Importance Hidradenitis suppurativa (HS) is relatively common, with the prevalence of 0.05% to 4.10%, yet many patients receive inadequate treatment. Objective To review the diagnosis, epidemiology, and treatment of HS with an emphasis on advances in the last 5 years. Evidence Review A literature search was conducted using PubMed, MEDLINE (Medical Subject Headings [MeSH]), and EMBASE to include recently published treatment studies (searched from September 1, 2011, to May 1, 2017). Reviews, guidelines, conference abstracts, and studies with less than 10 patients were excluded. Furthermore, internet searches for guidelines on hidradenitis suppurativa using Baidu, Bing, Google, and Qwant browsers were performed. Findings The diagnosis of HS is made by lesion morphology (nodules, abscesses, tunnels, and scars), location (axillae, inframammary folds, groin, perigenital, or perineal), and lesion progression (2 recurrences within 6 months or chronic or persistent lesions for ≥3 months). HS is more common than was previously thought based on epidemiological analysis (0.05%-4.10%). Disability from HS can be significant. Patients with HS may have significant comorbidities (eg, obesity, metabolic syndrome, diabetes, and arthritis) and increased all-cause mortality (incidence rate ratio, 1.35 [95% CI, 1.15-1.59]). Antibiotic treatment with combinations of clindamycin and rifampicin, or ertapenem followed by combination rifampicin, moxifloxacin, and metronidazole for 6 months is effective. Adalimumab is effective in a significant proportion of patients and treatment with IL-1 and IL-12 receptor subunit beta 1 (Rb1) antibodies may also be useful. Tissue-sparing surgical techniques and carbon dioxide laser treatments also are available, but the evidence on clinical outcomes with these approaches is limited. Conclusions and Relevance Hidradenitis suppurativa is more common than previously thought and may be treated by an array of pharmacological and surgical techniques. Hidradenitis suppurativa should be considered in the differential diagnosis of nodular lesions or sinus tracts present in the axillae, groin, perineal, and mammillary fold regions.

https://jamanetwork.com/journals/jama/articlepdf/2664466/jama_Saunte_2017_rv_170008.pdf

Hidradenitis Suppurativa: Advances in Diagnosis and Treatment

Hidradenitis suppurativa is a chronic inflammatory disorder affecting hair follicles, with profoundly negative impact on patient quality of life. Evidence informing ideal evaluation and management of patients with hidradenitis suppurativa is still sparse in many areas, but it has grown substantially in the last decade. Part I of this evidence-based guideline is presented to support health care practitioners as they select optimal management strategies, including diagnostic testing, comorbidity screening, and both complementary and procedural treatment options. Recommendations and evidence grading based on the evidence available at the time of the review are provided.

North American clinical management guidelines for hidradenitis suppurativa: A publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part I: Diagnosis, evaluation, and the use of complementary and procedural management.

This guideline has been initiated by the task force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology, including physicians from all relevant disciplines and patient organizations. It is a S3 consensus-based guideline that systematically reviewed the literature on mucous membrane pemphigoid (MMP) in the MEDLINE and EMBASE databases until June 2019, with no limitations on language. While the first part of this guideline addressed methodology, as well as epidemiology, terminology, aetiology, clinical presentation and outcome measures in MMP, the second part presents the diagnostics and management of MMP. MMP should be suspected in cases with predominant mucosal lesions. Direct immunofluorescence microscopy to detect tissue-bound IgG, IgA and/or complement C3, combined with serological testing for circulating autoantibodies are recommended. In most patients, serum autoantibodies are present only in low levels and in variable proportions, depending on the clinical sites involved. Circulating autoantibodies are determined by indirect IF assays using tissue substrates, or ELISA using different recombinant forms of the target antigens or immunoblotting using different substrates. The major target antigen in MMP is type XVII collagen (BP180), although in 10-25% of patients laminin 332 is recognized. In 25-30% of MMP patients with anti-laminin 332 reactivity, malignancies have been associated. As first-line treatment of mild/moderate MMP, dapsone, methotrexate or tetracyclines and/or topical corticosteroids are recommended. For severe MMP, dapsone and oral or intravenous cyclophosphamide and/or oral corticosteroids are recommended as first-line regimens. Additional recommendations are given, tailored to treatment of single-site MMP such as oral, ocular, laryngeal, oesophageal and genital MMP, as well as the diagnosis of ocular MMP. Treatment recommendations are limited by the complete lack of high-quality randomized controlled trials.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/jdv.17397

