Hidradenitis supurativa: reporte de un caso / Hidradenitis suppurativa: a case report

Resumen La hidradenitis supurativa (HS) es una enfermedad dolorosa y crónica, que afecta en especial la unidad folículo-pilosebácea de la piel ubicada en ingle, axilas, región perianal, perineo, genitales y submamaria,regiones anatómicas donde se encuentran glándulas sudoríparas apocrinas. El curso clínico de la HS es heterogéneo pues varía desde formas muy graves con abscesos fluctuantes profundos y drenajes eventuales; concicatricesresiduales graves, hasta otra forma de enfermedad comparativamente leve caracterizada por la aparición de algunos nódulos inflamatorios,pústulas ypápulas,de manera recidivante. Comunicamos el caso de un niño de 12 añosde edad, con diagnóstico de hidradenitis supurativa,quien fue tratado con Adalimumab. Realizamos la revisión del estado de arte de esta patología, describimos sus características clínicas, criterios diagnósticos, diagnósticos diferenciales y los posibles tratamientos. Nuestra presentación, tiene por objeto relatar nuestra experiencia en el seguimiento del caso del paciente, y las vicisitudes diagnósticas al respecto, que variaron desde acné inflamatorio grave hasta finalmente arribar al diagnóstico de certeza de hidradenitis supurativa, basándonos en criterios clínicos y ecográficos.Consideramos de interés haber podido emplear el "agente biológico" inhibidordel factor de necrosis tumoral (FNT) que hemos mencionado, con excelente respuesta.

Abstract Hidradenitis suppurativa (HS) is a painful and chronic disease that particularlyaffects the follicle-pilosebaceous unit of the skin located in the groins, armpits, perianal region, perineum, genitals, and submammary glands, which areanatomical regions whereapocrine sweat glands are found. The clinical course of HS is heterogeneous as it varies from very severe forms with deep fluctuating abscesses and eventual drainage; with severe residual scars, to another form ofacomparatively mild condition characterized by the presence of some recurrent inflammatory nodules, pustules and papules as well. We report the case of a 12-year-old boy, diagnosed with hidradenitis suppurativa, who was treated with Adalimumab. We reviewed the state of the art of this pathology, described its clinical characteristics, diagnostic criteria, differential diagnoses and carried out possible treatments. The purpose of our presentation is to report our experience in monitoring the patient's case, along with the diagnostic vicissitudes in this regard, which ranged from severe inflammatory acne to finally arriving at a certain diagnosis of hidradenitis suppurativa, based on clinical and ultrasound criteria. We consider of interest the possibility of having been able to use the "biological agent" inhibitor of the tumor necrosis factor (TNF) that we have mentioned, with an excellent response.

Can Ultrasound Examinations Generate Pain in Hidradenitis Suppurativa Patients? Results from a Multicentric Cross-Sectional Study.

BACKGROUND: Pain is not a trivial issue for hidradenitis suppurativa (HS) patients and has been considered a domain in the Core Outcome Set. To date, there is no evidence about pain caused by the ultrasound examinations. OBJECTIVE: The aim of the study was to assess the presence of pain generated by the ultrasound examinations of HS patients. METHODS: A multicentric cross-sectional study for detecting pain during the ultrasound examinations of HS patients using a validated verbal questionnaire immediately after the imaging studies. Statistical analysis included demographic data and possible associations with sex, age, location, clinical (Hurley), and ultrasonographic scoring (SOS-HS). The statistical tests were two proportions Z test, χ2 test, Student's t test, and ANOVA. A p < 0.05 was considered significant. RESULTS: 317 patients met the criteria. 77.3% of them did not present pain. Of cases with pain, 59.8% were mild, 16.7% moderate, and 23.6% severe. No significant association was found with sex, age, staging, location, or the number of affected regions. Although nonsignificant, severe pain cases were more frequent in the clinical Hurley III and ultrasonographic SOS-HS III stages. CONCLUSION: Pain generated by the ultrasound examination of HS patients is infrequent.

Transcriptome Meta-Analysis Confirms the Hidradenitis Suppurativa Pathogenic Triad: Upregulated Inflammation, Altered Epithelial Organization, and Dysregulated Metabolic Signaling.

