Alopecia areata (AA), também conhecida como pelada, é uma condição em que há perda de cabelo e pelos de algumas ou de todas as áreas do corpo. Frequentemente, resulta em algumas manchas carecas no couro cabeludo, cada uma do tamanho de uma moeda. Estresse psicológico e doenças são possíveis fatores para desencadear a alopecia areata em indivíduos de risco, mas na maioria dos casos não há um gatilho óbvio. De modo geral, as pessoas são saudáveis. Em alguns casos, todo o cabelo do couro cabeludo é perdido, ou todos os pelos do corpo são perdidos. A perda de cabelo pode ser permanente ou temporária.
Introdução
O que você precisa saber de cara
Visão geral
A Síndrome autoinflamatório genético com acne e/ou hidradenite supurativa é uma doença rara caracterizada por inflamação sistêmica desregulada de causa genética, que se manifesta principalmente por lesões cutâneas como acne grave e/ou hidradenite supurativa (inflamação crônica das glândulas sudoríparas). A condição faz parte do grupo das síndromes autoinflamatórias hereditárias, nas quais o sistema imune inato é ativado de forma inadequada, levando a episódios recorrentes de febre e inflamação em diversos órgãos.[1]
Sinais e sintomas
Os principais sintomas incluem acne inflamatória de início precoce e/ou hidradenite supurativa, caracterizada por nódulos dolorosos, abscessos e fístulas em regiões como axilas, virilhas e região perianal. Podem ocorrer episódios de febre recorrente, artrite, mialgia e fadiga. A gravidade e a idade de início variam amplamente entre os pacientes, mesmo dentro de uma mesma família.[1]
Causas genéticas
A síndrome é causada por mutações em genes que regulam a resposta inflamatória do organismo. Embora genes específicos ainda estejam sendo identificados, a condição segue um padrão de herança autossômico dominante ou recessivo, dependendo do gene envolvido. O diagnóstico genético pode ser confirmado por sequenciamento de painel de genes associados a síndromes autoinflamatórias.[1][2]
Diagnóstico
O diagnóstico é baseado na avaliação clínica dos sintomas cutâneos e sistêmicos, história familiar e exclusão de outras causas de acne e hidradenite supurativa. Exames laboratoriais podem mostrar marcadores inflamatórios elevados (como PCR e VHS). O teste genético molecular é fundamental para confirmar a causa genética, identificando variantes patogênicas em genes associados à via inflamatória. Até o momento, não há dados específicos sobre variantes depositadas no ClinVar para esta síndrome.[1][2]
Tratamento e manejo
O manejo é multidisciplinar e visa controlar a inflamação sistêmica e as lesões cutâneas. O tratamento inclui medicamentos imunomoduladores e anti-inflamatórios, como corticosteroides, anti-TNF (por exemplo, adalimumabe) e inibidores de interleucinas (como anakinra). A abordagem cirúrgica pode ser necessária para drenagem de abscessos ou remoção de tecido cicatricial em casos de hidradenite supurativa grave. No Brasil, o acesso a esses tratamentos pode ser parcial pelo SUS, dependendo da avaliação médica e da disponibilidade local.[1]
Prognóstico e qualidade de vida
O prognóstico varia conforme a gravidade da inflamação e a resposta ao tratamento. Com manejo adequado, muitos pacientes apresentam melhora significativa dos sintomas e qualidade de vida. No entanto, a doença pode causar cicatrizes permanentes, dor crônica e impacto psicológico. O acompanhamento regular com dermatologista e reumatologista é essencial para ajustar a terapia e prevenir complicações.[1]
Conteúdo informativo gerado e mantido automaticamente a partir de fontes oficiais (Orphanet, HPO, OMIM, SUS). Não substitui avaliação médica.
A síndrome autoinflamatório genético com acne e/ou hidradenite supurativa é um grupo de condições autoinflamatórias raras marcadas por lesões graves na pele. Os pacientes afetados costumam apresentar quadros inflamatórios de difícil controle que se manifestam como acne e hidradenite supurativa. O acompanhamento em serviços especializados é fundamental para orientar o diagnóstico clínico e definir as estratégias de cuidado.
Alopecia areata (AA), também conhecida como pelada, é uma condição em que há perda de cabelo e pelos de algumas ou de todas as áreas do corpo. Frequentemente, resulta em algumas manchas carecas no couro cabeludo, cada uma do tamanho de uma moeda. Estresse psicológico e doenças são possíveis fatores para desencadear a alopecia areata em indivíduos de risco, mas na maioria dos casos não há um gatilho óbvio. De modo geral, as pessoas são saudáveis. Em alguns casos, todo o cabelo do couro cabeludo é perdido, ou todos os pelos do corpo são perdidos. A perda de cabelo pode ser permanente ou temporária.
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Entender a doença
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Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome autoinflamatório genético com acne e/ou hidradenite supurativa
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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
IgA autoantibodies promote inflammation, Th17 polarization and fibrotic responses in hidradenitis suppurativa.
