Raras
Buscar doenças, sintomas, genes...
Síndrome Sweet
ORPHA:3243CID-10 · L98.2CID-11 · EB20OMIM 608068DOENÇA RARA

A Síndrome de Sweet (também conhecida como dermatose neutrofílica febril aguda) é caracterizada por um conjunto de sintomas que a pessoa sente, sinais visíveis no corpo e alterações encontradas em exames de tecido. Ela inclui febre, um aumento de neutrófilos (que são um tipo de glóbulo branco de defesa) no sangue, e lesões na pele que são avermelhadas, sensíveis e dolorosas ao toque (como bolinhas pequenas e elevadas, caroços e manchas maiores e planas). Além disso, há um acúmulo de células de defesa, principalmente neutrófilos maduros, espalhado pela parte de cima da derme (uma das camadas da pele).

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Introdução

O que você precisa saber de cara

📋

A Síndrome de Sweet (também conhecida como dermatose neutrofílica febril aguda) é caracterizada por um conjunto de sintomas que a pessoa sente, sinais visíveis no corpo e alterações encontradas em exames de tecido. Ela inclui febre, um aumento de neutrófilos (que são um tipo de glóbulo branco de defesa) no sangue, e lesões na pele que são avermelhadas, sensíveis e dolorosas ao toque (como bolinhas pequenas e elevadas, caroços e manchas maiores e planas). Além disso, há um acúmulo de células de defesa, principalmente neutrófilos maduros, espalhado pela parte de cima da derme (uma das camadas da pele).

Pesquisas ativas
1 ensaio
2 total registrados no ClinicalTrials.gov
Publicações científicas
964 artigos
Último publicado: 2026 Mar

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Adult
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: L98.2
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
6 sintomas
🧬
Pele e cabelo
3 sintomas
😀
Face
1 sintomas
❤️
Coração
1 sintomas
💪
Músculos
1 sintomas
🫃
Digestivo
1 sintomas

+ 30 sintomas em outras categorias

Características mais comuns

100%prev.
Artralgia
Frequente (79-30%)
100%prev.
Mialgia
Frequente (79-30%)
100%prev.
Acne cística
Frequência: 3/3
90%prev.
Nódulo cutâneo
Muito frequente (99-80%)
90%prev.
Placa eritematosa
Muito frequente (99-80%)
90%prev.
Dor
Muito frequente (99-80%)
44sintomas
Muito frequente (12)
Frequente (16)
Ocasional (11)
Muito raro (2)
Sem dados (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 44 características clínicas mais associadas, ordenadas por frequência.

ArtralgiaArthralgia
Frequente (79-30%)100%
MialgiaMyalgia
Frequente (79-30%)100%
Acne císticaCystic acne
Frequência: 3/3100%
Nódulo cutâneoSkin nodule
Muito frequente (99-80%)90%
Placa eritematosaErythematous plaque
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico964PubMed
Últimos 10 anos200publicações
Pico202496 papers
Linha do tempo
2026Hoje · 2026🧪 2004Primeiro ensaio clínico📈 2024Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

2 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.

PTPN6Tyrosine-protein phosphatase non-receptor type 6Major susceptibility factor inAltamente restrito
FUNÇÃO

Tyrosine phosphatase enzyme that plays important roles in controlling immune signaling pathways and fundamental physiological processes such as hematopoiesis (PubMed:14739280, PubMed:29925997). Dephosphorylates and negatively regulate several receptor tyrosine kinases (RTKs) such as EGFR, PDGFR and FGFR, thereby modulating their signaling activities (PubMed:21258366, PubMed:9733788). When recruited to immunoreceptor tyrosine-based inhibitory motif (ITIM)-containing receptors such as immunoglobul

LOCALIZAÇÃO

CytoplasmNucleus

VIAS BIOLÓGICAS (10)
GPVI-mediated activation cascadeInterferon alpha/beta signalingSARS-CoV-2 activates/modulates innate and adaptive immune responsesCo-inhibition by PD-1Co-inhibition by BTLA
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
442.6 TPM
Sangue
278.5 TPM
Baço
267.0 TPM
Pulmão
74.1 TPM
Intestino delgado
57.8 TPM
OUTRAS DOENÇAS (5)
bullous pyoderma gangrenosumvegetative pyoderma gangrenosumpustular pyoderma gangrenosumclassic pyoderma gangrenosum
HGNC:9658UniProt:P29350
MEFVPyrinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Involved in the regulation of innate immunity and the inflammatory response in response to IFNG/IFN-gamma (PubMed:10807793, PubMed:11468188, PubMed:16037825, PubMed:16785446, PubMed:17431422, PubMed:17964261, PubMed:18577712, PubMed:19109554, PubMed:19584923, PubMed:26347139, PubMed:27030597, PubMed:28835462). Organizes autophagic machinery by serving as a platform for the assembly of ULK1, Beclin 1/BECN1, ATG16L1, and ATG8 family members and recognizes specific autophagy targets, thus coordinat

LOCALIZAÇÃO

Cytoplasm, cytoskeletonCell projection, ruffleCell projection, lamellipodiumNucleusCytoplasmCytoplasmic vesicle, autophagosome

VIAS BIOLÓGICAS (2)
Purinergic signaling in leishmaniasis infectionThe NLRP3 inflammasome
MECANISMO DE DOENÇA

Familial Mediterranean fever, autosomal recessive

A hereditary periodic fever syndrome characterized by recurrent episodic fever, serosal inflammation and pain in the abdomen, chest or joints. It is frequently complicated by reactive amyloidosis, which leads to renal failure and can be prophylactically treated with colchicine.

