Hidradenitis supurativa glútea y perineal: diagnóstico y manejo terapéutico / Gluteal and perineal hidradenitis suppurativa: diagnosis and therapeutic management / Hidradenite suppurativa glútea e perineal: diagnóstico e manejo terapêutico

La hidradenitis supurativa (HS) es una enfermedad inflamatoria crónica de la piel que afecta a las glándulas sudoríparas apocrinas y causa nódulos inflamatorios, abscesos y fistulas dolorosas en áreas como las axilas, la ingle y los glúteos. Su impacto en la calidad de vida de los pacientes es significativo. La HS afecta al 1- 4% de la población mundial y se asocia con factores como el sobrepeso, el tabaquismo y antecedentes familiares. Su patogenia es compleja, involucrando inflamación y disfunción inmunológica. Su diagnóstico y tratamiento son desafiantes, especialmente en casos graves. El diagnóstico se basa en la presentación clínica, que a menudo se confunde con otras afecciones cutáneas. Se clasifica en etapas de Hurley según la gravedad. El tratamiento se centra en controlar los síntomas y prevenir recurrencias. Incluye educación del paciente, tratamiento médico y cirugía. La elección de la terapia antibiótica depende de la severidad y las cepas bacterianas presentes. Para casos graves, se investigan terapias biológicas. La cirugía, como la escisión simple o con injertos de piel, es efectiva en el control de la enfermedad. Presentamos un caso clínico de un paciente con HS, revisamos la epidemiología, etiopatogenia y su diagnóstico acompañado de las opciones terapéuticas existentes

A hidradenite supurativa (HS) é uma doença inflamatoria crônica da pele que afeta as glândulas sudoríparas apócrinas e causa nódulos inflamatórios, abscessos e fístulas dolorosas em áreas como axilas, virilha e nádegas. Seu impacto naqualidade de vida dos pacientes é significativo. A EH afeta de 1 a 4% da população mundial e está associada a fatores como excesso de peso, tabagismo e histórico familiar. Suapatogênese é complexa, envolvendoinflamação e disfunçãoimunológica, sendoseu diagnóstico e tratamento desafiadores, principalmente nos casos graves. O diagnóstico é baseadonaapresentação clínica, que muitasvezes é confundida comoutrasdoenças da pele. É classificado em estágios de Hurley com base nagravidade. O tratamento se concentra no controle dos sintomas e naprevenção de recorrências. Incluieducação do paciente, tratamento médico e cirurgia. A escolha da antibioticoterapia depende da gravidade e das cepas bacterianas presentes. Para casos graves, terapias biológicas são investigadas. A cirurgia, como excisão simples ouenxertos de pele, é eficaz no controle da doença. Apresentamosum caso clínico de umdoentecom HS, revisamos a epidemiologia, a etiopatogenia e o seu diagnóstico acompanhado das opçõesterapêuticas existentes.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that affects the apocrine sweat glands and causes inflammatory nodules, abscesses, and painful fistulas in areas such as the armpits, groin, and buttocks. Its impact on patients' quality of life is significant. HS affects 1-4% of the world's population and is associated with factors such as overweight, smoking, and family history. Its pathogenesis is complex, involving inflammation and immune dysfunction. Its diagnosis and treatment are challenging, especially in severe cases. Diagnosis is based on clinical presentation, which is often confused with other skin conditions. It is classified into Hurley stages based on severity. Treatment focuses on controlling symptoms and preventing recurrences. Includes patient education, medical treatment and surgery. The choice of antibiotic therapy depends on the severity and the bacterial strains present. For severe cases, biological therapies are investigated. Surgery, such as simple excision or skin grafts, is effective in controlling the disease. We present a clinical case of a patient with HS, we review the epidemiology, etiopathogenesis and its diagnosis accompanied by the existing therapeutic options.

Modification of Parks Classification of Cryptoglandular Anal Fistula.