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease with complex immune dysregulation. While previous studies have reported tertiary lymphoid structures (TLS) and IgA-producing B cells in HS skin lesions, the pathogenic role of IgA autoantibodies remains insufficiently characterized. Here, we demonstrate that lesional skin from patients with HS exhibits elevated expression of IGHA1, IGHA2, and J chain, as confirmed by qPCR, immunofluorescence, and Western blot analyses. Single-cell RNA sequencing identified local plasma cells and B cells as the primary source of IgA. Autoantigen profiling revealed a diverse repertoire of IgA autoantibodies targeting nuclear, cytoplasmic, and membrane antigens, with levels correlating with disease severity and other clinical manifestations. We identified IgA autoantibodies binding to CD68⁺ macrophages, which induced secretion of TNF, IL-6, and IL-1β and upregulated inflammasome and profibrotic pathways. Anti-neutrophil extracellular trap (NET) IgA was elevated in HS and promoted NET formation, establishing a pathogenic feedback loop. NET-IgA immune complexes induced macrophages to secrete CCL18, driving collagen production by fibroblasts and promoting a type I interferon (IFN) gene signature. IgA immune complexes presented by myeloid dendritic cells activated CD4⁺ T cells, triggering IFN-γ production and further amplifying local inflammation. Notably, supernatants from macrophages exposed to IgA were able to polarize naïve CD4⁺ T cells toward a Th17 phenotype, linking innate immune activation to the expansion of pathogenic adaptive immune responses. Direct exposure of fibroblasts to NET-IgA complexes triggered expression of adhesion molecules, chemokines, and regulators of adaptive immunity. Together, these findings uncover a role for IgA autoantibodies in HS, implicating them as central drivers of chronic inflammation, fibrosis, and immune crosstalk across neutrophils, macrophages, fibroblasts, and T cells.
Lutikizumab in Adults With Moderate to Severe Hidradenitis Suppurativa After Anti-TNF Therapy Failure: A Phase 2 Randomized Clinical Trial.
Hidradenitis suppurativa (HS) is a debilitating inflammatory skin disease with limited therapeutic options. To assess the efficacy and safety of lutikizumab, a dual-variable domain interleukin 1α/1β antagonist, for adult participants with moderate to severe HS who experienced anti-tumor necrosis factor (TNF) therapy failure. This phase 2, double-blind, placebo-controlled randomized clinical trial was conducted at 45 sites in 8 countries or territories (Australia, Canada, Germany, Greece, Japan, Puerto Rico, Spain, and the US) between December 28, 2021, and November 27, 2023, and included a 35-day screening period, a 16-week treatment period, and a 9-week safety follow-up. Data were analyzed in December 2023. Participants 18 years or older with a diagnosis of HS for 12 months or longer who experienced anti-TNF treatment failure were enrolled. Eligible participants were randomized 1:1:1:1 to receive lutikizumab, 300 mg, every week; lutikizumab, 300 mg, every other week; lutikizumab, 100 mg, every other week; or placebo, every week. The trial drug was administered starting at baseline and through week 16. The primary end point was achieving a Hidradenitis Suppurativa Clinical Response (HiSCR 50) at week 16. The secondary efficacy objective was achieving at least a 30% reduction and at least 1-unit reduction from baseline in Patient's Global Assessment of skin pain (Numerical Rating Scale [NRS] 30) after 16 weeks of treatment among among patients with a baseline NRS 3 or greater. Additional end points included achieving HiSCR 75 and HiSCR 90, change and percentage change from baseline in draining tunnels, and change from baseline in Dermatology Life Quality Index total scores. A total of 153 participants received at least 1 dose of the trial medication (94 female individuals [61.4%]; mean [range] age, 40.5 [19-75] years; 108 patients [70.6%] at Hurley stage III). At week 16, 19 (48.7%), 22 (59.5%), and 10 participants (27.0%) receiving lutikizumab, 300 mg, every week; lutikizumab, 300 mg, every other week; and lutikizumab, 100 mg, every other week, respectively, achieved HiSCR50 at week 16 compared with placebo (35.0%); posterior probabilities of positive treatment effect vs placebo were 89.3%, 98.5%, and 22.8%, respectively, using a bayesian analysis. Among participants with baseline NRS scores of 3 or greater, 10 (34.5%) who received lutikizumab, 300 mg, every other week and 8 (34.8%) who received lutikizumab, 300 mg, every week achieved an NRS30 response compared with 4 (12.9%) who received placebo. There were no deaths or treatment-emergent adverse events of neutropenia, serious hypersensitivity reactions, major adverse cardiovascular events, or opportunistic infections reported. In this phase 2 randomized clinical trial, lutikizumab, 300 mg, every week and 300 mg every other week showed positive treatment effects vs placebo in a hard-to-treat moderate to severe HS population that experienced anti-TNF therapy failure. ClinicalTrials.gov Identifier: NCT05139602.
singIST: An integrative method for comparative single-cell transcriptomics between disease models and humans.
