Does SAPHO syndrome exist in dermatology?

In the late 1960s, palmoplantar pustulosis (PPP) with sternocostoclavicular arthropathy was first described in Japan, predominantly affecting women in the perimenopausal age. In the 1970s, the chronic non-bacterial osteomyelitis and chronic recurrent multifocal osteomyelitis were initially observed in paediatric patients with approximately 70% girls. Acne fulminans accompanied by polyarthralgia have been observed since early 1970s, which almost exclusively occurs in adolescent boys. Report on spondyloarthropathy associated with hidradenitis suppurativa can be traced back to 1982. The SAPHO syndrome was coined in 1987 to lump together synovitis, acne, pustulosis, hyperostosis and osteitis to conceptualize a group of inflammatory osteocutaneous diseases of unclear etiopathogenesis and ill-defined associations spanning disparate age and gender groups. From historical view, Sasaki syndrome is proposed to replace SAPHO syndrome to represent PPP with sternocostoclavicular arthropathy in the absence of other skin manifestations. Hidradenitis suppurativa is folliculitis in pathogenesis and no longer classified as acne. PPP accompanied by psoriasis vulgaris is more likely psoriasis pustulosa palmoplantaris in dermatological aspect, and the associated arthritis is part of psoriatic arthropathy. Pathophysiology of these disorders is incompletely understood. To echo the advancement of high-throughput sequencing, splitting but not lumping of clinical findings would be a better strategy to decipher these multigenic complex inflammatory disorders.

Plastic Surgical Management of Hidradenitis Suppurativa.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe the pathogenesis of hidradenitis suppurativa. 2. Discuss perioperative multimodal therapy of hidradenitis suppurativa, including medical optimization. 3. Determine an appropriate surgical plan with excision and reconstruction based on hidradenitis suppurativa severity, size, and anatomical location. SUMMARY: Successful treatment of hidradenitis suppurativa requires a multidisciplinary team approach and multimodal therapy.

Proceeding report of the Fourth Symposium on Hidradenitis Suppurativa Advances 2019.

The Fourth Annual Symposium on Hidradenitis Suppurativa (SHSA) took place on November 1-3, 2019, at the Westin Book Cadillac Hotel in Detroit, Michigan. This symposium was a joint meeting of the US Hidradenitis Suppurativa Foundation and the Canadian Hidradenitis Suppurativa Foundation. This cross-disciplinary meeting with experts from around the world was an opportunity to discuss the most recent advances in the study of hidradenitis suppurativa (HS) pathogenesis, clinical trials, classification, scoring systems, complementary/alternative medical treatments, diet, pain management, surgical and laser treatment, and ultrasonographic assessment. A special preconference workshop was held on the use of neodymium-doped yttrium-aluminum-garnet laser hair reduction, sinus tract deroofing, and carbon dioxide laser excision with ultrasonographic mapping and tumescent anesthesia for the treatment of HS. The focused workshops on establishing an HS clinic, setting up an HS support group, the Hidradenitis Suppurativa Prospective Observational Registry and Biospecimen Repository, and wound care were held during the meeting. A special program called HS Ambassadors was established for patients who may have questions about the conference presentations, and in addition, a meet and greet for patients and HS Ambassadors was arranged. To facilitate networking between those early in their careers and clinical and research experts, a mentoring reception was held.

Hidradenitis Suppurativa: Proposal of Classification in Two Endotypes with Two-Step Cluster Analysis.

INTRODUCTION: Hidradenitis suppurativa (HS) is a chronic, inflammatory, recurrent disorder of the pilosebaceous unit. Currently, several attempts have been made to classify this disease according to its pathogenesis and clinical manifestations. We attempted at classifying 103 patients using two-step cluster analysis. METHODS: The final model included body mass index, C-reactive protein (CRP), and serum concentrations of IL-1, IL-6, IL-17, and IL-10 as continuous variables, and sex, later/early onset, anterior/posterior lesion sites, presence/absence of sinus tracts, nodules and abscesses, positive/negative history of pilonidal sinus, and presence/absence of mutations in gamma-secretase subunits (APH1A, APH1B, MEFV, NCSTN, PSEN1, PSEN2, PSENEN, PSTPIP1) as qualitative variables. RESULTS: The resultant model defined two groupings or clusters: cluster 1 (64.9% of patients) characterized by nonobese males, with nodular lesions in posterior sites, early-onset HS, higher IL-10, presence of gamma-secretase mutations, and history of pilonidal sinus; and cluster 2 (35.1% of patients) characterized by obese females or males, with lesions in anterior sites, more presence of sinus tracts and abscesses and less nodules, later-onset HS, and higher concentrations of IL-1, CRP, IL-17, and IL-6. Severity measures (Hurley, HS-PGA, and IHS4) and tobacco use were discarded because the analysis found them to be less relevant for clustering. CONCLUSION: Our resultant model confirms the clinical impression that HS is a disease spectrum with two pathogenic poles defining two clusters or endotypes. The probability of having severe disease was equally distributed in the two clusters. The variable with the highest predictive value for clustering was involvement of typical anterior sites (axillae, submammary) or atypical posterior sites (back, gluteal). Serum concentrations of interleukins, tobacco use, and sex had a lower predictive power for clustering.