European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology – Part I

Christos C. Zouboulis, Falk G. Bechara, Klaus Fritz, Hjalmar Kurzen, Aikaterini I. Liakou, Wolfgang C. Marsch, Annett Milling, Alexander Nast, Maurizio Podda, Klaus M. Taube, Volker Wienert, Thomas Winkler (1) Klinik für Dermatologie, Venerologie und Allergologie/Immunologisches Zentrum, Städtisches Klinikum Dessau, Dessau (2) Klinik für Dermatologie, Venerologie und Allergologie, Ruhr-Universität Bochum, Bochum (3) Hautärzte und Laserzentrum Landau und Kandel, Landau (Pfalz)/Kandel; Universitätsklinik für Dermatologie, Inselspital Bern, Bern; Fachgebiet Dermatologie, Umweltmedizin und Gesundheitstheorie, Universität Osnabrück (4) Niedergelassener Dermatologe, Freising (5) Universitätsklinik und Poliklinik für Dermatologie und Venerologie, Martin-Luther-Universität Halle-Wittenberg, Halle (Saale) (6) Klinik für Dermatologie, Klinikum Frankfurt (Oder) GmbH, Frankfurt (Oder) (7) Abteilung für Evidenz-basierte Medizin, Klinik für Dermatologie, Venerologie und Allergologie, Charité-Universitätsmedizin Berlin, Berlin (8) Hautklinik, Klinikum Darmstadt (9) Früherer Leitlinie-Beauftragter, Aachen (10) Patientenvertreter, 1. Vorsitzender der Deutschen Interessengemeinschaft Akne inversa e.V., Ibbenbüren

S1‐Leitlinie zur Therapie der Hidradenitis suppurativa/Acne inversa* (ICD‐10 Ziffer: L73.2)

Prior studies have reported that hidradenitis suppurativa (HS) is accompanied by a myriad of physical and mental conditions. However, given the small sample sizes and the limited number of pre-selected comorbidities, these studies do not provide a complete picture of the comorbidity burden of HS in the USA. Moreover, the relationship between HS severity and comorbidity burden has yet to be characterized. Using a large US claims database, we estimated the comorbidity burden associated with HS, stratified by disease severity. A retrospective matched cohort design was used. Patients with HS were classified into two severity cohorts (milder and more severe) using an empirical algorithm based on treatments received. The comorbidity burden was compared between each HS cohort and their matched HS-free cohort, and between patients with milder vs. those with more severe forms of HS. Several physical and mental comorbidities were found to be more prevalent in both cohorts of patients with milder and more severe forms of HS than in their matched HS-free cohorts. The comorbidity burden also increased greatly as the disease progressed to more severe forms. The results of this study highlight the complexity of the comorbidity burden of HS patients and the need for a multidisciplinary approach to optimize the management of HS and its numerous associated comorbidities. AbbVie, Inc.

/pdf/the-comorbidity-burden-of-hidradenitis-suppurativa-in-the-3na5yvwwh6.pdf

The Comorbidity Burden of Hidradenitis Suppurativa in the United States: A Claims Data Analysis

Mucous membrane pemphigoid is a rare blistering autoimmune disorder that can lead to significant disability. Recently, the monoclonal anti-CD20 antibody rituximab has shown efficacy in pemphigus and pemphigoid, but the data for pemphigoid are relatively sparse. The authors report a series of four patients with severe cicatrical pemphigoid treated with rituximab. All of these patients responded and two of them had a complete remission. Rituximab is a second-line treatment for severe and recalcitrant cicatrical pemphigoid.

Monoclonal anti-CD20 Antibody Therapy in Cicatrical Pemphigoid with Oral and Hypopharyngeal Involvement and Related Conditions.

Background

Severe anogenital acne inversa (AI) is a debilitating chronic inflammatory disease with a major negative effect on quality of life.



Objectives

To evaluate the role of surgery in the treatment of severe anogenital AI.



Methods

We analyzed the records of patients with anogenital AI from 2000 to 2010. Assessment was done using the Hidradenitis Suppurativa Lesion, Area, and Severity Index (HS-LASI), pain visual analogue scale, physician global assessment (PhGA), and patient global assessment (PaGA). Comorbidities and adverse events were analyzed.



Results

Sixty-seven patients with a Hurley score of 3 were identified. Mean follow-up was 56.9 ± 41.3 months. A number of comorbidities were observed. After surgery, mean pain scores decreased from 6.3 ± 1.5 to 0.8 ± 0.7, PhGA improved from 6.8 ± 1.2 to 0.9 ± 0.6, PaGA improved from 7.3 ± 1.2 to 1.1 ± 0.5, and HS-LASI decreased from 41.8 ± 21.3 to 2.4 ± 2.8. Adverse reactions were seen in 10.4%. The total relapse rate was 6% (5 patients) with only one case with healing by secondary intention (2% of 49 patients).



Conclusions

Surgery is the cornerstone of treatment for advanced AI. Healing by secondary intention results in significant reduction of complaints and achieves satisfying body contouring.

Management of severe anogenital acne inversa (hidradenitis suppurativa).

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Monoclonal anti-CD20 Antibody Therapy in Cicatrical Pemphigoid with Oral and Hypopharyngeal Involvement and Related Conditions.

Management of severe anogenital acne inversa (hidradenitis suppurativa).