Disease models are fundamental tools in drug discovery and early-stage drug development, but they only approximate human disease, and selecting a suitable model is challenging. Quantitative computational methods exist to assess molecular resemblance to human conditions, but approaching that work at single-cell resolution, and doing so in an explainable and generalizable way, remain very limited. We present singIST, a computational method for comparative single-cell transcriptomics analysis between disease models and human conditions. singIST provides explainable quantitative measures on disease model similarity to the human reference at the pathway, cell type and gene levels. These measures jointly account for gene orthology, cell type presence in the model, cell type and gene importance in the human condition, and gene level fold changes in the model, within a unifying framework that controls for the intrinsic complexities of single-cell data. We first test singIST in three well-characterized murine models against moderate-to-severe Atopic Dermatitis, showing that it recapitulates established biology while generating new hypotheses. We then apply it to Hidradenitis Suppurativa, comparing in vivo human lesions with ex vivo skin explants with and without CD3/CD28 stimulation, and show that stimulation selectively improves pathways that already recapitulate the human signal. Finally, we perform simulation studies that: (i) unit-test the implementation and behaviour of the algorithm under controlled scenarios and (ii) compare singIST against a naïve baseline based on overlapping differentially expressed genes.
Iron Deficiency in Immune-Mediated Inflammatory Skin Diseases: A Missing Link Between Systemic Inflammation, Immunometabolism, and Disease Burden.
Iron deficiency (ID) has emerged as a pivotal yet underrecognized factor in the pathogenesis of immune-mediated inflammatory skin diseases (IMISDs) such as psoriasis, atopic dermatitis, and hidradenitis suppurativa. Beyond its classical role in erythropoiesis, iron acts as a key modulator of immune cell activity, redox balance, and overall metabolic homeostasis. This review synthesises the latest evidence on the intricate relationship between systemic inflammation, disturbances of iron metabolism, and immunometabolic imbalances that underline the pathogenesis of IMISDs. Findings indicate that chronic inflammation drives functional iron deficiency through IL-6-hepcidin-mediated sequestration of iron, resulting in reduced bioavailability and altered mitochondrial activity in immune and epithelial cells. This imbalance is associated with excessive and chronically enhanced oxidative and inflammatory responses of these cells, further advancing inflammation, anaemia of chronic disease, and disturbances of tissue repair. Moreover, emerging evidence supports an "iron-skin axis," and suggests that skin cells, particularly epidermal keratinocytes, are actively involved in the regulation of iron pathways. Collectively, these insights position iron homeostasis as a missing link between systemic inflammation, immunometabolic imbalance, and disease burden in IMISDs.
Hidradenitis Suppurativa and Risk of Self-Harm and Suicide.
This cohort study evaluates the incidence of self-harm and suicide among individuals with hidradenitis suppurativa.
Publicações recentes
Topical Ruxolitinib for the Treatment of Hidradenitis Suppurativa.
Beneficial effects of upadacitinib on a subgroup of patients with atopic dermatitis and hidradenitis suppurativa: a multicenter, real-world retrospective study.
The Emerging Role of Gut Microbiota in Inflammatory Skin Diseases: A Systematic Review.
Intact Fish Skin Grafting in a Patient With Hidradenitis Suppurativa.
Treatment Strategy for Refractory Dissecting Cellulitis of the Scalp Using Bimekizumab, Isotretinoin, and Oral Antibiotics.
📚 EuropePMCmostrando 197
A skin colonizer disrupts inflammatory and humoral immune defenses in hidradenitis suppurativa.
EMBO molecular medicineIgA autoantibodies promote inflammation, Th17 polarization and fibrotic responses in hidradenitis suppurativa.
Nature communicationsCombined thermography and ultrasound in hidradenitis suppurativa: A preliminary study.
Journal of the European Academy of Dermatology and Venereology : JEADVEffectiveness and Safety of Antidiabetic Medications in Hidradenitis Suppurativa: A Systematic Review.
Clinical, cosmetic and investigational dermatologySpanish Clinical Management Guideline for Hidradenitis Suppurativa. Part I: Pathogenesis, Diagnosis, and Disease Staging.
Actas dermo-sifiliograficasHidradenitis Suppurativa in a Sample of Lebanese Patients With Acne: Rate and Correlates.
Actas dermo-sifiliograficasAlcohol Use and Hidradenitis Suppurativa: An Unclear Relationship.
Skin appendage disordersNerves and nodules: Mononeuritis multiplex as a reversible complication of severe Hidradenitis Suppurativa.
JAAD case reportsEfficacy and safety of secukinumab in conjunction with surgery in moderate to severe hidradenitis suppurativa.
JAAD internationalGamma secretase inhibitor nirogacestat and secondary hidradenitis suppurativa: An expected on-target effect.
Annales de dermatologie et de venereologieHidradenitis suppurativa in transgender patients: A case series.
Annales de dermatologie et de venereologieA Narrative Review of Local Therapies in Hidradenitis Suppurativa: Treatment Landscape and Current Issues.
Dermatology and therapyLutikizumab in Adults With Moderate to Severe Hidradenitis Suppurativa After Anti-TNF Therapy Failure: A Phase 2 Randomized Clinical Trial.
