysis. A chest x-ray revealed the development of left sided diffuse patchy opacities. The Patient was eventually intubated for acute hypoxemic respiratory failure. A Bronchoscopy revealed active bleeding from all left segmental bronchi. A skin biopsy showed pathological findings consistent with sweet syndrome (see figure), while being negative for vasculitis. Despite Pulse dose Methylprednisolone 1gram daily, and maximal supportive care , the progressed to develop refractory shock and Multi organ Failure, succumbing to her illness within the next 36 hours DISCUSSION: SS also known as acute febrile neutrophilic dermatosis is a rare inflammatory condition. It typically presents in three clinical settings: idiopathic or classic, malignancy associated and drug-induced sweet syndrome. Classic sweet syndrome commonly affects women between ages of 30 to 60, with no racial predilection. It is usually a multi system disease, with pulmonary involvement being rare, with symptoms ranging from flu like symptoms to acute respiratory distress syndrome. Most of patient responds well to prednisone. CONCLUSIONS: Our case highlights the importance of life threatening early recognition of the pulmonary manifestations in SS, and we emphasize the need for early and aggressive corticosteroid therapy to prevent severe respiratory compromise. Reference #1: Cohen PR. Sweet's syndrome--a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007;2:34. Published 2007 Jul 26. https://doi.org/10.1186/1750-1172-2-34 Reference #2: Villarreal-Villarreal CD1, Ocampo-Candiani J1, Villarreal-Martínez A2 Sweet Syndrome: A Review and Update. Actas Dermosifiliogr. 2016 Jun;107(5):369-78. https://doi.org/10.1016/j.ad.2015.12.001. Epub 2016 Jan 2 Reference #3: Vettakkara KMN, Banerjee S, Mittal A, et al. Not so sweet; severe Sweet's syndrome presenting as SIRS and pleural effusion. J Family Med Prim Care. 2018;7(6):1584-1587 DISCLOSURES: No relevant relationships by nargis jilani, source=Web Response No relevant relationships by Ravi Manglani, source=Web Response

Atypical sweet syndrome in the hands of small cell lung cancer patient associated with granulocyte colony-stimulating factor administration

Abstract Introduction Sweet syndrome (SS) characterized by papules, plaques or nodules. SS was divided into three subcategories as classical SS, malignancy associated SS, and drug-induced SS. Case presentation We present a case of G-CSF-associated Sweet Syndrome (SS) in a 50-year-old man with the diagnosis of extensive stage small cell lung cancer. We started preemptive methylprednisolone with a diagnosis of the sweet syndrome. Conclusion SS is the insidious complication of G-CSF . Clinical suspicion is the key to the early diagnosis.

A case of classical sweet syndrome with episcleritis

Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by a constellation of clinical symptoms, physical features, and pathologic findings which include fever, neutrophilia, tender erythematous skin lesions (papules, nodules, and plaques), and a diffuse infiltrate consisting predominantly of mature neutrophils that is typically located in the upper dermis. Sweet's syndrome presents in three clinical settings: classical (or idiopathic), malignancy-associated, and drug-induced. Classical Sweet's syndrome (CSS) usually presents in women between the age of 30 to 50 years, is often preceded by an upper respiratory tract infection and may be associated with inflammatory bowel disease and pregnancy. We report here a case of classical sweets syndrome with the typical histopathological findings who reported well to treatment.

Sweet Syndrome: Clinical Features, Histopathology, and Associations of 83 Cases.