EXPRESSÃO TECIDUAL(Tecido-específico)
Sangue
81.4 TPM
Baço
19.9 TPM
Pulmão
10.6 TPM
Testículo
4.1 TPM
Adipose Visceral Omentum
1.9 TPM
OUTRAS DOENÇAS (6)
autosomal recessive familial Mediterranean feversweet syndromefamilial Mediterranean fever, autosomal dominantBehcet disease
HGNC:6998UniProt:O15553

Variantes genéticas (ClinVar)

270 variantes patogênicas registradas no ClinVar.

🧬 MEFV: NM_000243.3(MEFV):c.1777G>A (p.Ala593Thr) ()
🧬 MEFV: NM_000243.3(MEFV):c.2176G>A (p.Val726Ile) ()
🧬 MEFV: NM_000243.3(MEFV):c.1006G>T (p.Glu336Ter) ()
🧬 MEFV: NM_000243.3(MEFV):c.1067A>G (p.Gln356Arg) ()
🧬 MEFV: NM_000243.3(MEFV):c.1198C>G (p.Leu400Val) ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
·Pré-clínico2
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Sweet

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

2 ensaios clínicos encontrados, 1 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
619 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 619

#1

Extraintestinal Cutaneous Manifestations in Inflammatory Bowel Disease Among Non-White Patients: A Retrospective Multicenter Study.

Journal of clinical gastroenterology2026 Jan 20

The presence of extraintestinal manifestations with cutaneous diseases in patients with inflammatory bowel disease (IBD) can pose significant complications. Though the prevalence of IBD has been increasing in racial and ethnic minority groups, most literature has characterized several cutaneous manifestations (CM) of IBD in patients of white skin, with a lack of studies describing these complications in patients with other skin tones. Our study aimed to determine the rates of various CM of IBD in skin of color using a health care database to identify white and non-white patients with a diagnosis of IBD who were prescribed at least one IBD-specific medication or advanced therapy. Of the total IBD patients, after propensity score matching there were 35,624 patients identified in both the white and non-white cohorts. Among non-white patients, there was a >2-fold odds of developing hidradenitis suppurativa and increased odds of vitiligo. Psoriasis, herpes zoster, and leukocytoclastic vasculitis exhibited a decreased association in non-white patients. There was no difference in erythema nodosum, pyoderma gangrenosum, oral aphthae, Sweet syndrome, and acquired epidermolysis bullosa. With the rise of IBD in non-white populations, the representation of CM of IBD in this population is significantly limited, underrecognized, and thus undertreated. Our results reveal increased prevalence of several CM in the non-white IBD patient population. Recognition of CM in non-white patients with IBD can enhance quality of life and reduce morbidity among this population.

#2

Clinicopathologic Spectrum of Dermatological Diseases in Patients with Acute Myeloid Leukemia (AML): A Retrospective Study in AML Patients with Cutaneous Manifestations.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion2026 Jan

Leukemia is one of the most common hematological malignancies. Cutaneous manifestations of leukemias consist of two groups: specific and non-specific. While the drug reactions, opportunistic infections due to myelosuppression are non-specific skin findings of leukemias, leukemia cutis is a specific sign of cutaneous involvement. In this retrospective study, we included patients over the age of 18 who were diagnosed with acute myeloid leukemia (AML) and underwent histopathological examination due to dermatological complaints. A total of 21 patients were included. Histopathological examination results were consistent with myeloid sarcoma, erythema nodosum, cutaneous drug eruption, necrosis due to vascular damage, cutaneous vasculitis, graft versus host disease, Sweet syndrome and viral infection. Dermatological examination plays an important role in AML patients. Skin biopsy and immunohistochemical examination should be performed to make early diagnosis of skin metastasis of leukemias and paraneoplastic syndromes to reduce the mortality and morbidity in AML patients.

#3

Myeloid dermatosis with features of sweet syndrome and leukemia cutis: a case report.

Annals of hematology2026 Mar 23

Occult hematologic malignancies can initially present with cutaneous manifestations. We describe a 53-year-old woman with chronic hepatitis C and intravenous drug use presenting with a painful, progressive leg rash and worsening bicytopenias. A skin punch biopsy revealed neutrophilic dermatosis morphologically compatible with Sweet syndrome (SS), along with rare CD117+/CD34 − blastoid cells. Subsequent bone marrow studies revealed acute myeloid leukemia with mutated NPM1. This case highlights the diagnostic challenge of distinguishing SS from leukemia cutis (LC) when skin biopsies demonstrate mixed neutrophilic with rare blastoid cells. LC represents cutaneous infiltration of leukemic blasts, whereas SS represents a non-neoplastic, cytokine-driven process. Prompt immunophenotypic evaluation and genomic testing of myeloid dermatoses, particularly those with blastoid cells, can be critical in distinguishing SS from LC to provide significant insight on prognosis and therapeutic options. This case highlights the clinical and histopathologic spectrum of myeloid dermatoses and further emphasizes the relevance of skin biopsies in the diagnosis of cutaneous involvement by hematologic malignancies, such as myeloid neoplasms.

#4

Wrong Place at the Right Height: Scalp Orf in a Toddler With Dense Dermal Neutrophilic Infiltrate Mimicking Sweet Syndrome.

