Persistent Sweet syndrome post-hematopoietic stem cell transplantation heralding molecular relapse of myelofibrosis.

Persistent Sweet syndrome in a patient with history of myelofibrosis thought to be in remission post-hematopoietic stem cell transplantation leads to diagnosis of molecular relapse of myelofibrosis.

A diagnostic challenge-First case of chronic lymphatic leukemia-associated necrotizing sweet syndrome.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a rare disorder typically characterized by the clinical triad including a sudden onset of fever, painful skin lesions, and neutrophilia. The histopathological findings are a dense neutrophilic infiltrate and oedema of the dermis and epidermis without evidence of a vasculitis. Besides treatment of the underlying cause, sweet syndrome is typically treated with high-dose corticosteroids leading to a relapse-free response in 70% of patients. However, if left unrecognized or untreated, the condition may lead to serious complications. Here, we report on the case of a 38-year-old patient in whom, under the assumption of the presence of necrotizing fasciitis, exarticulation of the right arm was performed. In the absence of pathogen detection and insufficient response to anti-infective therapies, the diagnosis of a sweet syndrome was assumed and, later, confirmed by an excellent response to high-dose administration of systematic glucocorticoids. The case emphasizes the need to be aware of this rare syndrome, which can be easily misdiagnosed due to its close resemblance to infection and stresses the need of further research to define distinct diagnostic tools.

Sweet Syndrome Associated with Active Inflammatory Bowel Disease: A Case Series of a Rare Extra-intestinal Manifestation.

BACKGROUND: Cutaneous extra-intestinal manifestations (EIM) occur in up to 20% of patients with IBD. Information about Sweet syndrome (SS)'s clinical course as a rare cutaneous EIM in IBD is limited to case reports. We present the largest retrospective cohort on the occurrence and management of SS in IBD. STUDY: Electronic medical records and paper charts since 1980 were retrospectively reviewed at a large quaternary medical center to identify all adult IBD patients with histopathology-proven SS. Patient characteristics and clinical outcomes were evaluated. RESULTS: 25 IBD patients with SS were identified; 3 patients were assessed to have AZA-induced SS. The majority of SS patients were female. Median age at diagnosis was 47 years (IQR 33-54 years) and SS appeared at a median of 6.4 years after IBD diagnosis. IBD patients with SS had a high rate of complicated IBD phenotypes (75% extensive colitis in UC and 73% stricturing or penetrating disease in CD, with 100% colonic involvement), as well as frequent co-occurring EIMs (60%). SS correlated with global IBD disease activity. Corticosteroids were an effective therapy for SS in IBD. Recurrence rate of SS was 36%. CONCLUSION: Contrary to previous case reports, SS was a cutaneous EIM occurring late after diagnosis of IBD in our cohort, with occurrences paralleling global IBD disease activity. Although AZA-induced and IBD-associated SS were both effectively treated with corticosteroids, distinguishing them is relevant for future IBD treatment strategies.

Reactive Neutrophilic Dermatoses in Adult-Onset Immunodeficiency due to Interferon-Gamma Autoantibody and Their Associated Factors.

BACKGROUND: Adult-onset immunodeficiency (AOID) due to interferon-gamma autoantibody is a rare, acquired immunodeficiency disease. Reactive neutrophilic dermatoses (RND), predominantly Sweet syndrome (SS), and generalized pustular eruption have been reported repeatedly. OBJECTIVES: The aims of this study were to describe the cutaneous manifestations in AOID patients and determine the incidence of RND and associated factors using a larger population size than have been previously reported. METHODS: A retrospective chart review of all confirmed AOID cases in Chiang Mai University Hospital from January 2006 to June 2020 was conducted. The demographics and characteristics of RND including type, onset, and laboratory information in every episode of cutaneous manifestations were collected. Generalized estimating equations of binary logistic regression were used to determine the indicators of RND. RESULTS: A total of 146 patients with confirmed AOID were identified. Of these, 57 cases (39%) developed at least one episode of RND. Thirteen cases (23%) of the patients experienced RND twice during the follow-up period. All recurrence of RND displayed the same cutaneous phenotype, with the exception of 2 cases who had both SS and generalized pustular eruption. Finally, 49 episodes of SS and 22 episodes of generalized pustular eruption were included in the analysis. All patients with RND had concomitant active opportunistic infections, of which most were non-tuberculous mycobacterium (NTM) infection. NTM infection (prevalence odds ratio [POR] 2.87), lymphadenopathy (POR 3.30) as well as lower serum alkaline phosphatase (ALP) level (POR 0.71 for every 100-unit increment in ALP) were found to be significantly associated with RND occurrence. CONCLUSIONS: 39% of our AOID patients experienced RND once during the course of the disease. Notable factors associated with RND occurrence were concomitant NTM infection, lymphadenopathy, and lower level of ALP.

Bullous Sweet syndrome as a presentation of chronic myelogenous leukaemia.

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.

New Practical Aspects of Sweet Syndrome.

Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is an inflammatory, non-infectious skin reaction characterized clinically by tender, erythematous papules/plaques/pustules/nodules commonly appearing on the upper limbs, trunk, and head and neck; histologically, SS is characterized by dense neutrophilic infiltrate in the dermis. SS is accompanied by fever; an elevation of inflammatory markers (e.g., erythrocyte sedimentation rate, C reactive protein) in serum may also be observed. Although most cases of SS are idiopathic, SS also occurs in the setting of malignancy or following administration of an associated drug. SS has also been reported in association with pregnancy and a burgeoning list of infectious (most commonly upper respiratory tract infections) and inflammatory diseases; likewise, the litany of possible iatrogenic triggers has also grown. Over the past several years, a wider spectrum of SS presentation has been realized, with several reports highlighting novel clinical and histological variants. Corticosteroids continue to be efficacious first-line therapy for the majority of patients with SS, although novel steroid-sparing agents have been recently added to the therapeutic armamentarium against refractory SS. New mechanisms of SS induction have also been recognized, although the precise etiology of SS still remains elusive. Here, we catalogue the various clinical and histological presentations of SS, summarize recently reported disease associations and iatrogenic triggers, and review treatment options. We also attempt to frame the findings of this review in the context of established and emerging paradigms of SS pathogenesis.

A case of recurrent acute febrile neutrophilic dermatosis in a patient with idiopathic cytopenia of undetermined significance.

This is the first report of recurrent acute febrile neutrophilic dermatosis in a patient with idiopathic cytopenia of undetermined significance. The patient progressed to acute myeloid leukaemia 4 months after onset.

Acute encephalitis, myoclonus and Sweet syndrome after mRNA-1273 vaccine.

A patient presented with fever, generalised rash, confusion, orofacial movements and myoclonus after receiving the first dose of mRNA-1273 vaccine from Moderna. MRI was unremarkable while cerebrospinal fluid showed leucocytosis with lymphocyte predominance and hyperproteinorrachia. The skin evidenced red, non-scaly, oedematous papules coalescing into plaques with scattered non-follicular pustules. Skin biopsy was consistent with a neutrophilic dermatosis. The patient fulfilled the criteria for Sweet syndrome. A thorough evaluation ruled out alternative infectious, autoimmune or malignant aetiologies, and all manifestations resolved with glucocorticoids. While we cannot prove causality, there was a temporal correlation between the vaccination and the clinical findings.

Neutrophilic Dermatosis of Hands as Oncological Finding: Importance of Follow-Up.

Neutrophilic febrile dermatosis (NFD) is a rare paraneoplastic syndrome that may be found in patients with head and neck cancer. NFD may appear before the neoplasia and may only concern the dorsal faces of the hands. This article reports the NFD findings of a patient with pharyngeal cancer, which was developed 2 years after the occurrence of NFD. The development of NFD in patient with alcohol and tobacco consumption should lead otolaryngologists and dermatologists to suspect head and neck malignancy. In cases of normal otolaryngological examination, patients have to be followed.

Sweet's syndrome triggered by ultraviolet light.

Sweet's syndrome is a neutrophilic dermatosis associated with many different underlying conditions but only rarely is it triggered by environmental factors such as ultraviolet (UV) exposure. We present two cases of photoinduced Sweet syndrome. Our first patient, who was taking hydrochlorothiazide, presented photodistributed lesions, pathological phototest and neutrophilic dermatosis histopathology. The phototest normalized after drug withdrawal, suggesting that both UV light and hydrochlorothiazide were necessary to cause the lesions. Our second case presented lesions clearly induced by UV light and histologically consistent with Sweet's syndrome. The MED was decreased and the lesions were reproduced with nbUVB, suggesting the diagnosis of photoinduced Sweet's syndrome. In conclusion, we report a case of neutrophilic dermatosis induced by hydrochlorothiazide and UV light and a case of photoinduced Sweet's syndrome with reproduction of the lesions after nbUVB. Both patients had a pathologic photobiological study. Our report emphasizes the need to perform phototests in patients with photodistributed Sweet's syndrome.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

SARS-CoV-2 vaccine induced Sweet syndrome : a case report.

Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis is a rare cutaneous disorder characterized by specific clinical, biological and microscopic findings. Although the exact cause of SS is still unknown, it may be triggered by infections, malignancies and drugs but also occurring after vaccinations such as bacille calmette guerin vaccination and influenza vaccine. While the recently discovered SARS COV2 vaccines are almost safe, many cutaneous and extracutaneous minor adverse effects are reported. We herein describe the fourth case of Sweet Syndrome induced by SARS-COV2 vaccine.

Sweet's syndrome associated with hematological malignancies.

BACKGROUND: Sweet's syndrome, or acute febrile neutrophilic dermatosis, is often mistaken for a skin infection given its similar clinical presentation. OBJECTIVE: To describe the clinical presentations and management of a rare dermatologic condition associated with hematological malignancies. METHODS: Case series; Chart review of patients at Moffitt Cancer Center between 2017 and 2020. RESULTS: The subjects are a 79 year-old man (Patient 1) with Myelodysplastic Syndrome (MDS), a 66 year-old woman (Patient 2) with Acute Myeloid Leukemia (AML), a 56 year-old man (Patient 3) with AML, and a 69 year-old man (Patient 4) with MDS. Patient 1 was initially misdiagnosed with neutropenic fever. Patient 2 was incidentally discovered to have erythematous skin lesions prior to initiating chemotherapy. Before starting second line chemotherapy, patient 3 developed pathergy at the site of a PICC line. Patient 4 developed erythema around a newly placed port before initiating chemotherapy. Only patients 1 and 3 received glucocorticoids. Patients 2, 3, and 4 were able to initiate chemotherapy without further complications. LIMITATIONS: Heterogeneity of subjects in terms of prognostic factors, stage at diagnosis, and treatment strategies. CONCLUSION: Early recognition and treatment of malignancy-associated Sweet's syndrome is imperative to limit patient morbidity and expeditiously provide anti-cancer treatments.