Sweet syndrome (SS) is an infrequent skin disease characterised by sudden onset of fever, leukocytosis, neutrophilia, and tender erythematous plaques infiltrated by neutrophils. Multiple conditions have been associated with this syndrome. The aim of this study was to evaluate the clinical, epidemiological, laboratory, and histopathological findings and associations of patients with SS. We conducted a retrospective study of 83 patients with SS followed between January 1, 2006, and January 31, 2015. Of the patients, 82% were female; the mean age at onset was 48 years. Clinical presentation was mainly characterised by erythematous and edematous plaques, mostly on upper extremities and trunk. Fever was observed in 32%; 60% presented leukocytosis and 39% neutrophilia. On histopathological examination, neutrophilic and lymphohistiocytic infiltrate and edema were the most frequent findings. Fourteen percent of patients had malignancy or hematologic disorders, 26% were classified as having drug-induced SS, and 24% noted recent infection. Only 2 cases occurred during pregnancy. Systemic corticosteroid was the most common choice of treatment, with excellent response. In malignancy-associated SS, the mean hemoglobin level was lower ( P = .01) and the erythrocyte sedimentation rate (ESR) was higher ( P = .04) in comparison to classic and drug-induced SS. Leukocytoclasia was associated with higher risk of recurrence ( P = .01). All patients with SS deserve careful investigation of possible underlying conditions. Higher ESR and lower hemoglobin levels might reinforce the need of malignancy screening. Also, leukocytoclasia appears to be a potential marker of higher recurrence rate, demanding closer and longer follow-up.

Azathioprine Induced Sweetʼs Syndrome in Crohnʼs Disease: An Important Distinction: 2016 ACG Presidential Poster Award

Sweet's syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory skin disease that has been described in three distinct clinical scenarios. Classical SS either follows infection or occurs during pregnancy. SS also occurs with inflammatory bowel disease (IBD). Lastly, SS can be an atypical drug reaction. We describe a case of Azathioprine induced Sweet's Syndrome (AISS) in a young male with Crohn's disease. A 35-year-old man with ileo-colonic Crohn's disease presented to the emergency department with rash and fever two weeks after starting azathioprine. Within 48 hours of starting this medication the patient noted a left hand purulent lesion that was drained and treated with cephalexin, without resolution. On admission, physical examination revealed multiple papules and nodules that were not painful. The differential diagnosis included pyoderma gangrenosum, drug reaction, and Crohn's related SS. Skin biopsy revealed a predominantly neutrophilic septal and lobular panniculitis. One week after withdrawal of the medication, the patient defervesced and the skin lesions healed without additional treatment. The non-tender character of the lesions, the onset following introduction of azathioprine, and the rapid resolution with azathioprine withdrawal made AISS the most likely etiology. SS is a neutrophilic dermatosis characterized by the acute eruption of erythematous papules, nodules or plaques which may progress to pustules, bullae or ulcers, with systemic symptoms including fever and leukocytosis. The pathogenesis of the condition is unknown, but thought to involve a local or systemic dysregulation of cytokine secretion. SS can be associated with infection, IBD, pregnancy, malignancy, or certain medications and may necessitate treatment with systemic corticosteroid therapy. The treatment of AISS is unique in that withdrawal of the medication alone leads to resolution of symptoms. Clinicians treating patients with IBD should look for signs of AISS in patients who have recently started azathioprine or 6MP. The syndrome can be misinterpreted as an IBD-associated skin eruption or as infection. It is important to make the diagnosis early, as discontinuation of the drug is the necessary treatment. Re-challenge with azathioprine is not recommended. TPMT status is not thought to be related to the incidence of AISS. Therefore, testing for the genetic polymorphism will not help predict likelihood of this atypical reaction.Figure 1Figure 2

A rheumatoid arthritis patient with chronic recurrent annular neutrophilic dermatosis: Are we dealing with a new type of rheumatoid arthritis-associated dermatosis?

Abstract Neutrophilic dermatoses comprise a heterogeneous group of disorders, which are characterized by inflammatory skin lesions that share a common histopathological feature, intense inflammatory infiltration consisting primarily of neutrophils, with no evidence of vasculitis. We describe a 75-year-old man with rheumatoid arthritis, who presented with erythematous edematous plaques. The histopathological findings indicated neutrophilic dermatosis; however, the patient was afebrile and without an elevated white blood cell count or systemic involvement. We think that the most suitable expression for this variant with clinical manifestations different from those of classical Sweet syndrome is chronic recurrent annular neutrophilic dermatosis, as introduced by Christensen et al. 1

Histiocytoid Sweet Syndrome Is More Frequently Associated With Myelodysplastic Syndromes Than the Classical Neutrophilic Variant: A Comparative Series of 62 Patients.