Journal of cutaneous pathology2026 Mar 08

We report a case of a 3-year-old male who presented with fever, posterior cervical lymphadenopathy, and a tender vesiculobullous lesion on the scalp following contact with goats 2 weeks prior. Histopathology revealed epidermal necrosis, massive papillary dermal edema, brisk mixed inflammation with abundant neutrophils, and inclusion bodies in the epidermis. These findings led to a diagnosis of orf virus infection, otherwise known as ecthyma contagiosum, which is a parapox virus acquired from goats and sheep. An abundance of neutrophils is not a feature classically thought to be associated with orf virus. Additionally, orf typically affects the hands following direct contact with infected animals; the scalp is an unusual site of infection. We suspect infection occurred as this child's scalp when standing was roughly the same height as a goat's mouth, and goats have a predilection to chew at hair. Unfortunately, despite extensive attempts, we were unable to find any laboratory that offers molecular testing for orf on formalin-fixed paraffin-embedded tissue samples. Because of the lack of readily available molecular testing for this infection, we seek to highlight the importance of considering orf in the differential diagnosis of vesicular lesions and obtaining animal exposure history to aid in this diagnosis.

#5

From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events.

Seminars in arthritis and rheumatism2026 Apr

Granulocyte colony-stimulating factor (G-CSF) and its PEGylated formulation (pegfilgrastim) are indispensable for preventing chemotherapy-induced neutropenia and for stem-cell mobilization. Beyond hematopoiesis, G-CSF modulates innate/adaptive immunity and may precipitate immune-mediated events in susceptible hosts. Clinical signals span large-vessel vasculitis/aortitis and non-vascular neutrophil-dominant phenotypes, yet timing, spectrum, and management remain variably characterized. To identify and synthesize primary clinical reports of autoimmune/rheumatic disease triggered or exacerbated by exogenous G-CSF (filgrastim or pegfilgrastim), describing phenotypes, latency, management, outcomes, and implications for practice. We performed a narrative review of primary clinical studies, including observational cohorts and case reports or short case series, identified through targeted searches of PubMed/MEDLINE, Scopus, and Embase, complemented by investigator-curated references. Eligible reports described autoimmune, inflammatory, or rheumatic manifestations occurring after exposure to exogenous G-CSF. Predefined phenotypes included large-vessel vasculitis/aortitis; neutrophilic dermatoses (e.g., Sweet's syndrome, neutrophilic dermatosis of the dorsal hands); cutaneous small-vessel vasculitis; inflammatory or crystal arthritis (e.g., calcium pyrophosphate deposition disease); pulmonary inflammatory injury (e.g., diffuse alveolar hemorrhage); and clear flares of established autoimmune disease. Narrative or mechanistic reviews and trials of GM-CSF pathway blockade were excluded from qualitative synthesis. Twenty-four studies met inclusion criteria: two observational cohorts and twenty-two single-patient reports/short series. Cohorts quantified a low but reproducible burden of pegfilgrastim-associated aortitis, with stereotyped involvement of the aortic arch/proximal branches and typical latency of 7-15 days. Case-level data confirmed marked elevation of acute phase reactants, usually negative autoantibodies, favorable outcomes after G-CSF withdrawal with or without short glucocorticoid courses, and recurrence/migration on re-exposure. Non-vascular events clustered earlier (2-7 days) and included biopsy-proven Sweet's/NDDH, leukocytoclastic vasculitis, CPPD flares, and rare diffuse alveolar hemorrhage; granulomatous dermatitis and perioperative pyoderma gangrenosum were also observed. Re-exposure information suggested phenotype-specific risk: recurrent CPPD with pegfilgrastim was mitigated by switching to short-acting filgrastim; selected limited aortitis resolved without steroids; refractory aortitis responded to IL-6 blockade, enabling uninterrupted chemotherapy. Exogenous G-CSF can precipitate a coherent spectrum of immune-mediated toxicity with distinct, clinically actionable timing windows: vascular events peak 7-15 days after pegfilgrastim, whereas cutaneous, articular, and pulmonary manifestations arise within 2-7 days. Recognition hinges on temporal linkage, imaging/histology, and exclusion of mimics. Management is phenotype-aware-drug withdrawal ± short glucocorticoids, formulation switching for CPPD, and targeted IL-6 blockade in selected refractory aortitis-allowing preservation of hematologic benefits while minimizing immune toxicity. Prospective surveillance and structured re-exposure studies (including formulation tailoring) are priorities to move from signal detection to prevention.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC688 artigos no totalmostrando 196

2026

Myeloid dermatosis with features of sweet syndrome and leukemia cutis: a case report.

Annals of hematology
2026

Sweet Syndrome in a Patient With Adult-Onset Immunodeficiency Due to Anti-Interferon-Gamma Autoantibodies: A Case Report and Brief Review of the Literature.

The American Journal of dermatopathology
2026

Acute bullous iododerma with cryptococcoid neutrophilic dermatosis: A case series.

JAAD case reports
2026

Wrong Place at the Right Height: Scalp Orf in a Toddler With Dense Dermal Neutrophilic Infiltrate Mimicking Sweet Syndrome.

Journal of cutaneous pathology
2026

Monoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.

Journal of the American Academy of Dermatology
2026

From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events.

Seminars in arthritis and rheumatism
2026

Beyond Sweet Syndrome: Untangling the Threads of Histiocytoid Sweet Syndrome and Cutaneous Myeloid Neoplasia.

Surgical pathology clinics
2025

Sweet syndrome presenting with pustular lesions and pulmonary involvement: a case report.

European journal of dermatology : EJD
2026

The Case | Rash, eosinophilia, adrenal mass, and acute kidney injury in a febrile patient.

