Association of hidradenitis suppurativa with Crohn’s disease

Hidradenitis suppurativa(HS)is a chronic inflammatory skin disorder characterized by recurrent nodules,abscesses,and sinus tracts.Crohn’s disease(CD)is characterized by inflammation of the entire digestive tract and belongs to the group of inflammatory bowel diseases,and there are many extraintestinal manifestations,among which hidradenitis suppurativa is one of the rare extraintestinal manifestations.There appears to be a strong association between CD and HS based on clinical and histological similarities(sinus tract development,granulomatous inflammation,and scarring),intersections in pathogenesis(genetic loci,immune dysregulation mechanisms,and microbiome changes),and commonality in treatment.In this review,we summarize recent studies on the association between HS and CD.

Topical 5-aminolevulinic acid photodynamic therapy improved refractory acne conglobata and perifolliculitis capitis abscedens et suffodiens rather than hidradenitis suppurativa

Acne conglobata(AC),perifolliculitis capitis abscedens et suffodiens(PCAS)and hidradenitis suppurativa(HS)are uncommon refractory chronic,inflammatory,scarring diseases but cause serious damage to the quality of life.These three diseases are associated with follicular occlusion.Several studies indicated topical 5-aminolevulinic acid photodynamic therapy(ALA-PDT)improved follicular occlusion besides acne treatment.So we attempted to apply ALA-PDT to medicine resistant AC,PCAS and HS.Topical ALA-PDT was applied to 10 patients with AC,seven patients with PCAS and three patients with HS for more than three sessions.All the patients completed the dermatology life quality index(DLQI)questionnaire and were assessed for the efficacy at the baseline and on two weeks after each treatment.Adverse effects were recorded at each visit.The results showed 25.5%(5/20,two cases of AC and three cases of PCAS)of patients achieved excellent improvement after three sessions of PDT and another 60.0%(12/20,eight cases of AC and four cases of PCAS)of patients achieved good improvement.15.0%(3/20,three cases of HS)got poor response(<20%lesions clearance).Anotherfive cases(three cases of AC and two cases of PCAS)also achieved excellent response after 5–7 sessions of PDT.We also found that papular/nodular,cyst/abscess showed higher clearance rate than sinus/¯stula(88.5%,86.1%versus 11.1%).DLQI was reduced after three sessions of PDT in AC and PCAS patients rather than HS patients.5-ALA-PDT could improve refractory AC and PCAS but could not lead to improvement in late stage of HS.The e±cacy increased with more treatment sessions.

Surgical Management of Severe Hidradenitis Suppurativa in Women: Outcome and Quality of Life Assessment

Background: Hidradenitis suppurativa (HS) or Acne inversa sometimes referred to as Verneuil’s disease, is a rare and debilitating condition arising in the intertriginous folds that bear apocrine glands (axillae, groin, perineum, pubis, ano-genital area). It has a deleterious impact on the life of affected persons who tend to be more depressive and poorly socially and sexually connected because of pain, malodor and stigmatization. Methods: We conducted a retrospective study in Henri Mondor Hospital in Créteil, France, in order to assess the effectiveness of surgery as a radical treatment and also assess the factors related to recurrence and the surgical and sexual satisfaction in women operated for severe HS of perineum and genitalia. Results: Fifty-five women were included. We got a recurrence rate of 23.6%. 66.7% of recurrence occurred after localized excision. 83% of patients were satisfied with surgery results and would undergo a revision surgery or recommend it. The factors significantly associated with recurrence were overweight (p = 0.0046), location in groin (p = 0.0040), localized excision (p = 0.02), total excision (p = 0.009), healing via secondary intention (p = 0.001) and disability due to HS (p = 0.006). Assessment of sexual response thanks to the FSFI showed an alteration of all the domains of sexual response but the composite index of 18.51 was below the threshold to diagnose pathologic sexual condition.

Hidradenitis Suppurativa: An Exploration of Genetic Perturbations and Immune Dysregulation

Hidradenitis suppurativa(HS)is a chronic,inflammatory skin condition that poses a significant diagnostic and therapeutic challenge for clinicians,as the underlying etiology and pathogenesis remains unclear.The host of genetic mutations and immune dysfunction has been identified to be involved in the pathogenesis of HS during recent years.These genetic defects,including monogenetic mutations altering subunits ofγ-secretase,a protease that functions through Notch signaling to maintain skin appendages,promote epithelial stability,suppress/terminate innate immune responses(ie,Toll-receptors),further have the propensity to induce aberrant cytokine responses that create to a proinflammatory environment,consequently induce hyperkeratosis and promote expression of pro-inflammatory,locally destructive matrix metalloproteinases.Cytokine-driven inflammation propagates the disease state of HS and contributes to the formation of painful subcutaneous nodules,abscesses,and eventually,fistulas and draining sinus tracts.A closer look at genetic mutations linked to the disease may explain the immune perturbations seen in HS.An understanding of the immune cells and inflammatory markers expressed in affected individuals provides insight into disease pathogenesis and can help identify therapeutic targets.

Prevalence of Acne Inversa(Hidradenitis Suppurativa)in China:A Nationwide Cross-Sectional Epidemiological Study

Objective:This study was performed to estimate the prevalence of hidradenitis suppurativa(HS)in China and describe the demographic and clinical features of HS in the Chinese population.Methods:This multicenter clinic-based cross-sectional study was conducted in dermatology clinics within 19 hospitals located in 15 cities/provinces across China from September to October 2020.The prevalence of HS was calculated as the percentage of patients with HS among all visitors at participating clinics during the 1-month study period.HS was independently diagnosed by two certified dermatologists at each site.All visitors at participating clinics were screened,and all patients with HS were invited to participate in a questionnaire survey to elucidate the demographics and clinical features of HS in the Chinese population.Results:We identified 92 patients with HS among 274,742 visitors at participating clinics.The prevalence rate was 0.03349%or 33.49 per 100,000 population(95%confidence interval,26.64–40.32),and the female:male ratio was 1.0:4.7.Conclusion:The overall prevalence of HS in China was lower than that in Western and other Asian countries with a predominance of male patients.