Histiocytoid Sweet syndrome (H-SS) is a histological variant of Sweet syndrome (SS) differing from classical neutrophilic SS (N-SS) by a dermal infiltrate mainly composed of lymphocytes and histiocytoid myeloperoxidase-positive cells. We aimed to report a large series of H-SS and compare the frequency and type of hematological malignancies associated to H-SS and N-SS. We included 62 patients with a coding histopathologic diagnosis of SS prospectively registered between 2005 and 2014 in the database of our Department of Pathology. Overall, 22 (35.5%) and 40 (64.5%) patients had a histological diagnosis of H-SS and N-SS, respectively. Median age, sex ratio, and cutaneous lesions were similar in the 2 groups. The frequency of extra-cutaneous manifestations was similar (50% vs 37.5%, P = 0.42). Recurrent forms were significantly more frequent in H-SS than in N-SS patients (21% vs 2.5%, P = 0.01). A hematological malignancy was diagnosed in 22 patients, 12 (55.5%) with H-SS and 10 (25%) with N-SS (P = 0.019). Hematological malignancy was of myeloid origin in 8/22 (36.3%) H-SS and 5/40 (12.5%) N-SS patients (P = 0.02), and of lymphoid origin without myeloid component in 4/22 (18.1%) H-SS and 4/40 (10%) N-SS patients (P = 0.35), respectively. One N-SS patient had a hematological malignancy of mixed (myeloid and lymphoid) phenotype. A myelodysplastic syndrome (MDS) was diagnosed in 7/22 (31.8%) H-SS and 1/40 (2.5%) N-SS patients (P < 0.001). Hematological disease was diagnosed before (in 8 H-SS and 3 N-SS patients) or at the time of the occurrence of the cutaneous lesions (in 1 H-SS and 7 N-SS patients). However, in 3 H-SS patients, all with MDS, cutaneous lesions preceded the hematological disease by ≤6 months. In conclusion, H-SS was associated with MDS in one third of patients but also with lymphoid malignancies, and cutaneous lesions could precede the hematological diagnosis in patients with MDS. A complete hematological assessment is mandatory at diagnosis, and monitoring blood cell counts should be recommended for at least 6 months after the diagnosis of H-SS.

Sweet's syndrome in a patient with compound heterozygous mutations in the Mediterranean fever gene (MEFV)

Sweet's syndrome (SS) or acute febrile neutrophilic dermatosis is a rare disorder that often occurs in association with other systemic diseases. The disorder is characterized by development of nonpruritic, painful erythematous plaques with pseudovesicles, occasional pustules and rare bullae. SS consists of a triad of erythematous plaques infiltrated by neutrophils in association with fever and leukocytosis. The pathological features of SS involve the dermis. The condition presents in three clinical settings: classic (or idiopathic) SS, malignancy-associated SS and drug-induced SS syndrome. The treatment of choice for SS are systemic corticosteroids, although colchicine and potassium iodide are also considered to be effective for SS. We present an unusual recurrent course of SS in a 38 year old man who carries compound heterogous mutations in the MEFV gene. A 38 year old, generally healthy man from Sephardic Jewish ancestry, had suddenly developed fever, malaise, artralgia and painful erythematous plaques with pustules and bullae on the anterior aspects of the upper extremities. Diagnostic evaluation included moderate leucocytosis, elevated erythrocyte sedimentation and C-reactive protein rate and normal liver functions. antistreptolysin O-titer. Blood cultures and tuberculosis test were negative. Chest radiography was normal. The symptoms exacerbated despite treatment with systemic corticosterids. Clinical improvement appeared after administration of colchicine. Mutational analysis of the MEFV gene revealed compound heterozygous M694V and V726A mutations. Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent attacks of fever with serosal inflammation. The FMF gene (MEFV) encodes the protein pyrin that plays an important role in modulating the innate immune response. MEFV mutations have been identified primarily in patients from Mediterranean populations and in Israel the carrier state is as high as1: 5. Sweet's syndrome has been described in a patient with classical FMF as a possible new cutaneous feature and has never been described as a presenting sign of FMF. Although various skin lesions have been described with FMF, erysipelas-like erythema (ELE) has been reported the only pathognomonic cutaneous manifestation. Our patient, carrying compound heterozygous mutations in MEFV presented with Sweet's syndrome. We suggest, that SS skin lesions might be an only cutaneous presentation of FMF. Written informated consent for publication of their clinical details was obtained from the patient/parent/guardian/relative of the patient.