Kidney international
2026

Extraintestinal Cutaneous Manifestations in Inflammatory Bowel Disease Among Non-White Patients: A Retrospective Multicenter Study.

Journal of clinical gastroenterology
2026

Periocular necrotizing sweet syndrome - a case series and review of literature.

Orbit (Amsterdam, Netherlands)
2026

Clinicopathologic Spectrum of Dermatological Diseases in Patients with Acute Myeloid Leukemia (AML): A Retrospective Study in AML Patients with Cutaneous Manifestations.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2025

Periorbital Necrotizing Sweet Syndrome: A Case Report and Literature Review.

Ophthalmic plastic and reconstructive surgery
2026

Concurrent paraneoplastic dermatomyositis and Sweet syndrome.

JAAD case reports
2025

A Rare Case of Metastatic Urethral Squamous Cell Carcinoma Presenting with Paraneoplastic Sweet Syndrome and Treated with Pembrolizumab.

Current oncology (Toronto, Ont.)
2026

Cryptococcid Sweet Syndrome in the Setting of Hydralazine-Induced ANCA Vasculitis: A Case Report.

Journal of cutaneous pathology
2025

A Case of Cutaneous Dirt-Adherent-Like Sweet Syndrome in Pregnancy.

International journal of women's health
2025

Disseminated Talaromyces marneffei infection mimicking tuberculosis in an HIV-negative adult with anti-IFN-γ autoantibodies: a case report.

BMC infectious diseases
2025

A Case of Sweet Syndrome Complicating Ulcerative Colitis Treated With Upadacitinib.

ACG case reports journal
2025

Rifampicin-Associated Sweet Syndrome: An Uncommon Adverse Event of Anti-Tuberculosis Therapy.

Respirology case reports
2026

Cutting-edge multiomic approaches shed light on the interleukin-36 axis in Sweet syndrome.

The British journal of dermatology
2025

Upper Respiratory Tract Infection Leading to a New Diagnosis of Sweet Syndrome and Monoclonal Gammopathy of Unknown Significance.

HCA healthcare journal of medicine
2025

A case of giant cellulitis-like Sweet syndrome and review.

JAAD case reports
2025

TNF Inhibitor Therapy in Corticosteroid-Resistant or -Dependent Pediatric Neutrophilic Dermatosis.

Pediatric dermatology
2026

Interleukin-36 as the central amplifier of neutrophilic inflammation: lessons from spatiotemporal transcriptomics in Sweet syndrome.

The British journal of dermatology
2025

Acute febrile neutrophilic dermatosis in the setting of pembrolizumab in a patient with nonsmall cell lung cancer.

Dermatology online journal
2025

Sweet syndrome successfully treated with upadacitinib.

JAAD case reports
2026

Cutaneous Manifestations and Dermatologic Adverse Events in IBD: A Clinical Update.

Inflammatory bowel diseases
2026

Spatiotemporal transcriptomics deciphered neutrophil-activated interleukin-36 as driver and effective therapeutic target in Sweet syndrome.

The British journal of dermatology
2025

Histiocytoid Sweet Syndrome - A systematic literature review of the clinical and histological characteristics and treatment outcomes.

Journal of the European Academy of Dermatology and Venereology : JEADV
2025

Keratinocyte-neutrophil interactions revealed as targetable drivers of sustained inflammation in Sweet syndrome.

The Journal of clinical investigation
2025

Leukoplakia: An uncommon presentation of acute febrile neutrophilic dermatosis (Sweet syndrome).

JAAD case reports
2025

Cryptococcoid Sweet syndrome or halogenoderma? Highlighting the crucial need for serum iodine testing.

JAAD case reports
2025

Drug-induced Sweet's syndrome: pharmacovigilance insights from FAERS with a cross-database consistency assessment in VigiBase via LASSO and multivariable logistic regression.

Frontiers in immunology
2025

Histiocytoid Sweet syndrome-like presentation of mature plasmacytoid dendritic cell proliferation.

Dermatology online journal
2025

A Case Report of Sweet Syndrome and Hemorrhagic Bullae Associated With Acute Myeloid Leukemia With Myelodysplasia-Related Changes.

Cureus
2025

Recurrent Annular Erythema as an Initial Presentation of Pediatric Histiocytoid Sweet Syndrome Associated With Myelodysplastic Syndrome: A Case Report.

The Journal of dermatology
2025

Sweet Syndrome-like Dermatosis as a Precursor to Overlapping Hematologic Malignancies: A Case Report and Review.

Journal of clinical medicine
2025

Epidemiology, Clinical Data, and Management of Aseptic Abscess Syndrome: Review of Published Cases Outside France.

Epidemiologia (Basel, Switzerland)
2025

Bleomycin-induced Sweet's syndrome.

Indian journal of pharmacology
2025

SAA1/FPR2 signaling between keratinocytes and neutrophils sustains chronic inflammation in Sweet syndrome.

The Journal of clinical investigation
2025

Sweet Syndrome in Patients With Hidradenitis Suppurativa: A Population Study in the TriNetX Research Database.

International journal of dermatology
2025

Cardiovascular involvement in Sweet's syndrome: A practical review.

Caspian journal of internal medicine
2025

Complex Interplay Between Sweet Syndrome and Therapy-Related Myelodysplastic Syndrome After B-Cell Lymphoma Treatment: A Case Report.

The American journal of case reports
2025

Nontuberculous scrofuloderma with sweet syndrome leading to anti-interferon-γ autoantibody-associated adult-onset immunodeficiency: A case report.

JAAD case reports
2025

Cryptococcoid Sweet Syndrome Following Gastrointestinal Neuroendocrine Tumor Removal: A Case Report.