Characterization of Hidradenitis Suppurativa Phenotypes: A Multidimensional Latent Class Analysis of the National Italian Registry IRHIS.

In spite of the large heterogeneity, limited data exist on hidradenitis suppurativa (HS) phenotypes. To identify the HS phenotypes that best explain the disease heterogeneity, a cross-sectional study using latent class (LC) analysis was conducted on a cohort of patients examined at 17 dermatological centers participating in the Italian Registry of Hidradenitis Suppurativa and being enrolled between January 2015 and January 2020. Overall, 965 patients aged 32.0 ± 12.4 years (mean ± SD) were evaluated. A three-class model in LC analysis best fitted the data. Patients in LC1 (20.1%) were females, mostly obese, with a high probability of axillary‒groin (0.85) and mammary (0.59) lesions and the highest HS severity. Patients in LC2 (29.6%) were nonobese males, with moderate disease severity; with a high probability of gluteal (0.50) and genital (0.17) lesions, besides axillary‒groin involvement; and with acne and pilonidal cysts. Patients in LC3 (50%) were nonobese females with a milder disease mostly limited to axillary (0.52) and groin (0.66) areas. The stratification of patients with HS into a severe axillary‒mammary‒groin phenotype with predominantly anterior body involvement in females, an axillary‒gluteal‒groin phenotype of intermediate severity mainly affecting males in the posterior body areas, and an axillary‒groin phenotype with mildest clinical symptoms and limited skin involvement may help in optimizing HS management.

Proposed Definitions of Typical Lesions in Hidradenitis Suppurativa.

BACKGROUND: Although not rare, hidradenitis suppurativa (HS) is often under-recognized by physicians. The diagnosis of HS is clinical via the recognition of lesions typical of the disease, but universally accepted definitions of these latter are currently lacking, which means that certain severity scores employed for HS classification/management are used differently by different physicians. Our aim was to develop a set of descriptive definitions and associated images of HS lesions, in order to enable doctors to better recognize and evaluate the disease. METHODS: MEDLINE-available literature and dermatological textbooks on HS morphology were retrieved (January 1996 to February 2016). A preliminary set of definitions of HS typical lesions was created, including 10 terms. Each term was associated with a pathophysiological classification and an image. This preliminary set was shown during the 5th Conference of the European HS Foundation (EHSF). The physicians attending the event were invited to vote on each term and make comments via a voting sheet. RESULTS: A total of 81 physicians answered the questionnaire. Their agreement/disagreement rates and comments were used to obtain a revised set of definitions and images. Pathophysiological classifications were dropped. CONCLUSION: A user-friendly set of definitions/images of HS typical lesions was proposed and will need to be validated by further studies. This set could ultimately serve as a tool to better recognize, score, and assess treatment efficacy.

Characterization of comorbid conditions burdening hidradenitis suppurativa: a multicentric observational study.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, disabling, inflammatory skin disorder that primarily affects the hair follicle localized at the apocrine-gland-bearing areas of the body, including axillary, inguinal, buttocks, and anogenital areas, and it may be associated with a wide array of comorbid conditions. This study aimed to described comorbid conditions affecting HS patients and to detect any correlation with disease severity. METHODS: Analyzing clinic database, we included all charts of patients visited at the HS outpatient clinic of three University Dermatologic Departments in order to describe demographic data, anthropometric measures, disease features, personal habits, clinical history, and presence of comorbidities. RESULTS: Two hundred thirty-four patients, mostly females (62%), were enrolled in this study. Based on Hurley staging classification 41% of patients were classified as Hurley Stage I, 43.0% as Hurley II, and 16% Hurley III, with a mean mSartorius Score value of 24.7 (SD: ±19.39) and a mean AISI score value of 12.5 (SD: ±11.93). The most frequently observed comorbidities were: obesity (26.1%), polycystic ovary syndrome (PCOS) (13.8% of the overall study population and 22.3% of females), hypertension (11.9%), dyslipidemia (9.9%), type II diabetes (9.5%), thyroid disorders (9.1%), nervous system disorders (7.1%), acne (6.7%), metabolic syndrome (4.4%), and Crohn's disease (3.6%). Obesity represented a key-comorbid condition increasing the likelihood of having more severe HS and PCOS (odds ratio 3.35 and 3.74, respectively). CONCLUSIONS: HS is associated with a variety of comorbid conditions that should be considered to perform targeted routine screening and to improve HS management.

