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A woman in her 40s presented with a 3-month-long history of fever and tender erythematous bullous skin lesions not responsive to antibiotics. There had been no previous gastrointestinal, respiratory or urinary infection, nor did she have any history of autoimmune disease, drug reaction or vasculitis.Histological evaluation of skin biopsy showed diffuse dense neutrophilic infiltrates located in dermis diagnostic of Sweet syndrome. Haematological investigations showed leucocytosis with circulating immature cells, which on further investigations with bone marrow biopsy, were evident of chronic myelogenous leukaemia in the accelerated phase. Sweet syndrome was the presenting characteristic of chronic myelogenous leukaemia in this case, which is a rare association. Investigating unusual skin lesions can aid in the suspicion of underlying cancer, allowing for prompt action.

Granulocyte colony-stimulating factor (G-CSF) administration is associated with a diverse range of cutaneous sequelae. Serious dermatological side effects of G-CSF include the development of Sweet’s syndrome and exacerbations of pre-existing inflammatory disorders such as psoriasis. Here, we describe a report of acute leucocytoclastic vasculitis caused by G-CSF therapy associated with anti-Ro and anti-La antibodies in a patient with multiple myeloma. This case highlights the importance of having a high index of suspicion for acute leucocytoclastic vasculitis in patients with haematological malignancies undergoing G-CSF therapy.

Background Neutrophilic dermatosis of the dorsal hands is an uncommon localised variant of Sweet syndrome first described in 1995. It is characterised by tender erythematous plaques, pustules and bullae on the dorsa of the hands. Literature Review A total of 123 cases of NDDH are included in this review. The mean patient age was 62.1 years and there was a slight female preponderance. 78.0% of cases had reported bilateral involvement and other sites were affected in almost a third of cases. Underlying disease was found in around 40% of patients, with the most common associations being haematological disorders (gammopathies, myelodysplasias or malignancies), recent infection, solid organ tumours and inflammatory bowel disease. Systemic and/or topical corticosteroids were employed in the treatment of 88.1% of cases while dapsone, colchicine and tetracyclines were the commonest steroid-sparing agents used. Improvement was often rapid and complete resolution the norm. Conclusions Whilst being uncommon, NDDH is frequently misdiagnosed and thus, its exact prevalence is probably underestimated. This may have significant implications including treatment delays or incorrect management. Moreover, recognition of NDDH is important since a correct diagnosis should trigger a search for underlying diseases and proper treatment with corticosteroids and/or steroid-sparing agents which is almost invariably curative.

Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

Sweet’s syndrome (acute febrile neutrophilic dermatosis) consists of acute onset of painful cutaneous erythematous lesions, mostly found in the upper extremities followed by the head and neck region, particularly in patients with underlying malignancies. We describe the case of a woman in her mid-30s, who was treated for acute myeloid leukaemia and presented with a severe painful and progressive erythematous lesion of the retroauricular skin. Clinical features, laboratory tests, blood cultures and histological biopsy yielded a diagnosis of Sweet’s syndrome. The treatment consisted of oral and topical corticosteroids and her signs and symptoms resolved within 1 week. Although Sweet’s syndrome is uncommon, awareness among otolaryngologists is crucial to ensure a prompt diagnosis, cure and referral to an oncologist (if not already involved) for patients with Sweet’s syndrome in the head and neck area.

Deficiency of adenosine deaminase type 2 (DADA2) is an autosomal recessive monogenic autoinflammatory syndrome that is classically characterised by polyarteritis nodosa, systemic vasculitis and stroke. The spectrum of disease manifestations has broadened to encompass a range of cutaneous, vascular and haematological manifestations. We report a novel association in two sisters with heterozygous p.R169G/p.M309l mutations in ADA2 with low serum ADA2 activity who both presented similarly with clinical and histological features consistent with histiocytoid Sweet syndrome.

Abstract: Sweet’s syndrome is a serious dermatological disorder characterized by a rapid onset of tender plaques or nodules, fever, joint pain, headache, and oral and genital lesions. According to the clinical features and underlying causes, Sweet’s syndrome is divided into three categories, i.e., classi-cal (or idiopathic), malignancy-associated Sweet's syndrome, and drug-induced Sweet's syndrome. It is multifactorial in etiology, and the exact cause is still undetermined. The diagnosis can be confirmed by the routine histopathologic evaluation of skin biopsy from the lesions. The first-line treatment options are topical and systemic steroids. Multiple databases, like Medline/PubMed, Scopus, and Google, were used to identify resources for this literature review. The relevant information was col-lected from various case reports, case series, reviews, meta-analyses, and large clinical trials reporting clinical description, etiology, diagnosis, and management of Sweet’s syndrome. This narrative review aimed to discuss recent understandings related to Sweet's syndrome, both in terms of clinical presen-tation and management approach.