Cureus
2025

Subcutaneous panniculitis-like T-cell lymphoma in pregnancy.

BMJ case reports
2025

Sweet Syndrome and Valley Fever: A Case Report.

Journal of the American College of Emergency Physicians open
2025

An Acute Onset Sweet Syndrome After First Vedolizumab Dose in Crohn's Disease.

Inflammatory bowel diseases
2025

Unveiling the bridge between neutrophilic and autoinflammatory spectrum. Good response to old but gold colchicine in a complex clinical association of Sweet Syndrome with psoriasis and hidradenitis suppurativa under treatment with ixekizumab.

Italian journal of dermatology and venereology
2025

Positionally distinct interferon-stimulated dermal immune-acting fibroblasts promote neutrophil recruitment in Sweet syndrome.

The Journal of allergy and clinical immunology
2025

Diffuse necrotizing pneumonia, cavitations and hemoptysis: A case of sweet syndrome.

Respiratory medicine case reports
2025

Sweet's syndrome as a presenting symptom of granulomatosis with polyangiitis.

BMJ case reports
2025

Rickettsia parkeri Rickettsiosis Resembling Sweet Syndrome: A Differential Diagnosis for Critical Discussion.

Infectious disease reports
2025

First Description of Simultaneous Sweet Syndrome and Tracheal Stenosis as Extraintestinal Manifestations in a Patient With Highly Active Ulcerative Colitis.

ACG case reports journal
2025

Bilateral Scleritis and Neutrophilic Dermatosis With Cytogenetic Chromosomal Aberrancy Related to Pyoderma Gangrenosum: A Case Report of a 20-Year Follow-Up.

Cureus
2025

Tigecycline Induced Sweet Syndrome in a Patient on Maintenance Hemodialysis.

Hemodialysis international. International Symposium on Home Hemodialysis
2025

Neutrophilic Urticarial Dermatosis: A Window into Systemic Inflammation and Autoimmune Disorders.

Clinical reviews in allergy &amp; immunology
2025

An Atypical Presentation of VEXAS Syndrome: A Diagnostic Conundrum.

The Ulster medical journal
2025

A Not-So-Sweet Crohn's Disease: A Case Report of Ileocecal Crohn's Disease Unmasked by Sweet Syndrome.

Case reports in medicine
2025

Histiocytoid Sweet Syndrome With Paradoxical Neutropenia in a Pediatric Patient.

Pediatric dermatology
2025

Pyoderma gangrenosum: a 22-year follow-up of patients in a tertiary reference hospital in Brazil.

Anais brasileiros de dermatologia
2025

Giant cellulitis-like Sweet syndrome with myofasciitis: Multimodality imaging findings and streptococcal trigger.

Radiology case reports
2025

[Classical Sweet syndrome with multiple organ lesions by 18F-FDG PET/CT: A case report].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2025

Histiocytoid Sweet Syndrome as a Manifestation of Myelodysplasia Cutis/Cutaneous VEXAS Syndrome.

The American Journal of dermatopathology
2025

Very rare adverse reactions to intravascular contrast media: From Kounis to sweet syndrome.

European journal of radiology
2025

Acute myeloid leukemia with DEK::NUP214 fusion resembling acute promyelocytic leukemia, initially presenting as sweet syndrome: A case report and literature review.

The Journal of international medical research
2025

Neuro-Sweet's disease manifesting as limbic encephalitis and ventriculitis.

Clinical neurology and neurosurgery
2025

SITRAME and Giant Cellulitis-Like Sweet Syndrome: A Spectrum of Autoinflammatory Dermatoses?

International journal of dermatology
2025

Reflectance Confocal Microscopy of Sweet Syndrome.

Dermatology practical &amp; conceptual
2025

An Atypical Presentation of Sweet's Syndrome in a Patient With Neutropenia: A Case Report.

Cureus
2025

Inflammatory Signatures in VEXAS Syndrome, Myelodysplasia Cutis, and Sweet Syndrome.

JAMA dermatology
2025

Case Report: First report of Legionella micdadei pneumonia and organizing pneumonia in a patient with myelodysplastic and Sweet syndromes.

Frontiers in immunology
2024

Neutrophilic Dermatosis of the Dorsal Hands.

Journal of Brown hospital medicine
2025

Sweet Syndrome With Painless Skin Lesions and Myopericarditis: A Case Report and Literature Review.

Clinical case reports
2025

A Case of Sweet's Syndrome Presenting With Tongue Ulcerations.

The Australasian journal of dermatology
2025

Cutaneous Hemophagocytosis in a Sweet Syndrome-An Unusual Phenomenon in a Rare Guise.

The American Journal of dermatopathology
2025

Rare skin manifestation of tularemia: Sweet syndrome.

Revista da Sociedade Brasileira de Medicina Tropical
2025

Sweet Syndrome: A Retrospective Analysis of 21 Patients.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2025

Wound care of Sweet syndrome in a patient with anaplastic lymphoma kinase-positive anaplastic large cell lymphoma: a case report.

Journal of wound care
2024

Cutaneous Adverse Drug Reactions Associated with SGLT2 Inhibitors.

Journal of clinical medicine
2024

Clinical Variant of Serpentine Supravenous Hyperpigmentation Following Subcutaneous Bortezomib Injection.

HCA healthcare journal of medicine
2025

Hydroxychloroquine-induced Sweet's Syndrome: A Case Report and Literature Review.

Acta dermato-venereologica
2025

Conceptual Contextualization of Neutrophilic Dermatoses.