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Journal of the European Academy of Dermatology and Venereology : JEADVLong-Term Outcomes of Secukinumab in Moderate-to-Severe Hidradenitis Suppurativa: A Multicentre Cohort Study.
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Clinical and experimental dermatologyPhenotype-genotype correlation and treatment outcomes in mevalonate kinase deficiency: A large Turkish cohort.
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International journal of dermatologyUltrasound-guided Percutaneous Galvanic Current Compared to Ultrasound-guided Intralesional Corticosteroid Infiltration in Tunnels of Hidradenitis Suppurativa.
Actas dermo-sifiliograficasRisk of Cancer in Patients with Hidradenitis Suppurativa: A Systematic Review and Meta-analysis.
Dermatology and therapyHidradenitis Suppurativa and Risk of Self-Harm and Suicide.
JAMA dermatologyBiologics, cytokine blockade, and mental health outcomes for patients with hidradenitis suppurativa.
The Journal of investigative dermatologyDiabetes status is associated with greater disease severity in hidradenitis suppurativa.
JID innovations : skin science from molecules to population healthTumor Necrosis Factor-Alpha (TNF-α) Inhibitor Treatment in Hidradenitis Suppurativa: A Population-Based Retrospective Cohort Study of Comorbid Risks.
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The Australasian journal of dermatologyDefining Moderate Disease and Progression in Hidradenitis Suppurativa: An Expert Framework to Unlock the Window of Opportunity for Prompt Treatment.
American journal of clinical dermatologyA geophysical model for understanding inflammatory lesion dynamics in hidradenitis suppurativa.
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bioRxiv : the preprint server for biologyThe Emerging Role of Gut Microbiota in Inflammatory Skin Diseases: A Systematic Review.
Experimental dermatologyDiagnostic delay for hidradenitis suppurativa: No change in the 2015-2024 decade.
Journal of the European Academy of Dermatology and Venereology : JEADVGenomic and proteomic insights into hidradenitis suppurativa.
Journal of the European Academy of Dermatology and Venereology : JEADVSecondary Amyloidosis in Hidradenitis Suppurativa: A Case Series and a Proposal for Practical Screening Recommendations.
International journal of dermatologyEthics of Insurance-driven Barriers to Biologic Therapy in Hidradenitis Suppurativa.
Journal of the American Academy of DermatologySevere Hidradenitis Suppurativa and Subsequent Myelodysplastic Syndrome: A Case Report Highlighting Chronic Inflammatory Burden and Carcinogenic Risk.
CureusIntact Fish Skin Grafting in a Patient With Hidradenitis Suppurativa.
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The British journal of dermatologyHighlights from JAAD Case Reports: May 2026.
Journal of the American Academy of DermatologyTreatment Strategy for Refractory Dissecting Cellulitis of the Scalp Using Bimekizumab, Isotretinoin, and Oral Antibiotics.
Journal of drugs in dermatology : JDDImpact of TNF-α and IL-17 Biologics on Cardiometabolic Outcomes in Hidradenitis Suppurativa: Retrospective Cohort Study.
Journal of drugs in dermatology : JDDWide excision and latissimus dorsi flap reconstruction for advanced axillary hidradenitis suppurativa: a case series.
Case reports in plastic surgery & hand surgeryPsychiatric and Psychological Approach in Patients with Atopic Dermatitis, Hidradenitis Suppurativa and Psoriasis: A Propensity-Matched Real-World Cohort Study.
Dermatology and therapyClosing the implementation gap for HS surgery and laser therapies in the NHS- Comment on 'The role of surgery in hidradenitis suppurativa: Expert-based holistic approaches'.
Clinical and experimental dermatologyTranscriptomic profiling reveals distinct molecular signatures among lesion types in hidradenitis suppurativa.
Frontiers in immunologyBurden of hidradenitis suppurativa in Germany: a retrospective claims data analysis and comparison with non-HS patients.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGGlobal pain management trends and barriers to care in hidradenitis suppurativa.
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JMIR dermatologyPredictors for Remission versus Refractory Disease Following Wide Local Excision Surgery for Hidradenitis Suppurativa: A Single-Center Case-Control Analysis.
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Experimental dermatologyTargeting Neutrophil Function as Therapy for Hidradenitis Suppurativa.
International journal of molecular sciencesModestly Increased Incidence of Irritable Bowel Syndrome in Hidradenitis Suppurativa: A Retrospective Cohort Study Using TriNetX.
International journal of dermatologySOX9/Kruppel-like factor 5 and the infiltrating epidermal hyperplasia of hidradenitis suppurativa.
Journal of the American Academy of DermatologyThe Intratunnel Implementation of Gentamicin-Loaded Calcium Sulfate Beads for Hidradenitis Suppurativa.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]A Matched Pairs Analysis of GLP-1 Receptor Agonists in Hidradenitis Suppurativa.
American journal of clinical dermatologyAn Update to Ethnoracial Gaps in Global Hidradenitis Suppurativa Clinical Trials.