VEXAS (Vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic mutation) syndrome is a genetically defined disorder identified in 2020, describing patients with inflammatory syndromes associated with haematological dysfunction. It is a severe, treatment-resistant condition, with estimated mortality between 40% and 63%. A wide range of cutaneous manifestations have been described. Here, we report on two patients with treatment-resistant neutrophilic dermatosis and myelodysplastic syndrome, who were subsequently diagnosed with VEXAS syndrome. Our cases highlight the need for dermatologists' awareness of this novel condition and to initiate early referral to haematologists for appropriate multidisciplinary care.

Sweet syndrome (SS), also referred as acute febrile neutrophilic dermatosis, is an inflammatory process characterised by the abrupt appearance of erythematous papules or nodules with predominant neutrophilic infiltration in the dermis. Fever and neutrophilia are common presenting features. However, extracellular manifestations, including ocular and musculoskeletal, may occur. SS is divided into three subtypes: classical (or idiopathic), malignancy associated and drug induced. Medication-induced subtype accounts for up to 26% of cases. In recent years, emerging evidence has showed that SS may also occur in neutropenic patients who underwent induction for acute myeloid leukemia (AML). The identification of FMS-like tyrosine kinase 3 (FLT3) gene mutation in approximately 30% of patients with AML has promoted the targeted therapy with FLT3-internal tandem duplication (ITD) inhibitors. Midostaurin, a recently Food and Drug Administration-approved medication for FLT3-ITD-positive AML, was reported once as cause for SS. We report a midostaurin-induced SS with neutropenia in a patient following induction chemotherapy of AML

Diagnosing and treating neutrophilic dermatoses (NDs) in clinical practice can be challenging because of various presentations and stubborn treatment responses. Establishing a diagnosis is necessary, though, because many NDs are associated with underlying conditions, including malignancy. In this article, the authors provide information about Sweet syndrome, pyoderma gangrenosum, and other NDs and describe their clinical presentation, pathophysiology, diagnostic criteria, and associated conditions. The authors also present a case report describing the coexistence of two NDs and hidradenitis suppurativa in one patient and review the treatment modalities for those conditions.

Azathioprine hypersensitivity syndrome is a rare but potentially severe side effect of azathioprine use. It has a variable and non-specific presentation making it difficult to distinguish from sepsis or disease relapse. High clinical suspicion is therefore required for recognition and prompt cessation of azathioprine for symptom resolution. Herewith two cases of severe azathioprine hypersensitivity syndrome are described, one in association with Sweet syndrome. Both presented with vague symptoms 2 weeks after commencing azathioprine for antineutrophil cytoplasmic antibody vasculitis. The differentials of sepsis and disease relapse were considered prior to cessation of azathioprine which resulted in a dramatic improvement in both cases. These cases highlight the diagnostic challenge azathioprine hypersensitivity syndrome presents. It should be suspected when there is a temporal relationship to drug initiation, with absence of infection or serological evidence of disease relapse.

Sweet syndrome is a neutrophilic dermatosis occasionally associated with malignancies. Due to its rarity, the clinical features of Sweet syndrome are still unclear. Thus, we aimed to analyze clinical features, treatment, and outcomes of these patients according to associated disease. We conducted a retrospective, longitudinal cohort study from January 2000 to August 2020. We reviewed the medical records of 52 patients with Sweet syndrome. The median age of patients was 57.5 years old (range, 17-84), and 48.1% were female. Of the 52 patients analyzed, 27 patients (51.9%) had malignancy-associated Sweet syndrome. Sweet syndrome was diagnosed concurrently with (N = 8), before (N = 5), and after (N = 14) the diagnosis of malignancy. The idiopathic Sweet syndrome was most common in the non-malignancy group (56.0%). Myelodysplastic syndrome was the most common malignancy associated with Sweet syndrome (47.6%). Leukopenia (p = 0.005), anemia (p 0.001), and thrombocytopenia (p = 0.008) were significantly associated with malignancy. The majority of patients showed rapid improvement of symptoms after steroid administration. The symptoms of some patients with malignancy did not improve with steroid alone; however, their symptoms often improved when steroids were combined with a treatment for the associated malignancy. Relapse and aggravation of Sweet syndrome were common in the malignancy group. Sweet syndrome showed a broad spectrum of clinical features related to various diseases. Sweet syndrome often occurred as a paraneoplastic feature. Therefore, active systemic evaluation is needed in the first diagnosis of Sweet syndrome without clear etiology.