BACKGROUND: The Parks classification has been used for the classification of anal fistula for several years, but it does not allow for risk factors for failure after surgery. OBJECTIVE: This study aimed to describe a modification of the Parks classification of anal fistula and examine its predictive validity in the assessment of the outcome of anal fistula in terms of failure of healing and fecal incontinence. DESIGN: This is a retrospective review of a prospective database. SETTING: This study was conducted in the Colorectal Surgery Unit, Mansoura University Hospitals. PATIENTS: Adult patients with anal fistula who underwent surgery were included. INTERVENTIONS: Five risk factors for failure after fistula surgery were identified from the literature and were examined by multivariate analysis of our patients. Four risk factors proved to be significant independent predictors of failure: secondary extensions, horseshoe fistula, previous fistula surgery, and anterior anal fistula in women. We modified the Parks classification by dividing the transsphincteric type into high and low and by grouping supra- and extrasphincteric anal fistulas into 1 group. The first 3 stages were subdivided according to the absence or presence of predictors of failure. MAIN OUTCOME MEASURES: The primary outcome measured was the validity of the modified Parks classification with regard to the rates of failure and fecal incontinence after surgical treatment of each stage of anal fistula. RESULTS: A total of 665 patients with cryptoglandular anal fistula were included. Failure rates increased from 2.3% (95% CI, 0.9%-4.7%), to 17.4% (95% CI, 10.8%-25.9%), 19.5% (95% CI, 15%-24.6%), and 30.7% (95% CI, 9.1%-61.4%) across the 4 stages. The area under the receiver operating characteristic curve was 0.90 (95% CI, 0.85-0.94) indicating the strong discriminative ability of the final multivariable predictive model. The increase in failure and incontinence rates across the fistula stages was significant. LIMITATIONS: This is a retrospective, single-center study. CONCLUSION: Inclusion of predictors of poor outcome into the modified classification helped differentiate simple and complex fistulas within each stage and between the different stages, which can help in assessment and decision making for anal fistula. See Video Abstract at http://links.lww.com/DCR/B441. MODIFICACIN DE LA CLASIFICACIN DE PARKS DE LA FSTULA ANAL CRIPTOGLANDULAR: ANTECEDENTES:La clasificación de Parks se ha utilizado para la clasificación de la fístula anal durante varios años, sin embargo, no tuvo en cuenta los factores de riesgo de fracaso después de la cirugía.OBJETIVO:Describir una modificación de la clasificación de Parks de fístula anal y examinar su validez predictiva en la evaluación de los resultados de la fístula anal en términos de fracaso de la cicatrización e incontinencia fecal.DISEÑO:Revisión retrospectiva de la base de datos prospectiva.AJUSTE:Unidad de Cirugía Colorrectal, Hospital Universitario de Mansoura.PACIENTES:Pacientes adultos con fístula anal intervenidos quirúgicamente.INTERVENCIONES:Se identificaron cinco factores de riesgo de fracaso después de la cirugía de fístula de la literatura y se examinaron mediante análisis multivariante de nuestros pacientes. Cuatro factores de riesgo demostraron ser importantes predictores independientes de fracaso: extensiones secundarias, fístula en herradura, cirugía de fístula previa y fístula anal anterior en mujeres. Modificamos la clasificación de Parks dividiendo el tipo transesfinteriano en alto y bajo y agrupando la fístula anal supraesfinteriana y extraesfinteriana en un grupo. Las tres primeras etapas se subdividieron según la ausencia o presencia de predictores de fracaso.PRINCIPALES MEDIDAS DE RESULTADO:Validez de la clasificación de Parks modificada con respecto a las tasas de fracaso e incontinencia fecal después del tratamiento quirúrgico de cada etapa de la fístula anal.RESULTADOS:Se incluyeron 665 pacientes con fístula anal criptoglandular. Las tasas de fracaso aumentaron del 2,3% (IC del 95%: 0,9-4,7%), al 17,4% (IC del 95%: 10,8 al 25,9%), 19,5% (IC del 95%: 15-24,6%) y 30,7% (95% IC: 9,1- 61,4%) en las cuatro etapas. El área bajo la curva característica operativa del receptor fue 0,90 (IC del 95%: 0,85-0,94), lo que indica una fuerte capacidad discriminativa del modelo predictivo multivariable final. El aumento en las tasas de fracaso e incontinencia en las etapas de la fístula fue significativo.LIMITACIONES:Estudio retrospectivo, unicéntrico.CONCLUSIÓN:La inclusión de predictores de mal resultado en la clasificación modificada ayudó a diferenciar las fístulas simples y complejas dentro de cada etapa y entre las diferentes etapas, lo que puede ayudar en la evaluación y toma de decisiones para la fístula anal. Consulte Video Resumen en http://links.lww.com/DCR/B441.