Skin appendage disordersObesity, skin disorders, and the microbiota: Unraveling a complex web.
Journal of microbiology (Seoul, Korea)Steatocystoma Multiplex Suppurativa-A Case Report and Review of Treatments.
The Australasian journal of dermatology[Inflammatory dermatosis in adult chronic non-bacterial osteomyelitis (adult CNO) from a dermatological perspective].
Zeitschrift fur RheumatologieFractional Microneedling Radiofrequency for Hidradenitis Suppurativa: A Real-World Retrospective Study Demonstrating Clinical Efficacy and Safety Across Diverse Anatomical Sites.
Journal of cosmetic dermatologyUltrasound-guided cooled radiofrequency ablation for sacroiliac joint pain in a patient with PsAPASH syndrome: A case report.
Interventional pain medicineHidradenitis suppurativa in patients with inflammatory bowel disease: a national multicenter study from the GETECCU-ENEIDA Registry.
Therapeutic advances in gastroenterologyGlucagon-Like Peptide-1 Receptor Agonists as Adjunctive Therapy for Hidradenitis Suppurativa in Patients With Overweight/Obesity: A Narrative Review of Efficacy, Safety, and Quality-of-Life Outcomes.
International journal of dermatologyTherapeutic drug monitoring of adalimumab in hidradenitis suppurativa: a prospective observational study.
Frontiers in immunologyTime to Reclassify Autoinflammatory Syndromes Linked to Acne, Hidradenitis Suppurativa, and Arthritis?
International journal of dermatologyBaseline monocytes ≥ 925/μL appear to represent a rare outlier phenotype rather than a generalizable predictor of adalimumab response in hidradenitis suppurativa.
Journal of the American Academy of DermatologyEconomic Burden of Hidradenitis Suppurativa in Spain.
Dermatology and therapySpatial transcriptomics maps early B-cell and T-cell activation in hidradenitis suppurativa.
The Journal of investigative dermatologyPsychopharmacology in Dermatology.
Indian dermatology online journalPeculiar Phenotype of Linear Prepubertal Hidradenitis Suppurativa Associated With Trisomy 13: Report of Two Clinical Cases.
Pediatric dermatologyPhenotypic clustering in adult SAPHO syndrome: A multicenter retrospective study of 102 patients.
Journal of the American Academy of DermatologySwiss S1 guidelines for the management of hidradenitis suppurativa.
Dermatology (Basel, Switzerland)The effect of the plant-derived prenylated flavonoid icariin on acne vulgaris and atrophic acne scars.
The British journal of dermatologyJAAD CME Part 1: Mechanism of Action of GLP-1 Receptor Agonists and Potential Pathways in Skin Health.
Journal of the American Academy of DermatologyThe Effects of Walk-In Dermatology Appointments for Atopic Dermatitis and Hidradenitis Suppurativa Management.
Pediatric dermatologyManagement of Hidradenitis Suppurativa in the United Arab Emirates (UAE): A Consensus Statement from the Emirates Dermatology Society.
Dermatology and therapy[Acne inversa/Hidradenitis suppurativa].
MMW Fortschritte der MedizinJAAD CME Part 2: Clinical Evidence and Safety Considerations for GLP-1 Receptor Agonists in Dermatology.
Journal of the American Academy of DermatologyStress in dermatology patients: A multicenter observational study of 8295 outpatients and controls from 22 European clinics.
JAAD internationalReal-world experience with upadacitinib in hidradenitis suppurativa: efficacy and safety in patients with dermatological comorbidities.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGManaging Skin Diseases that Flare During Pregnancy and in the Postpartum period: Part 2- Management & Safety Considerations.
Journal of the American Academy of DermatologyIncidence of hidradenitis suppurativa in transgender and cisgender individuals: A multicenter matched cohort study.
Journal of the American Academy of DermatologyArtificial intelligence-enabled precision medicine for inflammatory skin diseases.
The Journal of investigative dermatologyBimekizumab efficacy using IHS4 outcomes in hidradenitis suppurativa: Results from BE HEARD I and II.
Journal of the European Academy of Dermatology and Venereology : JEADV"Efficacy and safety of bimekizumab in moderate-to-severe hidradenitis suppurativa: A systematic review and meta-analysis of RCTs".
MedicineTwo phase 3 trials of bimekizumab for the treatment of moderate-to-severe hidradenitis suppurativa: a critically appraised research paper.
The British journal of dermatologyPSMA4 as a Druggable Target in Hidradenitis Suppurativa: Evidence From Mendelian Randomization and Single-Cell Transcriptomics.
Mediators of inflammationMicrobiome-Derived Indole-3-Lactic Acid Attenuates Cutibacterium Acnes-Induced Inflammation via the Aryl Hydrocarbon Receptor Pathway.
International journal of molecular sciencesAssessing Disease Progression in Hurley I Hidradenitis Suppurativa Patients: A Nested Case-Control Study.