Histiocytoid Sweet syndrome (HSS) is an uncommon histologic variant of Sweet syndrome (SS). HSS can be distinguished from the classic SS with an infiltrate of histiocyte-like immature myeloid cells rather than dense neutrophilic infiltration, although the clinical features are similar. Previous studies have shown that the risk of hematologic malignancy is significantly higher in HSS compared with classic SS. To lesser extent, HSS is also associated with infections, inflammatory diseases, and drugs, particularly with antineoplastic agents as well. Here, we report a case of 2 patients with an abrupt onset of erythematous, tender plaques accompanied by fever, with that revealed similar histopathologic and immunohistochemical features, whom had a history of antibiotic use. Clinicopathologic correlation led to diagnosis of drug-induced HSS, associated with the use of levofloxacin and amoxicillin-clavulanate, respectively. Both patients were then successfully treated with systemic corticosteroid therapy, and neither of them had recurrence during the period of 24-month follow-up.

Behçet disease and its related disorder, Sweet disease, are multifactorial disorders whose susceptibility loci have been identified in the genes of various immunological factors aside from human leukocyte antigens. The neurological involvement of these diseases, including encephalitis, myelitis, and meningitis, referred to as neuro-Behçet disease (NBD) and neuro-Sweet disease (NSD) respectively, is sometimes difficult to diagnose, especially when the characteristic mucocutaneous symptoms do not precede neurological symptoms or when characteristics of both diseases are present in a single patient. NBD and NSD constitute a spectrum of diseases that are differentiated according to the combination of risk factors, including the genetic background. Encephalitis, myelitis, and meningitis similar to NBD or NSD can be diagnosed as spectrum disorders, even if the characteristic mucocutaneous symptoms fail to be detected. Understanding these conditions as a disease spectrum may help elucidate the disease pathogenesis and assist in the development of therapeutic agents.

Tumor necrosis factor (TNF) α inhibitors are widely used to treat inflammatory bowel disease (IBD); however, some patients have unexpected inflammatory episodes during anti-TNF therapy. The objective of our research was to highlight a paradoxical case of anti-TNF-agent-induced Sweet syndrome compared with Sweet syndrome treated by anti-TNF agents. We describe a 62-year-old male with a history of ulcerative colitis presenting with multiple polymorphic indurated skin macules and plaques after 2 months of adalimumab therapy. Neutrophilic dermatosis was diagnosed based on the clinical presentation and skin biopsy and may have resulted from extraintestinal manifestations of a flare-up of IBD or been induced by adalimumab therapy. We conclude that when facing this dilemma, adalimumab should be discontinued, and the dose of prednisolone should be increased before determining the definitive cause. Based on drug hypersensitivity syndrome (DHS) risk assessment in the 10-D assessment system, this case was classified as grade 1 (no risk). Finally, we review the molecular and cellular mechanisms connecting cytokine dysregulation to Sweet syndrome.

Background Sweet’s syndrome (SS), also known as acute febrile neutrophilic dermatosis, is a rare neutrophilic dermatitis characterized by pyrexia, neutrophilia and painful papulonodular lesions with a neutrophilic dermal infiltrate. Case presentation We presented a case report of classical SS associated with ulcerative colitis (UC) and mucosal prolapse polyps (MPPs) in a male patient. Conclusions The particularity of this case is the occurrence of MPPs in a male patient with UC and classical SS. We also discussed whether this patient with concurrent Epstein–Barr virus infection could be treated with corticosteroids.

Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, was first reported in 1964 by Dr Robert Douglas Sweet.1 The entity is characterized clinically by an abrupt cutaneous eruption of painful erythematous nodules and plaques accompanied by fever and leukocytosis.2 On histopathologic examination, prominent papillary dermal edema and a dense dermal infiltrate of neutrophils are classic.3 In 2005, Requena et al4 described a variant termed “histiocytoid Sweet syndrome” (HSS). HSS, although clinically indistinguishable from its classic counterpart, demonstrates an inflammatory infiltrate composed of myeloperoxidase (MPO)-positive immature mononuclear cells with a histiocytoid morphology rather than neutrophils.2,4,5 These immature mononuclear cells simulate histiocytes both in appearance and with CD68 staining.2,5 Therefore, MPO staining is necessary to confirm that these cells are neutrophil precursors and not true histiocytes.5 Sweet syndrome has been associated with underlying systemic diseases of infectious, inflammatory, autoimmune, and neoplastic nature.3 Children account for only 5% to 8% of all cases worldwide.6 HSS in the pediatric population is even more rare, with only 5 cases previously reported, none of which were in an infant without an underlying association.2,7, 8, 9

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors’ intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Induction therapy with all-trans retinoic acid (ATRA) is effective for acute promyelocytic leukemia (APL). ATRA induces neutrophil differentiation and its associated side effects. The differentiation syndrome is the most characterized ATRA-induced adverse effect. Sweet's syndrome, also known as neutrophilic dermatosis, is another form of ATRA-associated disease characterized by neutrophil infiltrating erythema that develops with fever. This is a case of a 34-year-old Japanese man diagnosed with APL. At the onset, the patient did not have skin involvement of APL cells. He was treated with ATRA and induction chemotherapy with idarubicin and cytarabine. Scrotal skin rash occurred at day 14, which developed into scrotal ulceration up to day 28 even after eliminating APL cells in his peripheral blood. Sweet's syndrome is a pathological diagnosis of scrotal skin ulceration representing neutrophil infiltration. The infiltrating neutrophils showed PML-RARα rearrangement. The patient was diagnosed with ATRA-associated Sweet's syndrome with skin ulcer. His cutaneous lesion did not respond to intravenous prednisolone therapy; thereby, ATRA was discontinued. After the cessation of ATRA, the skin lesion improved in the next week. We confirmed he achieved a complete response after induction chemotherapy. In our observation, ATRA-associated Sweet's syndrome is characterized by the following clinical manifestations: preferable occurrence in the scrota, tend to progress into skin ulcer, and pathogenicity associated with PML-RARα-positive matured neutrophils. The etiology, pathogenesis, and risk factors of ATRA-associated scrotal ulceration were discussed in the literature review.

Sweet's syndrome (SS) also known as acute febrile neutrophilic dermatosis, is an uncommon disease characterised by acute onset of tender, violaceous or erythematous, oedematous papules, nodules or plaques, with fever. It is classified into classic, malignancyassociated, and drug-induced subtypes.The aims of this study is to evaluate the subtypes, clinical features, laboratory profiles, and treatment of patients with SS.We did a retrospective medical record review of all patients with SS from July 2014 to July 2018 at Hospital Queen Elizabeth and Hospital Pulau Pinang, both tertiary hospitals in Malaysia.Twenty-nine patients were included. Approximately half of the patients (15) were females with a mean age of onset of 50.93 (± 11.52) years. The most common subtype was classic (62.0%) followed by malignancy-associated (31.0%) and drug-induced (6.9%). Among the patients with the classic subtype, infective-related causes (50.0%) were the most common. Among the patients with malignancy, eight had haematological malignancy and one had a solid tumour. Two-third of the malignancies were diagnosed within a year after the diagnosis of SS. Eight of our patients in Sabah had mycobacterial infections with three having concomitant haematological malignancies. Patients with malignancy-associated SS had lower mean haemoglobin (p=0.018) and mean platelet count (p=0.031). Itch was associated with the presence of pustules (p=0.038). Histopathological examination of all skin lesions showed dermal neutrophilic infiltrates and 25 (86.2%) of them had papillary dermal oedema. The study was limited by its retrospective design. The sample size was small likely due to the uncommon occurrence of this condition.SS is an uncommon dermatosis with distinctive clinical and histopathological features. Screening for underlying malignancy is essential especially for those who present with anaemia, thrombocytopenia, and pathergy phenomenon. Mycobacterial infection should be considered in this region due to high tuberculosis burden.