Diagnosis and Treatment of Acne Inversa/Hidradenitis Suppurativa in China: An Expert Consensus Statement (2021 Version)

Acne inversa/hidradenitis suppurativa is a chronic,recurrent,inflammatory skin disease that affects the pilosebaceous units,causinfollicular occlusion.The etiology and pathogenesis of acne inversa/hidradenitis suppurativa involves internal and external factors such as genetic susceptibility,inflammation and immunity,microorganisms,obesity,and smoking.acne inversa/hidradenitis suppurativa is difficult to treat,and the current aim of treatment is to control the frequency and duration of disease flares and improve the quality of life.Treatment protocols for acne inversa/hidradenitis suppurativa should be selected based on the disease severity grade.Medical treatments include antibiotics,retinoids,biologics,immunosuppressive agents,and antiandrogen agents.Adjuvant treatments include surgery and laser/light therapies.This consensus aims to further standardize the diagnosis and treatment procedures of acne inversa/hidradenitis suppurativa in China to facilitate its diagnosis and treatment.

Mucinous adenocarcinoma of the buttock associated with hidradenitis:A case report

BACKGROUND Mucinous adenocarcinomas of the buttock are rare and have an uncertain etiology and natural course.They are usually related to chronic anal fistulas,hidradenitis suppurativa,or Crohn's disease.Here,we report a case of mucinous adenocarcinoma associated with hidradenitis and contradictory immunochemistry results.CASE SUMMARY A 62-year-old man complained of recurrent abscesses of the buttock for 3 years.He had several scars and nodules in bilateral buttocks,with purulent discharge.The skin lesions did not appear to originate from the anus.The patient was diagnosed with recurrent abscesses due to hidradenitis suppurativa at the first visit.He showed purulent and subsequent mucin discharge in the first operation and was diagnosed with mucinous adenocarcinoma.Several examinations were performed to determine disease origin and staging.There were no significant findings or evidence of anal fistulas.Hence,he underwent wide local excision and V-Y advancement flap in the second operation.The final diagnosis was mucinous adenocarcinoma without any evidence of anal fistulas.Additional immunochemistry test results were negative for cytokeratin(CK)7 and positive for CK20 and CDX2,with a colorectal origin.A pathologist suggested that the disease originated from a chronic anal fistula.The patient has remained free of recurrence for 24 mo.CONCLUSION Although the patient with mucinous adenocarcinoma showed an atypical course,immunochemistry helped detect the disease origin.

Novel Mutations in PSENEN Gene in Two Chinese Acne Inversa Families Manifested as Familial Multiple Comedones and Dowling-Degos Disease

Background： Acne inversa （AI）, also called hidradenitis suppurativa, is a chronic, inflammatory, recurrent skin disease of the hair follicle. Familial AI shows autosomal-dominant inheritance caused by mutations in the γ-secretase genes. This study was aimed to identify the specific mutations in the y-secretase genes in two Chinese families with AI. Methods： In this study, two Chinese families with AI were investigated. All the affected individuals in the two families mainly manifested with inultiple comedones, pitted scars, and a few inflammatory nodules on their face, neck, trunk, axilla, buttocks, upper arms, and thighs. Reticulate pigmentation in the flexures areas resembled Dowling-Degos disease clinically and pathologically. In addition, one of the affected individuals developed anal canal squamous cell carcinoma. Molecular mutation analysis of γ-secretase genes including PSENEN, PSENI, and NCSTN was performed by polymerase chain reaction and direct DNA sequencing. Results： Two novel mutations of PSENEN gene were identified, including a heterozygous missense mutation c. 194T〉G （p.L65R） and a splice site mutation c. 167-2A〉G. Conclusions： The identification of the two mutations could expand the spectrum of mutations in the γ-secretase genes underlying AI and provide valuable information for further study of genotype-phenotype correlations.

Reconstruction of the male external genitalia in diverse disease processes:Our reconstructive algorithm,techniques,and experience

Background:Male genital form and function may be rendered abnormal by a number of disease processes,with profound associated psychological and functional consequences.The aim of the study is to review our reconstructive experience with cases of genital loss or distortion due to nonmalignant diseases processes and atypical neoplasia.Materials and methods:A retrospective review of a prospectively maintained database was performed to identify reconstructive cases performed from 2018 to 2020 under the care of a single surgeon.Male patients 18 years or older with a disease diagnosis other than squamous cell carcinoma affecting genital form were included.Disease processes,patient factors,surgical techniques,and both functional and cosmetic outcomes were reviewed.Results:Fourteen cases were identified.The patients had a mean age of 52.2 years(range,21-72 years).Acquired buried penis was present in 8 patients.Etiology of genital abnormality included balanitis xerotica obliterans(n=6),excess skin loss at circumcision(n=2),self-injection of petroleum jelly to penile shaft(n=1),Fournier gangrene(n=1),hidradenitis suppurativa(n=1),extramammary Paget disease(n=1),idiopathic lymphoedema(n=1),and penoscrotal webbing(n=1).Reconstructive techniques performed included penile debridement/shaft skin release,scrotectomy,suprapubic apronectomy,and division of penoscrotal webbing,in combination with split-thickness skin grafting where required.A penile implant was inserted in one patient.Reconstructive planning,techniques,and outcomes are described.Conclusions:A variety of reconstructive techniques in andrology can be used to improve the aesthetic and functional outcomes of multiple disease processes affecting the male external genitalia.