Dermatology (Basel, Switzerland)Clinical Characteristics and Management of Pediatric Hidradenitis Suppurativa: A Canadian Single-Centre Multidisciplinary Experience.
Journal of cutaneous medicine and surgeryTopical roflumilast 0.3% cream for mild hidradenitis suppurativa: A prospective case series.
JAAD case reportsThe Impact of Glucagon-Like Peptide-1 (GLP-1) Agonists on Acne, Hidradenitis, and Sebaceous Activity.
CureusTreat-to-target: optimising outcomes for hidradenitis suppurativa.
The British journal of dermatologyRook to the Rescue: A Case Series on the Novel Use of Eggcrate Foam Bolsters for Skin Grafts in Penile and Genital Reconstruction: Rook Bolster Technique for Genital Reconstruction.
Annals of plastic surgeryEfficacy and Safety of Moxifloxacin in Pediatric Hidradenitis Suppurativa: Results From the Canadian Multidisciplinary Registry of Pediatric HS.
Journal of cutaneous medicine and surgeryUnusual Sternoclavicular Joint Infection With Pseudomonas in a Young Adult With Hidradenitis Suppurativa: A Case Report.
CureusEthics of school and work accommodations in psychosocial management of hidradenitis suppurativa.
Journal of the American Academy of DermatologyVenous Abnormalities in Biopsy-confirmed Lipodermatosclerosis.
Advances in skin & wound careA Case of Estrogen Dermatitis Successfully Treated With Spironolactone.
CureusSuicide in patients with skin diseases: What to look out for?
Journal of the European Academy of Dermatology and Venereology : JEADVMulti-omics profiling of chronic immune-mediated skin diseases: SKINERGY protocol and strategic evaluation.
Journal of the European Academy of Dermatology and Venereology : JEADVUpadacitinib for the management of refractory hidradenitis suppurativa, psoriasis, and pyoderma gangrenosum.
Dermatology reportsPD-L1-Inhibitor-Associated Hidradenitis Suppurativa.
Journal of cutaneous pathology[Hidradenitis suppurativa and psoriasis. Therapeutic response to secukinumab].
MedicinaGLP-1 Receptor Agonists in Overweight and Obese Patients With Hidradenitis Suppurativa.
Journal of drugs in dermatology : JDDPolyclonal Hypergammaglobulinemia in Severe Hidradenitis Suppurativa.
CureusAnoscrotal fistulas: causes and management in a 10-year French case series.
International journal of colorectal diseaseLife-Threatening Potential of Deep Fistulas in Hidradenitis Suppurativa: A Case of Axillary Vessel-Adjacent Lesion With MMP Overexpression.
The Journal of dermatologyRisk of irritable bowel syndrome in patients with hidradenitis suppurativa: a global-federated, multicenter cohort study.
Scientific reportsA genome-wide association study of hidradenitis suppurativa from the VA's Million Veteran Program.
Dermatology (Basel, Switzerland)Remote monitoring in chronic immune-mediated inflammatory diseases: A systematic review of active and passive approaches.
JAAD internationalGLP-1 receptor agonist use in patients with psoriasis or hidradenitis suppurativa.
JAAD internationalEvaluation of ASCA IgA/IgG seropositivity, fecal calprotectin levels and NOD2 gene polymorphisms in patients with hidradenitis suppurativa: A prospective case-control study.
JAAD internationalImpact of smoking on disease severity and treatment outcomes in hidradenitis suppurativa.
Journal of the European Academy of Dermatology and Venereology : JEADVHidradenitis Suppurativa Associated With Gamma-Secretase Inhibitor Therapy: A Systematic Review.
Journal of cutaneous medicine and surgeryNon-invasive imaging for monitoring nodular inflammatory lesions in hidradenitis suppurativa.
Dermatology reportsPharmacomicrobiomics in inflammatory skin diseases: past, present, and the future.
Frontiers in microbiologyAdalimumab-Induced Hepatocellular Injury in a Young Male With Hidradenitis Suppurativa and Underlying Metabolically Dysfunctional Liver Disease: A Case Report.
CureusCombined Nonablative Fractional Laser and Surgical Debulking for Facial Hidradenitis Suppurativa.
Lasers in surgery and medicineRisk of Inflammatory Dermatoses Following Covid-19 Infection: A cohort Study of 204,241 Patients.
Clinical and experimental dermatologyThe role of surgery in hidradenitis suppurativa: Expert-based holistic approaches.
Clinical and experimental dermatologyHidradenitis Suppurativa Patient-Reported Outcome Measures: A Systematic Review and Meta-Analysis.
JAMA dermatologyThe Safety of Perioperative Liposomal Bupivacaine Injections in Dermatologic Surgery Patients.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Successful Treatment of Pyoderma Gangrenosum Associated With Hidradenitis Suppurativa With Bimekizumab.
The Australasian journal of dermatologyLong-term efficacy of biologics in moderate-to-severe hidradenitis suppurativa: A systematic review and MAIC.