A 58-year-old woman presented to the emergency department with a 3-day history of painful, reddish skin lesions over her legs and high-grade fever. Her prior medical and surgical history was unremarkable. She denied a history of similar lesions, and she was not taking any medication prior to the onset of these lesions. (

The aim of this article is to present the case of acute febrile neutrophilic dermatosis (Sweet syndrome-SS) after Ad26.COV2.S vaccination against SARS-CoV-2. To the best of our knowledge, this is the second case of SS provoked by this specific vaccine. What is more, the mildly symptomatic beginning of the disease, later followed by typical SS manifestation with a variety of symptoms including nodular erythema of the feet and oral ulcerations, made it very challenging to establish the diagnosis. The article focuses on the current literature on the acute febrile neutrophilic dermatosis, along with the coexistence with other neutrophilic dermatoses and anti-SARS-CoV-2 vaccinations as provoking factors. It emphasizes the necessity for sharing the knowledge and experience on the subject of SS's clinical manifestations and underlying causes to facilitate prompt diagnosis and introduction of appropriate treatment.

Paraneoplastic autoimmune and inflammatory disorders are often associated with myelodysplastic syndromes (MDS). The etiopathogenesis of MDS-associated autoimmune and inflammatory disorders is still unclear and treatment options are limited. Patients with MDS are at high risk of infections, which can be increased by the use of steroids. In the present study, we report on two patients with MDS-related autoimmune and inflammatory disorders who were in remission and reduced the steroid dose with 5-azacytidine treatment. The first case was a 67-year-old patient diagnosed with MDS and the whole-body erythroderma was the chief complaint. When the patient was treated with decitabine, steroid treatment was needed to control the erythroderma. When we changed decitabine to 5-azacytidine, both his erythroderma and his dependency on the steroid treatment were resolved. The second patient was a 68-year-old man with MDS who presented with Sweet's syndrome. Sweet's syndrome was completely treated after the first cycle of 5-azacytidine. In addition, Sweet's syndrome can occur as an adverse reaction of 5-azacitidine, so we illustrate that it is important to distinguish whether Sweet's syndrome is MDS-related skin disorders or 5-azacitidine-related skin side-effects.

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an uncommon skin eruption characterized by fever, leukocytosis, and tender erythematous papules, nodules, and plaques. Histopathologically, SS lesions are characterized by marked superficial papillary edema with a dense neutrophilic infiltrate. SS is known to demonstrate both the Koebner phenomenon and pathergy. The majority of reported cases of these phenomena occur following significant cutaneous injury (e.g., biopsies, burns) rather than minor trauma such as pressure and friction. Here, we present the first known reported case of SS koebnerization secondary to minor grooming-related hair plucking. In addition, this is also the first reported case to our knowledge of SS with perifollicular involvement on histopathology.

Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.

There is paucity of data regarding the diagnostic yield and safety of skin biopsies in patients with acute myeloid leukemia (AML), though skin eruptions are common in these patients. We evaluated 216 patients treated in our hemato-oncology unit at a tertiary medical center between 2007 and 2018 and identified 35 patients who underwent 37 skin biopsies. The majority of biopsies were performed during induction treatment for AML (n = 26, 70%), whereas the remainder of biopsies were done prior to induction initiation (n = 8, 22%) or during consolidation chemotherapy (n = 3, 8%). Pathology findings were inconclusive in 13 cases (35%), while diagnostic biopsies were positive for drug eruptions (24%), leukemia cutis (16%), infections (11%), reactive processes (8%) and Sweet syndrome (5.5%). In almost half of cases (16/37) tissue cultures were performed. Of those, only a quarter (4/16) were positive. Histopathology and tissue culture results altered immediate patient care in 3 cases (8%), yet information obtained from biopsies had potential to affect long term patient care in 8 additional cases (21.6%). Although most skin biopsies were performed while patients had severe thrombocytopenia and neutropenia, only one patient had a complication due to the biopsy (fever and local bleeding). With the limitation of a retrospective analysis, our study suggests that skin biopsies in patients treated for AML are relatively safe. Although biopsy results infrequently alter immediate patient management, long term effect on patient care expand the potential diagnostic yield of skin biopsies.

Sweet Syndrome (SS) belongs to a group of diseases known as neutrophilic dermatoses. An uncommon variant named Histiocytoid Sweet Syndrome (HSS) can be associated with a variety of conditions, including cancer, infections, drug toxicity and others. Here we present an instance of HSS in an HIV-positive patient in the setting of an infectious disease.