Trophobiotic organ and perianal structures in aphid genus Stomaphis WALKER (Aphididae, Lachninae).

Stomaphis Walker is an obligatorily myrmecophilous, tree-trunk feeding aphid genus, having many peculiar features in its morphology. One poorly studied trait is its perianal anatomy, with suspected existence of trophobiotic organ and intriguing tubercles near abdominal segment VIII. Tubercles on rear end of abdomen in Stomaphis were considered to be marginal tubercles, structure typical of aphids. Only after their investigation under scanning electron microscope they revealed to possess a very rough surface, consisting of many protuberant, sclerotic scales with finger-like processes. Analysis and comparison with other aphids suggests, that these structures - perianal tubercles - may be considered remnants of abdominal sternite IX, atrophied in aphids. Due to their similarity to the surface of cauda of non myrmecophilous aphids, they are suspected to serve as sort of auxiliary caudas, protecting from sticky secretions of aphids e. g. honeydew or mucus while giving birth or ovipositing in confined space. A very well developed trophobiotic organ in this genus has been shown to exist for the first time.

Año 1938: primer reconocimiento de la enfermedad perianal como parte de la enfermedad de Crohn / Year 1938: First recognition of the perianal disease as part of Crohn's disease

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Vedolizumab for perianal Crohn's disease: a multicentre cohort study in 151 patients.

BACKGROUND: The management of Crohn's disease patients with perianal lesions and anti-TNF failure is challenging. AIMS: To assess the effectiveness of vedolizumab in perianal Crohn's disease and the predictors of success in a real-life cohort. METHODS: We conducted a nationwide multicentre cohort study in patients with perianal Crohn's disease who received vedolizumab. In patients with active perianal Crohn's disease, the success of vedolizumab was defined by clinical success (no draining fistula at clinical examination and no anal ulcers for primary lesions) at 6 months without medical or surgical treatment for perianal Crohn's disease. Logistic regression analyses were performed to identify predictors of success. In patients with inactive perianal Crohn's disease, recurrence was defined by the occurrence of lesions and/or the need for medical or surgical treatments. RESULTS: One hundred and fifty-one patients were included. Among them 102 patients had active perianal disease, 33 (32.4%) males, mean age 39.8 years, mean Crohn's disease duration 14.6 years; 101 (99%) had received at least one anti-TNF. The median follow-up time was 52 weeks. Sixty-eight per cent of patients discontinued therapy after a median time of 33 weeks. Vedolizumab success was reached in 23/102 (22.5%). Among patients with setons at initiation, 9/61(15%) had a successful removal. In multivariable analysis, factors associated with success were the number of prior biologic agents (≥3, odds ratio, OR: 0.20, 95% CI 0.04-0.98) and no antibiotics at initiation (OR: 4.76, 95% CI 1.25-18.19). In 49 patients with inactive perianal Crohn's disease, perianal disease recurred in 15/49 (30.6%), 11/49 (22.4%) needed dedicated treatments. Median time to recurrence was 22 weeks. CONCLUSIONS: We identified a low rate of success of vedolizumab in patients with active perianal Crohn's disease, and nearly one third of patients with inactive perianal Crohn's disease had perianal recurrence. Further evaluation is warranted in prospective studies.

Sarcoma de Kaposi perianal / Perianal Kaposi sarcoma

Se presenta una imagen de un tumor perianal en un paciente masculino, de 27 años de edad, con antecedentes patológicos personales de infección por el virus de la inmunodeficiencia humana (VIH) de siete años de evolución. Durante la anamnesis refirió que desde seis meses anteriores a la consulta presentaba sensación de masa perianal y constipación. Mediante la biopsia se corroboró que se trataba de un sarcoma de Kaposi (Fig. 1) de aproximadamente 20 cm de superficie irregular, friable, con abundantes secreciones mucosas(AU)

Cytological differentiation between benign and malignant perianal gland proliferative lesions in dogs: a preliminary study.