Journal of the European Academy of Dermatology and Venereology : JEADVInfectious Disease Screening and Vaccinations Guidelines for Patients Initiating Immunosuppression for Dermatologic Conditions: A Multidisciplinary Design and Quality Improvement Initiative.
The Australasian journal of dermatologyClinical and laboratory characteristics of hidradenitis suppurativa in a Chinese cohort: a retrospective analysis of 197 cases.
Frontiers in medicineA Cross-Sectional Study on Relationships Between Depression and Anxiety in Hidradenitis Suppurativa Patients and Disease Severity, Subjective Symptoms and Quality of Life.
Journal of clinical medicineSpecies-Level Comparative Metagenomic Analysis of the Bacterial Abundance of the Gut Microbiome in Psoriasis, Hidradenitis Suppurativa, and Pemphigus Foliaceous Patients Using Shotgun Next-Generation Sequencing.
International journal of molecular sciencesSpecialized Pro-Resolving Lipid Mediators and Dietary Omega-3/6 Fatty Acids in Selected Inflammatory Skin Diseases: A Systematic Review.
Antioxidants (Basel, Switzerland)Adalimumab 80mg weekly dose escalation for hidradenitis suppurativa: A multi-centre retrospective cohort study incorporating therapeutic drug monitoring.
Clinical and experimental dermatologyHow can we improve patient care in hidradenitis suppurativa?
Journal of the European Academy of Dermatology and Venereology : JEADVClinical and Transcriptomic Response to Secukinumab in Korean Patients with Moderate-to-Severe Hidradenitis Suppurativa.
Dermatology (Basel, Switzerland)GLP-1 receptor agonists as adjunct therapy in hidradenitis suppurativa: A hypothesis.
International journal of clinical pharmacology and therapeuticsMicrobial species in deep tissue lesions of Hurley stage III hidradenitis suppurativa: a retrospective observational study.
BMC microbiologyDelphi consensus: First-line use of biologics and small molecules in hidradenitis suppurativa.
Journal of the European Academy of Dermatology and Venereology : JEADVOcular and auditory associations in patients with vitiligo: A single-center retrospective study of 8512 patients.
Journal of the American Academy of DermatologyRisk factors for sacrococcygeal pilonidal sinus: a systematic review and meta-analysis supplemented by genetic causal assessment.
Frontiers in surgeryExtensive Genital Hidradenitis Suppurativa With Massive Fibrosis After Delayed Surgery in the Biologic Era.
The Journal of dermatologyMyosteatosis as a metabolic risk in hidradenitis suppurativa: a CT-based study.
Frontiers in medicineCase Report: Refractory hidradenitis suppurativa complicated by IgA vasculitis with nephritis: successful long-term control with surgery and bimekizumab.
Frontiers in immunologyExtraintestinal Cutaneous Manifestations in Inflammatory Bowel Disease Among Non-White Patients: A Retrospective Multicenter Study.
Journal of clinical gastroenterology[Genetic basis of chronic nonbacterial osteomyelitis].
Zeitschrift fur RheumatologieThe Roles of Keratinocytes in the Initiation, Progression and Maintenance of Hidradenitis Suppurativa.
Experimental dermatologyMultiomics analyses prioritize disease genes and pathways in hidradenitis suppurativa.
The Journal of investigative dermatologyQuantifying Treatment Burden in Hidradenitis Suppurativa: A Cohort Study.
Journal of cutaneous medicine and surgery"I Am An HS Warrior": A Reflexive Thematic Analysis of #HidradenitisSuppurativa on TikTok.
Journal of cutaneous medicine and surgeryCombination Ablative Fractional CO 2 Laser, Intense Pulsed Light, and Triamcinolone for the Management of Hidradenitis Suppurativa: A Case Series.
Advances in skin & wound careThe role of γ-secretase in familial hidradenitis suppurativa: Implications for pathogenesis and targeted therapy.
JAAD case reportsImprovement of Chronic Spontaneous Urticaria After Glucagon-Like Peptide 1 Receptor Agonist Therapy: Report of Two Cases.
Dermatology and therapyPaediatric Hidradenitis Suppurativa: Successful Treatment With Bimekizumab and Multidimensional Impact on Quality of Life.
The Australasian journal of dermatologyHidradenitis Suppurativa With Concomitant Arthritis: A Call for Disease Interception and Combination Therapy?
International journal of dermatologyFamilial Dowling-Degos Disease with Hidradenitis Suppurativa associated with loss-of-function NCSTN mutation.
Clinical and experimental dermatologyGluteal-anogenital-perineal reconstruction in patients with chronic hidradenitis suppurativa and related squamous cell carcinoma.
Journal of wound careReevaluating red nodules in hidradenitis suppurativa: clinical, dermatoscopic and histopathologic correlation.
Italian journal of dermatology and venereologyFrom Years of Pain to a Name: Fibromyalgia Uncovered.
CureusInsulin Resistance, Metabolic Syndrome, and Inflammatory Skin Disease.
Journal of clinical medicineOut-of-pocket costs of hidradenitis suppurativa are significantly higher than those for psoriasis: A cross-sectional survey study.