We describe a strikingly robust presentation of trimethoprim-sulfamethoxazole (TMP-SMX)-induced pustular Sweet syndrome and discuss how to distinguish it from iododerma and other neutrophil-rich conditions. A review of the literature indicates that TMP-SMX-induced Sweet syndrome (SS) may have higher rates of neutrophilia and greater ocular, mucosal, and musculoskeletal involvement compared to SS from other drugs. Recognizing these features and identifying the offending agent are critical for correctly diagnosing TMP-SMX-induced SS in a timely manner.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS syndrome) is a recently described genetic disorder that gathers autoinflammatory symptoms and myeloid dysplasia. The first description was reported in 2020, and subsequently, a growing number of cases have been described worldwide. Herein, we describe a case of a 72-year-old male patient with VEXAS syndrome with p.Met41Val mutation of the UBA1 gene, prominent supraglottic larynx involvement, and costochondritis. To our knowledge, this is the first report of VEXAS syndrome in Colombia and South America. This disease could present features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. Supraglottic larynx chondritis and costochondritis are atypical manifestations. These features were proposed previously to differentiate relapsing polychondritis from VEXAS syndrome but are not entirely reliable like in the case described. A diagnosis of VEXAS should be considered in male patients with incomplete or complete features of the previously described conditions, refractory to treatment, requiring high-dose glucocorticoids, and associated progressive hematologic abnormalities. Key Points • VEXAS syndrome is a recently described genetic (somatic mutations in UBA1 gene) disorder that gathers autoinflammatory and hematologic manifestations. • VEXAS syndrome should be considered in male patients with incomplete or complete features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, and Sweet syndrome, refractory to treatment, associated with hematologic involvement, including cytopenias, myelodysplastic syndrome, or thromboembolic disease. • Glucocorticoids ameliorate symptoms effectively. However, other treatment options are limited due to a lack of evidence. Traditional immunosuppressants and biological therapy have been used empirically with limited efficacy and a transient effect. Bone marrow transplant offers a curative approach, but it has high morbidity and mortality.

International audience; Neutrophilic dermatoses (ND) are a group of inflammatory skin conditions characterized by a neutrophilic infiltrate on histopathology with no evidence of infection. ND are classified based upon the localization of neutrophils within the skin and clinical features. Recent findings suggest that ND are due to two main mechanisms: i) a polyclonal hereditary activation of the innate immune system (polygenic or monogenic); or ii) a clonal somatic activation of myeloid cells such as encountered in myelodysplastic syndrome or VEXAS syndrome. ND belong to internal medicine as a great number of patients with ND suffer from an underlying condition (such as hematological malignancy, inflammatory bowel disease, auto-immune and auto-inflammatory diseases). ND are diagnoses of exclusion and physicians should always consider differential diagnoses, particularly skin infections. Here, we review the pathophysiology and classification of the main ND (i.e., subcorneal pustular dermatosis (Sneddon-Wilkinson Disease) and Intercellular IgA dermatoses, aseptic pustulosis of the folds, Sweet syndrome, neutrophilic eccrine hidradenitis, pyoderma gangrenosum, erythema elevatum diutinum, neutrophilic urticarial dermatosis and neutrophilic panniculitis), their clinical and histopathological features, and we highlight the investigations that are useful to identify ND-associated diseases and to exclude the differential diagnoses.

Acute myeloid leukemia (AML) is the most common acute leukemia in American adults and portends a poor prognosis if untreated. Commonly, AML presents with symptoms related to concurrent leukopenia, anemia, or thrombocytopenia; however, due to its ability to affect many organ systems in the body, AML can have a highly varied clinical presentation. One such presentation is myocarditis, which is a rarely reported manifestation of AML. Myocarditis can have a varied clinical picture and often requires exclusion of other causes of cardiac dysfunction. Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is another presentation of AML; however, it is more commonly associated with AML than cardiac involvement. Sweet syndrome can occur in patients with an already established malignancy or can occur de novo in a patient with previously undiagnosed cancer and, interestingly, can also be accompanied by extracutaneous manifestations, one of which is myocarditis. Herein, we report a case of a 45-year-old male with a history of obesity and depression who presented with chest pain, a tender and diffuse rash, and pancytopenia. Heart catheterization performed at outside institution was negative for coronary artery disease. Cardiac MRI images were compatible with myocarditis. Dermal biopsy of the rash was consistent with sweet syndrome. Peripheral blood flow cytometry and bone marrow biopsy confirmed the diagnosis of AML. He was treated with an induction chemotherapy regimen of 7 days of cytarabine and 3 days of daunorubicin with resolution of his chest pain and skin lesions. The patient had persistent leukemia cells on day 14 postinduction bone marrow biopsy and was treated with high-dose cytarabine reinduction treatment. Bone marrow biopsy with count recovery after reinduction therapy revealed complete response (CR).