Hidradenitis suppurativa (HS) is an inflammatory skin condition clinically characterized by recurrent painful deep-seated nodules, abscesses, and sinus tracks in areas bearing apocrine glands, such as axillae, breasts, groins, and buttocks. Despite many recent advances, the pathophysiological landscape of HS still demands further clarification. To elucidate HS pathogenesis, we performed a meta-analysis, set analysis, and a variant calling on selected RNA-Sequencing (RNA-Seq) studies on HS skin. Our findings corroborate the HS triad composed of upregulated inflammation, altered epithelial differentiation, and dysregulated metabolism signaling. Upregulation of specific genes, such as KRT6, KRT16, serpin-family genes, and SPRR3 confirms the early involvement of hair follicles and the impairment of barrier function in HS lesioned skin. In addition, our results suggest that adipokines could be regarded as biomarkers of HS and metabolic-related disorders. Finally, the RNA-Seq variant calling identified several mutations in HS patients, suggesting potential new HS-related genes associated with the sporadic form of this disease. Overall, this study provides insights into the molecular pathways involved in HS and identifies potential HS-related biomarkers.

Tratamiento quirúrgico en fístulas pilonidales en el síndrome de oclusión folicular / Surgical Treatment in Pilonidal Fistulas in Follicular Occlusion Syndrome

Introducción: el síndrome de oclusión folicular está compuesto por acné inverso, acné conglobata y la fístula pilonidal (triada); cuando se asocia la foliculitis disecante del cuero cabelludo entonces constituye la tétrada de oclusión folicular. El acné inverso es una enfermedad supurativa, crónica e invalidante, cuyas características clínicas son la presencia de comedones con múltiples aberturas que vinculan dos o más folículos, abscesos con comunicaciones epitelizadas, y sinus drenantes en regiones con glándulas apocrinas. La génesis de estas afecciones es básicamente genética con expresión dermatológica. Objetivo: caracterizar física e histopatológicamente a los pacientes que padecen de síndrome de oclusión folicular, así como, los resultados del tratamiento quirúrgico de la fístula pilonidal en este trastorno. Método: estudio del comportamiento de la fístula pilonidal en el síndrome de oclusión folicular en 37 enfermos con síndrome de oclusión folicular en Hospital Lenin. Se utilizó la exéresis local de las fístulas pilonidales y perianales, esta exéresis se extendió por el tejido celular subcutáneo hasta la facia. Luego la zona se reparó con injerto libre de piel. Resultados: la fístula pilonidal se presentó en cuatro pacientes de los estudiados con largos períodos evolutivos, los cuales fueron tratados con autoinjerto libre de piel. La hiperqueratosis, así como, la perifoliculitis fueron los cambios histopatológicos de mayor relevancia. Conclusiones: la fístula pilonidal es de difícil manejo en el curso del síndrome de oclusión folicular y solo encuentra solución en el tratamiento quirúrgico con autoinjerto libre de piel.

Introduction: follicular occlusive syndrome includes inverse acne, acne conglobata and pilonidal fistula (triad); when it is associated to the dissecting folliculitis of the scalp then it constitutes the tetrad of follicular occlusion. Reverse acne is a suppurative, chronic and invalidating disease. Its clinical features are the presence of comedones with multiple openings that link two or more follicles, abscesses with epithelial communications, and draining sinuses in regions with apocrine glands. The genesis of these affections is basically genetic with dermatological expression. Objective: to describe physically and histopathologically the patients with follicular occlusion syndrome, as well as the results of surgical treatment of pilonidal fistula in this disorder. Method: the study is on the prevalence of pilonidal fistula in follicular occlusion syndrome in 37 patients with follicular occlusion syndrome. The local excision of the pilonidal and perianal fistulas was performed, this exeresis extended by the subcutaneous cellular tissue until the facia. The area was then repaired with a skin-free graft. Results: four patients had the pilonidal fistula, with long periods of evolution, who were treated with autograft of skin. Hyperkeratosis, as well as, perifolliculitis were the most relevant histopathological changes. Conclusion: the pilonidal fistula is difficult for follicular occlusion syndrome management and only the surgical treatment is the most suitable .

The role of negative pressure wound therapy in the management of hidradenitis suppurativa: a case report and literature review.

Hidradenitis suppurativa (HS), also known as acne inversa, is a chronic, recurrent inflammatory disease affecting skin that bears apocrine glands. It is characterised by the presence of tender subcutaneous nodules that may rupture, resulting in deep dermal abscesses, fibrosis with dermal contractures and induration of the skin. The management of HS is a challenge for physicians as the pathogenesis is not clearly defined and prevents the use and development of directed therapies. Treatment options are oral agents (antibiotics, immunomodulators) and surgical excision. Historically, surgical management has been complicated by difficult closure and high recurrent rates. In the last 10 years, negative pressure wound therapy (NPWT) has proven to be a great adjunct for wound management as it provides the adequate conditions for wound healing, promotes granulation and helps to control infection. Here, we report a case of severe perineal HS treated with radical excision and NPWT as an adjunct. The patient only had a recurrence 3 years after primary treatment and was surgically treated for cosmetic reasons without any complications. Finally, we present a review of the relevant literature.