The American Journal of dermatopathology
2025

Orbital apex syndrome secondary to Sweet syndrome.

BMJ case reports
2024

Sweet Syndrome-Like Disorder Induced by the Oxford-AstraZeneca® SARS-CoV-2 Vaccine.

Skinmed
2024

Molecular Characteristics of Sweet Syndrome: A Systematic Review.

Experimental dermatology
2024

Decitabine-induced Sweet syndrome.

BMJ case reports
2024

Sweet Syndrome Mimicking Flagellate Dermatitis.

Indian journal of dermatology
2025

Atypical Type 2 Lepra Reaction Masquerading as Recurrent Sweet Syndrome.

Journal of cutaneous pathology
2025

Neurological Sweet's syndrome: a rare but treatable manifestation of an autoinflammatory disease.

Practical neurology
2025

VEXAS Syndrome: Histiocytoid Cells With Feathery Cytoplasm as a Clue to the Diagnosis.

Journal of cutaneous pathology
2024

Sweet syndrome in patients with and without malignancy: a retrospective study of 66 cases from a tertiary care centre.

European journal of dermatology : EJD
2025

A unique case of steroid-resistant, giant cellulitis-like Sweet syndrome mimicking alpha-1-antitrypsin deficiency-associated panniculitis: successful treatment with dapsone.

Clinical and experimental dermatology
2024

Sweet syndrome: an update on clinical aspects, pathophysiology, and treatment.

Italian journal of dermatology and venereology
2025

Sweet syndrome with multiorgan involvement exacerbated by gilteritinib.

Leukemia &amp; lymphoma
2025

Spesolimab Response in a Patient With Steroid-Resistant Sweet Syndrome.

JAMA dermatology
2025

Mimickers of erysipelas and cellulitis: A narrative review.

Indian journal of dermatology, venereology and leprology
2024

Sweet's Syndrome Unveiling a Primary Cervical Malignancy: A Case Report.

Cureus
2024

[Translated article] Giant Cellulitis-like Sweet Syndrome: Contribution of a New Case and Medical Literature Review.

Actas dermo-sifiliograficas
2024

[Canine sterile neutrophilic dermatosis (Sweet-like syndrome): A description of 3 cases].

Tierarztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
2024

Cryptococcoid Sweet syndrome: a case report.

Frontiers in medicine
2024

Drug-induced acute febrile neutrophilic dermatosis (Sweet syndrome): A case report presented at Delhi State Cancer Institute.

Journal of cancer research and therapeutics
2025

Subcutaneous Sweet Syndrome With Vasculitis Features: Case Report and Review.

The American Journal of dermatopathology
2024

Clinical features, treatment, and outcome of granulocyte colony stimulating factor-induced sweet syndrome.

Archives of dermatological research
2024

Giant cellulitis-like Sweet syndrome mimicking cellulitis: a case report.

Journal of medical case reports
2024

Sweet Syndrome Presenting with Features of Cellulitis Shortly after Femoral Angioplasty.

European journal of case reports in internal medicine
2024

Successful treatment of Sweet syndrome complicated by Behcet disease with adalimumab.

Pediatrics international : official journal of the Japan Pediatric Society
2024

Longitudinal Presentation and Management of Neutrophilic Dermatosis of the Dorsal Hand: A Case Report Over Six Visits.

Cureus
2024

Disseminated Yersinia enterocolitica infection associated to Sweet's syndrome.

BMC infectious diseases
2024

Vulvar ulcers as a rare manifestation of hematologic malignancy.

JAAD case reports
2024

Sweet syndrome after vaccination.

Medicina clinica
2024

A Mysterious Rash Following Cardiothoracic Surgery: A Not So Sweet Ending.

Cureus
2024

Sweet Syndrome in the Central Nervous System: A Case of Orbital Inflammation, Sixth Nerve Palsy, and Pachymeningitis.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2024

Aseptic Splenic Abscesses With Concomitant Sweet Syndrome as Extraintestinal Manifestations of New-Onset Crohn's Disease.

ACG case reports journal
2024

Crusted plaques on the face.

International journal of dermatology
2024

Pyoderma Gangrenosum: A Presenting Feature of Rheumatoid Arthritis.

Cureus
2024

Tocilizumab-Induced Sweet Syndrome in a Familial Mediterranean Fever Patient: A Case Report.

Dermatology practical &amp; conceptual
2024

Neutrophilic Dermatosis: A Variant of Sweet Syndrome.

Journal of cutaneous medicine and surgery
2024

Mucocutaneous manifestations of inflammatory bowel disease.

Annales de dermatologie et de venereologie
2024

Histiocytoid Pyoderma Gangrenosum: A Challenging Case With Features of Sweet Syndrome.

Cutis
2024

Sweet syndrome associated with moderate leukocyte adhesion deficiency type I: a case report and review of the literature.

Frontiers in immunology
2024

Pyrin-associated autoinflammation with neutrophilic dermatosis: A case report.

The Journal of dermatology
2024

Case 22-2024: A 30-Year-Old Woman with Postpartum Fever, Abdominal Pain, and Skin Ulcers.

The New England journal of medicine
2024

A case of Sweet's syndrome successfully treated with minocycline.

European journal of dermatology : EJD
2024

Myelodysplasia cutis and VEXAS syndrome initially diagnosed as histiocytoid Sweet syndrome: A diagnostic pitfall.

Journal of cutaneous pathology
2024

VEXAS syndrome: A 2-case series report.