Sweet syndrome is an inflammatory disease characterized by fever, neutrophilia, papules and erythematous plaques, and a skin neutrophilic infiltrate. Syphilis has been reported among the infectious causes of Sweet syndrome. Syphilis can present atypical manifestations; a rare presentation is nodular syphilis, characterized by nodules with granulomas and plasma cells at histopathology. This case report presents a 20-year-old woman patient, with plaques and nodules, and systemic symptoms. The histopathological exam revealed both non-tuberculoid granulomas and a dense infiltration of polymorphonuclear neutrophils in the dermis. These findings, plus laboratory abnormalities, characteristic of both conditions, were conclusive for Sweet syndrome and nodular syphilis association.

Background and AimsSweet syndrome [SS] is a dermatological condition associated with both inflammatory bowel disease [IBD] and azathioprine use. We performed a systematic review to better delineate clinical characteristics and outcomes of SS in IBD patients.MethodsPeer-reviewed, full-text journal publications from inception to April 2020 in English language and adult subjects with IBD were included. Skin biopsy was required as SS gold-standard diagnosis. Azathioprine-associated SS required recent azathioprine introduction or recurrence of SS after azathioprine re-challenge.ResultsWe included 89 publications with 95 patients [mean age of SS diagnosis: 44 years; 59% female; 20 with azathioprine-associated SS and 75 without]. SS was diagnosed prior to IBD in 5.3%, at time of IBD diagnosis in 29.5% and after diagnosis in 64.2%. In total, 91% of patients with SS had known colonic involvement and the majority [76%] had active IBD at diagnosis; 22% had additional extra-intestinal manifestations. Successful therapies for SS included corticosteroids [90.5%], anti-tumour necrosis factor [TNF]-α inhibitor therapy [14.8%] and azathioprine [11.6%]. Azathioprine-associated SS was distinct, with 85% male patients, mean age of SS diagnosis of 50 years and a lower likelihood to be prescribed corticosteroids for treatment [75% vs 94.7% of non-azathioprine-associated SS, p = 0.008]. All patients with azathioprine-associated SS improved with medication cessation and developed recurrence after re-challenge.ConclusionsSS may precede or occur with IBD diagnosis in almost one-third of cases. Azathioprine and IBD-associated SS present and behave distinctly, especially with regard to gender, age at diagnosis and recurrence risk. Corticosteroids and TNF-α inhibitors have demonstrated efficacy in treating SS in IBD.

Syphilis has received its classical designation as one of “the great imitators,” reflecting a wide variety of symptoms and presentations, which can cause difficulties in diagnosis. Here we report an unusual case of secondary syphilis in a person with acute necrotizing tonsillitis and Sweet syndrome. A 33-year-old female presented with fever, bilateral cervical lymphadenopathy, tonsillar enlargements with ulcerated pus-filled lesions on the right tonsil, and multiple pseudovesicular, mammillated, edematous plaques on her neck, face, and extremities. Syphilis serology was positive and a skin biopsy demonstrated a neutrophil-rich dermatitis characteristic of Sweet syndrome. The association of Treponema pallidum infection with Sweet syndrome may be a coincidence; nevertheless, our case serves as a reminder that secondary syphilis should remain in the differential diagnosis of the acute febrile neutrophilic dermatosis.

Sweet syndrome aka acute febrile neutrophilic dermatosis is one of the serious dermatological condition which goes undiagnosed due to several reasons. Unawareness of this condition may be one of the primary factors for missing the diagnosis which may lead to inaccurate treatment which may only lead to worsening the condition in a sick individual. Very few studies have been published in this area, hence the present investigator felt the need of reviewing few literatures and creating a material which will aid in early and accurate diagnosis of the condition. About 20 literature was reviewed based on the history, presentation, diagnosis and its probable homoeopathic treatment accordingly. All the information required for accurate diagnosis and its homeopathic approach was compiled into the present paper.