Chronic relapsing remitting Sweet syndrome--a harbinger of myelodysplastic syndrome.

Sweet syndrome (SS) is an acute febrile neutrophilic dermatosis. It has been associated with malignant disease, especially acute myeloid leukaemia (AML), infections, autoimmune disorders and drugs, particularly granulocyte colony-stimulating factor (GCSF). No cause is found in the rest, which are labelled idiopathic. We describe 15 patients with SS, which we believe represent 'immune dysregulation' secondary to myelodysplastic syndrome (MDS). We initially identified 31 patients with SS in a cohort of 744 patients with MDS and 215 with AML seen over a 6-year period (2004-10). The cause in 16 patients could be attributed either to administration of GCSF or chemotherapy. The eruption was brief and resolved spontaneously or following withdrawal of GCSF. Fifteen patients however, had a chronic debilitating illness dominated by the skin eruptions. Diagnosis of chronic relapsing SS was delayed because the pathology was not always typical of classical neutrophil-rich SS and included lymphocytic and histiocytoid infiltrates and bone marrow was not always performed because the relevance of the eruption to MDS was often not immediately appreciated. All these patients had 'low risk' MDS, diagnosed at a median of 17months (range 0-157) following the diagnosis of SS. We describe a chronic debilitating episodic clinically distinctive skin eruption with features of SS but not always definitive histopathology often associated with immunological abnormalities affecting other systems related to underlying low risk MDS.

Sweet syndrome: Clinical presentation, associations, and response to treatment in 77 patients

Sweet syndrome is a neutrophilic dermatosis with cutaneous tender lesions that can be associated with malignancies, infections, systemic inflammatory disorders, and medications. Although numerous studies have described Sweet syndrome, few studies have systematically investigated Sweet syndrome. We sought to describe characteristics and treatments of patients with Sweet syndrome and evaluate clinical differences depending on the underlying cause. A retrospective study was conducted to identify patients with Sweet syndrome evaluated at Mayo Clinic from 1992 to 2010. Of 77 patients with Sweet syndrome (mean age of onset 57 years), 43 (56%) were male. Eighteen patients (23%) reported a preceding infection. A total of 41 (53%) patients were classified as having classic Sweet syndrome, 27 (35%) patients had malignancy-associated Sweet syndrome, and in 9 (12%) patients drug-induced Sweet syndrome was considered. In all, 21 patients had a hematologic malignancy or myeloproliferative/myelodysplastic disorder, whereas 6 patients had solid tumors. The mean hemoglobin level, in both male and female patients (P < .0443 and P < .0035, respectively), was significantly lower in malignancy-associated versus classic and drug-induced Sweet syndrome. Systemic corticosteroids were the most frequently used treatment (70%). This is a retrospective study and represents patients from a single academic center. Sweet syndrome is a distinctive disorder with certain clinical and histologic characteristics, which usually has a complete response to systemic corticosteroids. It is important to evaluate Sweet syndrome patients who have laboratory evidence of anemia for an underlying malignancy.