Reumatologia clinica
2024

Neonatal Sweet syndrome with associated rectovestibular fistula and review of the literature.

Pediatric dermatology
2024

Sweet syndrome in pregnancy: A narrative review.

International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics
2024

Skin Manifestations of VEXAS Syndrome and Associated Genotypes.

JAMA dermatology
2024

One case of Sweet Syndrome after treatment of MDS-RS-MLD with azactidine and literature review.

Minerva medica
2024

Bowel-associated dermatosis-arthritis syndrome, a diagnostic challenge in a young child with very early-onset inflammatory bowel disease.

Pediatric dermatology
2024

A Rare Case of Drug-Induced Sweet Syndrome After Pembrolizumab Therapy.

Cureus
2024

Neutrophilic dermatosis of the dorsal hands in a Mexican woman.

Anais brasileiros de dermatologia
2024

Single-lesion sporotrichosis triggering Sweet's syndrome.

Anais brasileiros de dermatologia
2024

Drug-Induced and Malignancy-Associated Neutrophilic Dermatoses in Patients with Hematologic Malignancies: A Single Institution Experience.

Dermatology (Basel, Switzerland)
2024

Histiocytoid Sweet's Syndrome in the Setting of Myelodysplastic Syndrome.

Cureus
2024

Reflectance confocal microscopy in pediatric patients with Sweet's syndrome: a useful method to differentiate from discoid lupus erythematosus.

Archives of dermatological research
2024

Brodalumab for the treatment of refractory Sweet syndrome.

International journal of dermatology
2024

Sweet Syndrome as a Herpetiform Mimic: A Diagnostic Challenge.

The American Journal of dermatopathology
2024

Suspected coexistence of perianal necrotizing sweet syndrome in chronic myelomonocytic leukemia: A case report.

World journal of gastrointestinal surgery
2024

Noninflammatory extrafacial edema as a clue to the diagnosis of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome.

JAAD case reports
2024

Sweet syndrome with peripheral neuropathy in a patient with metastatic clear cell renal cell carcinoma.

BMJ case reports
2024

Sweet syndrome and acute pulmonary coccidioidomycosis in West Texas.

JAAD case reports
2024

Sweet syndrome and secondary syphilis: a rare association that should not be missed.

Italian journal of dermatology and venereology
2024

To ASLO or Not to ASLO: Utility of the ASLO Test in Dermatology.

The Israel Medical Association journal : IMAJ
2024

An atypical facial eruption in skin of color: A rare presentation of histiocytoid Sweet syndrome.

JAAD case reports
2024

Sweet syndrome induced by FLT3 inhibitors: case report and literature review.

Hematology (Amsterdam, Netherlands)
2024

Sweet or Not? Azathioprine-Induced Sweet Syndrome Mimicking Erythema Nodosum in a Patient With Inflammatory Bowel Disease.

ACG case reports journal
2024

Proteasome inhibitor-associated histiocytoid Sweet's syndrome: Clinical and histological similarities to Nakajo-Nishimura syndrome suggest a potential mechanism.

The Journal of dermatology
2024

COVID-19 vaccination-induced Sweet syndrome in a patient with squamous cell carcinoma treated with nivolumab.

European journal of dermatology : EJD
2024

Description of a novel splice site variant in UBA1 gene causing VEXAS syndrome.

Rheumatology (Oxford, England)
2024

Comparative immunohistochemical analysis of inflammatory cytokines in distinct subtypes of Sweet syndrome.

Frontiers in immunology
2024

Periorbital necrotizing sweet syndrome: A report of two cases mimicking necrotizing soft tissue infections.

American journal of ophthalmology case reports
2023

A case of filgrastim-induced neutrophilic dermatosis of the dorsal hands in a patient with Felty Syndrome.

Dermatology online journal
2024

Pulmonary coccidioidomycosis mimicking malignancy associated with Sweet's syndrome (acute febrile neutrophilic dermatosis).

Clinical case reports
2023

Neutrophilic Dermatosis of the Hands: A Case Report.

Acta dermatovenerologica Croatica : ADC
2024

Sweet syndrome in a patient receiving encorafenib and binimetinib therapy for malignant melanoma.

JAAD case reports
2024

Neutrophilic Dermatoses: A Medley of Inflammatory Cutaneous and Systemic Disorders.

Dermatologic clinics
2024

Quality of Life with Neutrophilic Dermatoses.

Dermatologic clinics
2024

Neutrophilic Panniculitides.

Dermatologic clinics
2024

Pediatric Neutrophilic Dermatoses.

Dermatologic clinics
2024

Neutrophilic Urticarial Dermatosis.

Dermatologic clinics
2024

From Histiocytoid Sweet Syndrome to Myelodysplasia Cutis: History and Perspectives.

Dermatologic clinics
2024

Sweet Syndrome and Neutrophilic Dermatosis of the Dorsal Hands.

Dermatologic clinics
2024

Overview of Neutrophilic Biology, Pathophysiology, and Classification of Neutrophilic Dermatoses.

Dermatologic clinics
2024

The Neutrophilic Dermatoses, or the Cutaneous Expressions of Neutrophilic Inflammation.

Dermatologic clinics
2024

Mercaptopurine-induced Sweet's syndrome.

BMJ case reports
2024

Neutrophilic dermatosis in a patient with an IKZF1 variant and a review of monogenic autoinflammatory disorders presenting with neutrophilic dermatoses.

Pediatric dermatology
2024

Cryptococcoid Sweet syndrome: A case report and literature review.

International journal of dermatology
2024

Clinical and histological features of histiocytoid Sweet syndrome associated with VEXAS syndrome.