OBJECTIVES: To evaluate the diagnostic utility of individual cytological criteria and their best combination to differentiate benign from malignant perianal gland proliferative lesions in dogs. MATERIALS AND METHODS: Retrospective study of cytological samples of canine perianal gland proliferative lesions that had subsequent histopathological confirmation. RESULTS: Seventy-seven perianal gland nodules from 56 dogs were included. Histologically, lesions were diagnosed as hyperplasia (n=2), adenoma (n=53), epithelioma (n=6) and carcinoma (n=16). Of the 28 cytological criteria assessed, 13 showed promise for distinguishing benign from malignant lesions. A diagnostic algorithm with an 87% accuracy (sensitivity, 90.9%; specificity, 85.4%) was developed from these data. CLINICAL SIGNIFICANCE: Cytological evaluation can provide useful information for presurgical differentiation between benign and malignant hepatoid gland proliferative lesions. The proposed algorithm must be validated and tested for reproducibility in further, preferably larger, series of cases.

Dissecting Allo-Sensitization After Local Administration of Human Allogeneic Adipose Mesenchymal Stem Cells in Perianal Fistulas of Crohn's Disease Patients.

Adipose mesenchymal stem cells (ASC) are considered minimally immunogenic. This is due to the low expression of human leukocyte antigens I (HLA-I), lack of HLA-II expression and low expression of co-stimulatory molecules such as CD40 and CD80. The low rate of observed immunological rejection as well as the immunomodulatory qualities, position ASC as a promising cell-based therapy for the treatment of a variety of inflammatory indications. Yet, few studies have addressed relevant aspects of immunogenicity such as ASC donor-to-patient HLA histocompatibility or assessment of immune response triggered by ASC administration, particularly in the cases of presensitization. The present study aims to assess allo-immune responses in a cohort of Crohn's disease patients administered with allogeneic ASC (darvadstrocel formerly Cx601) for the treatment of complex perianal fistulas. We identified donor-specific antibodies (DSA) generation in a proportion of patients and observed that patients showing preexisting immunity were prone to generating DSA after allogeneic therapy. Noteworthy, naïve patients generating DSA at week 12 (W12) showed a significant reduction in DSA titer at week 52 (W52), whereas DSA titer was reduced in pre-sensitized patients only with no specificities against the donor administered. Remarkably, we did not observe any correlation of DSA generation with ASC therapeutic efficacy. In vitro complement-dependent cytotoxicity (CDC) studies have revealed limited cytotoxic levels based upon HLA-I expression and binding capacity even in pro-inflammatory conditions. We sought to identify CDC coping mechanisms contributing to the limited cytotoxic killing observed in ASC in vitro. We found that ASC express membrane-bound complement regulatory proteins (mCRPs) CD55, CD46, and CD59 at basal levels, with CD46 more actively expressed in pro-inflammatory conditions. We demonstrated that CD46 is a main driver of CDC signaling; its depletion significantly enhances sensitivity of ASC to CDC. In summary, despite relatively high clearance, DSA generation may represent a major challenge for allogeneic cell therapy management. Sensitization may be a significant concern when evaluating re-treatment or multi-donor trials. It is still unknown whether DSA generation could potentially be the consequence of donor-to-patient interaction and, therefore, subsequently link to efficacy or biological activity. Lastly, we propose that CDC modulators such as CD46 could be used to ultimately link CDC specificity with allogeneic cell therapy efficacy.

Dermatitis perianal y test de diagnóstico rápido / Perianal dermatitis and rapid diagnostic test

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Lesiones ulcerosas en sacabocados en la región del pañal / Ulcerous damage in punched out lesions around the diaper area

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Enfermedad de Paget perianal / Perianal Paget disease

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Canine Perianal Fistulas: Clinical Presentation, Pathogenesis, and Management.

Canine perianal fistulas are painful sinus tracts and ulcers that spontaneously develop in the skin around the anus. Middle-aged German shepherd dogs are most commonly affected and may have a genetic susceptibility. Although the disease was once believed related to conformational factors and primarily managed surgically, an immune-mediated pathogenesis is now recognized. Long-term medical management with immunomodulatory agents has become standard of care for canine perianal fistulas. Perianal fistulas can be debilitating and have a negative impact on quality of life of dogs and owners. Accurate diagnosis and aggressive medical therapy are key to successful management of canine perianal fistulas.

A 10-Year Follow-up Study of the Natural History of Perianal Crohn's Disease in a Danish Population-Based Inception Cohort.