Pyoderma gangrenosum (PG) is a rare skin disease of chronic course and a tendency for recurrence. Pathergy phenomenon, which is characterized by the rapid development of skin lesions at the site of a mechanical trauma, affects about 30% of patients with PG. However, this symptom is not pathognomonic for PG and may develop in other conditions including Behcet’s disease, Sweet syndrome and Crohn’s disease. In the current paper, we present a 25-year-old woman with an infiltrative lesion with a tendency for ulceration located in the interscapular region. Excision of the skin lesion led to the development of infiltration with small ulcerations at the site of surgical intervention. Clinical presentation corresponded to the classical pathergic reaction, which in turn raised the suspicion of PG. Therapy consisted of cyclosporin 5 mg/kg/day and topical ointment with 0.05% of betamethasone and 0.1% of gentamicin. Complete healing was observed after three weeks of treatment. We present the case in order to draw attention to the diagnostic significance of pathergy phenomenon in dermatology.

Acute Febrile Neutrophilic Dermatosis, also known as Sweet Syndrome, is an uncommon inflammatory disorder. Though the exact etiology is unclear, it has been presented in association with various entities. The majority of cases present following upper respiratory infections or viral gastroenteritis. Other causes include drug-induced reactions, pregnancy-related manifestations, or in association with specific hematologic or solid tumors. Rarely, it has been associated with Coccidioidomycosis, a prevalent fungus endemic to the Southwestern regions of the United States with a literature review revealing only three previous cases of Coccidioidomycosis-associated Sweet Syndrome. Here we report two new cases in individuals residing in Arizona.

Neutrophilic dermatoses (NDs) are a group of reactive, noninfectious autoinflammatory diseases characterized by (1) infiltration of the epidermis, dermis, and or/hypodermis by neutrophils; (2) their association with distinct diseases (eg, hematologic malignancy and chronic inflammatory diseases); (3) potential extracutaneous involvement; and (4) response to anti-inflammatory drugs, such as corticosteroids, dapsone, colchicine, and novel biologic therapies, such as the anti-interleukin-1 blockade. Although distinct NDs have been described, transitional forms with overlapping features are often identified. These justify a simplified classification of NDs with three major forms: superficial (epidermal or pustular) NDs, dermal (en plaques) NDs, and deep NDs. We review selected or novel variants of NDs, including subcorneal pustular dermatosis, the group of immunoglobulin A neutrophilic dermatoses, amicrobial pustular dermatosis of the folds, and neutrophilic urticarial dermatosis, as well as atypical forms of Sweet syndrome and pyoderma gangrenosum closely mimicking severe infectious diseases. Knowledge of these variants is essential for proper diagnosis, adequate management, and avoidance of a dangerous escalation of therapy, such as unnecessary immunosuppression or extensive surgery.

We report a 57-year-old man with recurrent meningoencephalitis resulting in bouts of altered consciousness, encephalopathy, tremors, focal seizures, and paraparesis. The neurological manifestations were accompanied by fever and leukocytosis in the absence of other systemic manifestations. MRI abnormalities of the brain, brainstem, spinal cord and meninges and CSF pleocytosis and elevated protein were observed. Exhaustive studies failed to reveal an etiology. Brain biopsy revealed nodules of neutrophils and macrophages, but no vasculitis. The lesions were not vasocentric as would be expected with neuro-Behcet's disease and neuro-Sweet's disease. The disorder was responsive to high-dose corticosteroid therapy and, ultimately, to anakinra, an IL-1α and IL-1β receptor antagonist.

We report a rare case of xanthomatized Sweet's syndrome with myelodysplastic syndrome (MDS) in a patient who presented with erythematous plaques on his chest that were elevated and became yellowish. A diagnosis of MDS with single lineage dysplasia was made during the development of the eruption. Bone marrow biopsy showed an increased number of megakaryoblasts. Histopathologically, there was neutrophil infiltration with leukocytoclasia and the infiltration of xanthomatous cells. Immunohistochemical analysis revealed that the xanthomatized cells were predominantly CD163 positive. We propose that our case of xanthomatized neutrophilic dermatosis is a rare clinicopathological variant of Sweet's syndrome associated with a hematologic disorder.