Journal of the American Academy of DermatologyAndrogenic hidradenitis suppurativa onset linked to gamma secretase inhibitor.
JAAD case reportsA Case of VEXAS Syndrome.
WMJ : official publication of the State Medical Society of WisconsinPAPASH Syndrome: A Case Report and Lessons for Clinical Practice.
WMJ : official publication of the State Medical Society of WisconsinValidation of International Classification of Diseases Codes for Dermatologic Conditions: A Systematic Review.
JAMA dermatologyWHO-5 Well-Being Index Reveal the Burden of Hidradenitis Suppurativa.
International journal of dermatologyCutaneous Adverse Events Associated With GLP-1 Receptor Agonists: A FAERS Database Analysis From 2018-2024.
Journal of drugs in dermatology : JDDDermatologic indications for microwave-induced local hyperthermia.
The Journal of dermatological treatmentPrevalence of Otitis Media and Eustachian Tube Dysfunction in Adults With Hidradenitis Suppurativa.
International journal of dermatologyA cross-sectional study of the pediatric hidradenitis suppurativa treatment landscape.
JAAD internationalTirzepatide in dermatology: cutaneous adverse events, emerging therapeutic roles, and cosmetic implications - A comprehensive review.
Anais brasileiros de dermatologiaAssociation Between Urolithiasis and Hidradenitis Suppurativa: A Retrospective Cohort Study.
In vivo (Athens, Greece)Accessibility as a Key Factor in the Clinical Evolution and Therapeutic Management of Patients With Hidradenitis Suppurativa.
Actas dermo-sifiliograficasGlucagon-like peptide-1 receptor agonists may decrease biologic initiation in hidradenitis suppurativa: A TriNetX retrospective analysis.
Journal of the American Academy of DermatologyBridging the Gap: Characterizing Complex Immune-Mediated Inflammatory Diseases with Cutaneous Manifestations.
Dermatology (Basel, Switzerland)Editor's Highlights-February 2026.
International journal of dermatologyDysbiosis in Acne Vulgaris and Hidradenitis Suppurativa: A Comparative Microbiome Analysis.
Experimental dermatologyHidradenitis Suppurativa Education for General Practitioners: A Pilot Study.
The Australasian journal of dermatologyHidradenitis suppurativa: Why dose escalation often fails and what biology tells us about next steps.
The Journal of allergy and clinical immunologyInflammatory Skin Disease in Latin America: Treatment Challenges and Opportunities.
Journal of inflammation researchTumor Necrosis Factor (TNF)-Alpha Inhibitors in Hidradenitis Suppurativa: A Systematic Review of Inflammatory Markers and Alternative Monitoring Strategies.
CureusAssociation Between Inflammatory Comorbidities and Hidradenitis Suppurativa Disease Characteristics: A Systematic Review and Meta-Analysis.
Journal of cutaneous medicine and surgeryShort-term efficacy of biologics in moderate-to-severe hidradenitis suppurativa: A systematic review and NMA.
Journal of the European Academy of Dermatology and Venereology : JEADVDefining Surgical Recurrence Patterns in Hidradenitis Suppurativa: Insights from 206 Procedures and Literature Review.
Dermatology and therapyIncreased incidence of neoplasms among patients with hidradenitis suppurativa: A retrospective cohort study using the TriNetX dataset.
Journal of the American Academy of DermatologyResearch progress on the application of nanobodies in immunity and infectious skin diseases.
Frontiers in immunologyBimekizumab for Hidradenitis Suppurativa from Trials to Real Life: A Review of the Published Literature.
Clinical, cosmetic and investigational dermatologyDevelopment of Digital Algorithms for the Early Identification of Rare Clinical Outcomes Using Electronic Medical Records: A Case Study of Hidradenitis Suppurativa.
Population health managementLooking beyond the scalpel: Assessing patient risk factors for complications following surgical excision of hidradenitis suppurativa.
Burns : journal of the International Society for Burn InjuriesIsotretinoin-related adverse sexual and reproductive outcomes: a real-world pharmacovigilance study of the FDA Adverse Event Reporting System (FAERS).
Cutaneous and ocular toxicologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Síndrome autoinflamatório genético com acne e/ou hidradenite supurativa.
É de uma associação que acompanha esta doença? Fale com a gente →
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Ainda não achamos doenças com sintomas parecidos o suficiente.
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Existe um documento oficial para "Hidradenite Supurativa". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
Trata-se de uma categoria clínica cadastrada na Orphanet sob o código ORPHA:652510 que reúne manifestações autoinflamatórias com lesões cutâneas. Ela envolve principalmente a ocorrência de quadros inflamatórios como acne grave e hidradenite supurativa.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
2 fontes citadas no texto · 10 publicações do grafo RarasNet (PubMed) · 2 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (2026). Síndrome autoinflamatório genético com acne e/ou hidradenite supurativa. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/sindrome-autoinflamatorio-genetico-com-acne-eou-hidradenite-supurativa
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Síndrome autoinflamatório genético com acne e/ou hidradenite supurativa
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