Hidrosadenitis supurativa (Parte I) / Hdradenitis suppurativa (Part I)

La hidradenitis supurativa es una entidad crónica, debilitante, que afecta las regiones axilares, perineo y submamarias. Se describe una prevalencia del 4 por ciento y su causa tiene que ver con oclusión folicular más que con un proceso de inflamación o de infección de las glándulas apocrinas. Su inicio es en la pubertad con nódulos inflamatorios recurrentes que se rompen espontáneamente o coalescen formando lesiones dérmicas y abscesos dérmicos profundos y dolorosos que dejan eventualmente cicatrices fibrosas, extensas y deformantes. Los tratamientos médicos clásicos o más recientes como inhibidores de FNT- alfa se pueden realizar en etapas tempranas pero la cirugía y últimamente el láser es lo que da mejor resultado en etapas más avanzadas. En esta primera parte abordamos el tema con su epidemióloga, etiología, clínica, diagnóstico y diagnóstico diferencial. En la parte II se revisará enfermedades asociadas, complicaciones y tratamiento.

Hidradenitis suppurativa is a chronic and often debilitating disease affecting primaraly the axillae, perineum, and inframammary regions. A prevalence of 4 percent has been estimated and its etiology includes follicular occlusion rather than an inflammatory and infectious process of the apocrine glands. It start usually in puberty with recurrent inflammatory nodules, that rupture spontaneously or coalesce forming painful and deep dermal abscesses followed eventually by extensive, fibrous and disfigured scars. Medical treatments are useful ininitial stages of the disease like alpha tumor necrosis factor inhibitors but surgery and recently lasers are the solution for better results in advanced stages. In this first section epidemiology, etiology, clinical picture, diagnosis and differential diagnosis are reviewed. In the second section associated diseases, complications and treatment will be revised.

Hidrosadenitis supurativa (Parte II) / Suppurative hidrosadenitis (Part II)

La Hidrosadenitis Supurativa (HS) es una enfermedad inflamatoria,folicular, crónica, recurrente y debilitante de la piel, caracterizadapor la presencia de abscesos y/o nódulos recurrentes, formación de fístulas y cicatrices. Las lesiones inflamatorias son dolorosas profundas y se localizan en zonas del cuerpo que poseen glándulas sudoríparas apocrinas, principalmente en la región axilar, inguinal y anogenital. Es una patología que presenta gran retraso en laconsulta y diagnóstico, ya que la mayoría de los casos leves sonreportados tardíamente. La resolución espontánea es poco probable y la discapacidad progresiva es comúnmente experimentada. Hay gran compromiso de la calidad de vida el que se correlaciona con la gravedad de la enfermedad. En esta segunda parte revisaremos las enfermedades asociadas a HS, complicaciones y alternativas terapéuticas de tipo médico y quirúrgico.

Hidradenitis suppurativa (Hs) is a chronic inflammatory, follicular,recurrent and debilitating disease characterized by painful and recurrent nodules forming extensive, fibrous anddisfigured scars. Inflammatory lesions are painful and affect primarily the axillae, inguinal and anogenital areas. Is a late disease in being diagnosed because of late consultation andreport. Spontaneous resolution is very rare and progressive disable is common. There is great quality of life involvementand related with the severity of the disease. In this second Part associated diseases, complications, and medical and surgery treatments are discussed.

Hidradenitis supurativa: evaluación por resonancia magnética / Hidradenitis suppurativa (hs), evaluation using mri

This corresponds to an inflammatory disease of the apocrine glands, distributed mainly in the axillary, perineal and perianal regions, clinically characterized by pain, swelling, and smelly discharge. Treatment is expensive, long and leaves large scars, generating economic and psychological problems for the patient. Magnetic resonance imaging (MRI) helps to confirm the diagnosis and determine its extension, which allows it to be distinguished from other entities and to choose the best treatment. To our knowledge, there is only one review of literature dedicated to the characteristics of this pathology using MRI, therefore we present an illustrated literature review in different patients treated at our center.

Corresponde a una enfermedad inflamatoria de las glándulas apocrinas, distribuidas principalmente a nivel axilar, perineal y perianal, caracterizado clínicamente por dolor, tumefacción y secreción mal oliente. Su tratamiento es costoso, largo y deja grandes cicatrices, generando problemas económicos y psicológicos para el paciente. La resonancia magnética (RM) ayuda a confirmar el diagnóstico y a determinar su extensión, lo que permite diferenciarlo de otras entidades y elegir el mejor tratamiento. A nuestro conocimiento, existe sólo una revisión de la literatura dedicada a las características de esta patología en resonancia magnética, por lo que presentamos una revisión de la literatura ilustrada en diferentes pacientes tratados en nuestro centro de atención.