BACKGROUND: Perianal complications in patients with Crohn's disease are common and have a negative impact on the patients' quality of life. Data about the long-term disease course of perianal Crohn's disease in the era of biological treatment are limited. In this population-based cohort study, we sought to investigate the occurrence, clinical risk factors, and disease course of perianal disease. METHODS: A total of 213 Crohn's disease patients were included in a prospective population-based inception cohort. Data were retrieved from medical records and national health administrative databases. Perianal disease was defined as a perianal fistula and/or abscess. Associations between outcomes and covariates were analyzed by Cox regression analysis. RESULTS: A total of 48 (22.5%) patients developed perianal disease after 10 years. Colonic disease location (hazard ratio [HR], 1.99; 95% confidence interval [CI], 1.01-3.92) and penetrating behavior (HR, 5.65; 95% CI, 2.65-12.03) were associated with the development of perianal disease. The cumulative risk of undergoing abdominal surgery was 51% after 10 years. Patients with perianal disease had a higher rate of resection (HR, 3.92; 95% CI, 1.86-8.67) and hospitalization (HR, 1.01; 95% CI, 1.00-1.01). There was no significant difference in the rate of sick leave, unemployment, or disability pension between patients with and without perianal disease. CONCLUSIONS: Patients with perianal disease carry a higher risk of surgery and hospitalization, and this suggests a more severe disease course and poorer prognosis among these patients, even in the era of biological treatment. These findings underline the importance of optimizing treatment strategies for patients with perianal disease.

Basal cell enumeration does not predict malignancy in canine perianal gland tumor cytology.

BACKGROUND: Perianal (hepatoid) gland tumors are common in dogs, and the distinction between the benign and malignant forms is clinically important. Cytology of these tumors typically contains many large hepatoid cells and fewer small basal cells. OBJECTIVES: The objective of this study was to determine whether the proportion of the smaller basaloid reserve cells in cytologic samples from perianal tumors correlates with malignancy. METHODS: Eighty-three cases of cytologically diagnosed perianal gland tumors with corresponding histopathologic sections were identified from two separate institutions and included six (7.2%) malignant tumors and 77 (92.8%) benign tumors. The proportion of basal cells from each sample was evaluated. RESULTS: No difference between these groups was found, although the study was sufficiently powered to detect an approximately 1.5-fold change in basal cell proportion. CONCLUSIONS: This report found no evidence that the proportion of basal cells in canine perianal tumor cytology is an indication of the potential for malignancy. We, therefore, do not recommend citing this feature in cytologic reports or when communicating with clinicians.

Repercusión diagnóstica y terapéutica del estudio ecográfico en los trayectos fistulosos perianales en pacientes con sospecha de hidradenitis supurativa / Diagnostic and Therapeutic Implications of Ultrasound Imaging of Perianal Fistulous Tracts in Patients with Suspected Hidradenitis Suppurativa

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Agentes anti-factor de necrosis tumoral en enfermedad de Crohn y colitis ulcerosa: más allá de la enfermedad luminal / Anti-tumor necrosis factor agents in Crohn's disease and ulcerative colitis: Beyond luminal disease

Los agentes anti-factor de necrosis tumoral (anti-TNF) son fármacos de uso común en los pacientes con enfermedad inflamatoria crónica intestinal (EICI) y han demostrado ser efectivos en inducción y mantenimiento en enfermedad de Crohn y colitis ulcerosa, así como en pacientes con afectación fistulizante perianal. Sin embargo, la evidencia relativa al uso de estos fármacos en otros escenarios dentro de EICI es menos sólida. Es el caso de la enfermedad de Crohn con afectación estenosante, penetrante o perianal no fistulizante, de las manifestaciones extraintestinales de la EICI y de las complicaciones del reservorio ileoanal. El objetivo de esta revisión fue realizar un análisis de la literatura disponible y determinar el papel de los anti-TNF en la práctica clínica en pacientes afectos por estas complicaciones

Anti-tumor necrosis factor agents (anti-TNF) drugs are commonly used in patients with inflammatory bowel disease (IBD) and have proven effective in both induction and maintenance therapy in luminal Crohn's disease and ulcerative colitis. Their efficacy has also been proven in fistulising perianal Crohn's disease. However, the evidence in other scenarios, such as stricturing, penetrating and non-fistulising perianal Crohn's disease, extraintestinal IBD manifestations and ileoanal reservoir complications, is not as robust. The aim of this review was to perform an analysis of the available literature and to determine the role of anti-TNF drugs in common clinical practice in patients affected by these complications