Clinical and experimental dermatology
2024

Case Report: Type 2 Leprosy Reaction Mimicking Sweet Syndrome: A Case Report and Literature Review.

The American journal of tropical medicine and hygiene
2024

Paradoxical skin reaction to certolizumab, an overlap of neutrophilic dermatoses.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Multiple Abscess Collections: Antibiotics or Steroids?

Case reports in immunology
2023

Rheumatoid-associated chronic recurrent annular neutrophilic dermatosis.

European journal of dermatology : EJD
2024

Sweet syndrome in a patient with rectal adenocarcinoma and HIV following neoadjuvant chemoradiation.

JAAD case reports
2024

[Translated article] Patient With Relapsing Polychondritis and Cutaneous Nodules Being Followed by the Hematology Department for a Myelodysplastic Syndrome.

Actas dermo-sifiliograficas
2024

Sweet syndrome following the ChAdOx1-S vaccine.

BMJ case reports
2024

Neutrophil extracellular traps and neutrophilic dermatosis: an update review.

Cell death discovery
2024

Preface to Journal of Dermatology special issue: Neutrophilic dermatoses and autoinflammation.

The Journal of dermatology
2024

Concurrent Sweet syndrome and erythema nodosum.

Proceedings (Baylor University. Medical Center)
2024

VEXAS syndrome: Clinical manifestations, diagnosis, and treatment.

Reumatologia clinica
2024

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

American journal of hematology
2024

Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.

Rheumatology international
2023

Sweet Syndrome: Clinical Presentation, Malignancy Association, Autoinflammatory Disorders and Treatment Response in a Cohort of 93 Patients with Long-term Follow-up.

Acta dermato-venereologica
2024

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

European journal of haematology
2023

A Not-So-Sweet Syndrome: A Case Report of a Male Presenting With Acute Febrile Neutrophilic Dermatosis.

Cureus
2023

Neutrophilic Dermatosis of the Dorsal Hand: A Distinctive Variant of Sweet Syndrome.

Cutis
2023

Progressive, edematous plaques, mild pancytopenia, and inflammation.

JAAD case reports
2023

Sweet Syndrome With Vasculitis: Time To Adopt a New Criteria?

Cureus
2023

Neutrophilic Dermatosis of Dorsal Hands as Paradoxical Reaction to Infliximab Treatment.

Skinmed
2023

An uncommon culprit of neutropenic fever: a case of Sweet syndrome following induction therapy for acute myeloid leukemia.

Archive of clinical cases
2023

Painful plaques in a woman with recurrent squamous cell carcinoma.

JAAD case reports
2024

Acute Febrile Neutrophilic Dermatosis (Sweet Syndrome) in Acute Myeloid Leukemia Patients: A 28-Year Institutional Experience.

Acta haematologica
2024

Cutaneous vasculitis in autoinflammatory diseases.

The Journal of dermatology
2023

VEXAS-Syndrome, a newly described autoinflammatory systemic disease with dermatologic manifestations.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
Ver todos os 688 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Extraintestinal Cutaneous Manifestations in Inflammatory Bowel Disease Among Non-White Patients: A Retrospective Multicenter Study.
    Journal of clinical gastroenterology· 2026· PMID 41564030mais citado
  2. Clinicopathologic Spectrum of Dermatological Diseases in Patients with Acute Myeloid Leukemia (AML): A Retrospective Study in AML Patients with Cutaneous Manifestations.
    Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion· 2026· PMID 41522545mais citado
  3. Myeloid dermatosis with features of sweet syndrome and leukemia cutis: a case report.
    Annals of hematology· 2026· PMID 41870653mais citado
  4. Wrong Place at the Right Height: Scalp Orf in a Toddler With Dense Dermal Neutrophilic Infiltrate Mimicking Sweet Syndrome.
    Journal of cutaneous pathology· 2026· PMID 41795885mais citado
  5. From Aortitis to Sweet's: The Immune Spectrum of G-CSF Adverse Events.
    Seminars in arthritis and rheumatism· 2026· PMID 41655516mais citado
  6. A Rare Hybrid Presentation: Coexistence of Necrotizing and Histiocytoid Variants of Sweet Syndrome (SS) in a Patient With Acute Myeloid Leukemia (AML).
    Cureus· 2026· PMID 41994822recente
  7. Subcutaneous Sweet Syndrome as an uncommon mucocutaneous manifestation in systemic lupus erythematosus: a case report and literature review.
    Immunol Res· 2026· PMID 41945165recente
  8. Vaping and Orthopaedic Surgery: A scoping review of the literature.
    Acta Orthop Belg· 2025· PMID 41928739recente
  9. Sweet Syndrome in a Patient With Adult-Onset Immunodeficiency Due to Anti-Interferon-Gamma Autoantibodies: A Case Report and Brief Review of the Literature.
    Am J Dermatopathol· 2026· PMID 41824289recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:3243(Orphanet)
  2. OMIM OMIM:608068(OMIM)
  3. MONDO:0011959(MONDO)
  4. GARD:521(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Artigo Wikipedia(Wikipedia)
  8. Q1514476(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Sweet
Compêndio · Raras BR

Síndrome Sweet

ORPHA:3243 · MONDO:0011959
Prevalência
Unknown
Herança
Multigenic/multifactorial
CID-10
L98.2 · Dermatose neutrofílica febril [Sweet]
CID-11
Ensaios
1 ativos
Início
Adult
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0085077
EuropePMC
Wikidata
Wikipedia
Papers 10a
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