Guía clínica chilena para el manejo de hidradenitis supurativa / Chilean clinical guidelines for the management of hidradenitis suppurativa

La hidradenitis supurativa es una enfermedad inflamatoria crónica del folículo piloso que se caracteriza por la aparición recurrente de lesiones inflamatorias dolorosas y profundas predominantemente en pliegues. Debido a sus secuelas físicas y en la calidad de vida, debemos estar familiarizados con esta enfermedad, a fin de poder realizar un diagnóstico oportuno e implementar un tratamiento precoz. Esta guía clínica, elaborada por el grupo de trabajo de hidradenitis supurativa de la Sociedad Chilena de Dermatología y Venereología (SOCHIDERM), revisa su definición, epidemiología, fisiopatogenia, factores de riesgo, comorbilidades, impacto psicoemocional, presentación clínica, diagnóstico, clasificaciones, evaluación ecográfica, y tratamientos médico y quirúrgico. Finalmente se propone un algoritmo de enfrentamiento terapéutico.

Hidradenitis suppurativa is a chronic inflammatory disease of the hair follicle characterized by recurrent painful and inflamed lesions, predominantly affecting intertriginous regions. Due to its physical sequelae and impact on life quality, we should be familiarized with this disease to make an appropriate diagnosis and implement an early treatment. This clinical guideline, elaborated by the hidradenitis suppurativa workgroup of the Chilean Society of Dermatology and Venereology (SOCHIDERM), review its definition, epidemiology, pathophysiology, risk factors, comorbidities, psycho-emotional impact, clinical presentation, diagnosis, classifications, ultrasonographic evaluation, and its medical and surgical treatments. Finally, a therapeutic approach algorithm is proposed.

[What's new in the treatment of suppurative hidradenitis in 2018 ?] / Quoi de neuf dans le traitement de l'hidradénite suppurative en 2018 ?

Hidradenitis suppurativa (HS), also known as Verneuil's disease and acne inversa, is a chronic inflammatory skin disease characterized by a dysfunction of the pilosebaceous structures resulting in occlusion and inflammation. The disease usually starts after puberty, on average between twenty and thirty years, with subcutaneous painful inflammatory nodules, abscess collections, fistulization and scars mainly in the axillary, inguinal, sub-mammary, perineal and perianal regions. It is a multifactorial disease with the most commonly cited risk factors or aggravating factors being smoking, obesity, and HS family history. The first line of treatment relies on antibiotics, including tetracyclins, clindamycin and rifampycin. In case of non-response, TNF? antagonists, more specifically adalimumab, may be considered.

L'hidradénite suppurative (HS), aussi connue sous le nom de maladie de Verneuil et d'acne inversa, est une maladie inflammatoire chronique caractérisée par un dysfonctionnement des structures pilo-sébacées aboutissant à l'occlusion et à l'inflammation de celles-ci. Elle se manifeste souvent après la puberté, en moyenne entre vingt et trente ans, par des nodules sous-cutanés inflammatoires, des collections abcédées, des fistules et des cicatrices au niveau principalement des régions axillaires, inguinales, sous-mammaires, périnéale et périanale. Il s'agit d'une maladie multifactorielle dont les facteurs de risque ou facteurs aggravants les plus fréquemment cités sont le tabagisme, l'obésité et l'histoire familiale d'HS. La première ligne de traitement fera appel aux antibiotiques de type tétracycline, clindamycine et rifampicine. En seconde intention, on aura recours aux antagonistes TNF? et, plus précisément, à l'adalimumab.

Low and high body mass index in hidradenitis suppurativa patients-different subtypes?

INTRODUCTION: Overweight is a well-established risk factor for hidradenitis suppurativa (HS). In this cross-sectional study, we compare HS patients with a high body mass index (BMI) with HS patients with a low BMI to investigate differences in disease characteristics. MATERIALS AND METHOD: Patients were recruited from 17 dermatological centres from four continents. A total of 246 patients with a BMI below 25 were compared to 205 patients with a BMI of above 35. RESULTS: Patients with a high BMI suffered more severe disease (Hurley, physician global assessment, number of areas affected and patient-reported severity (PRS), P < 0.001 for all). There was no difference in smoking (P = 0.783) nor in family history (P = 0.088). In both low and high BMI patients, early onset of HS was a predictor of positive family history (P < 0.001, for each). For low BMI patients, an increase in BMI significantly increased PRS (P < 0.001). For patients with a high BMI, number of pack-years significantly increased PRS (P = 0.001). Cluster analysis of eruption patterns was location specific for low BMI patients but severity specific for high BMI patients. DISCUSSION: Patients with a low and high BMI could represent two clinically different subtypes. We suggest a non-linear relationship between BMI and impact of HS. As patients go from a low BMI patient to a high BMI patient (or from high to low), eruption patterns and risk factors may change.

[Thoracodorsal artery perforator fasciocutaneous flap in the treatment of chronic hidradenitis suppurativa of axilla]. / Az arteria thoracodorsalis perforátor fasciocutan lebeny a krónikus axillaris hidradenitis suppurativa kezelésében.

BACKGROUND: The only definitive treatment of chronic axillar hidradenitis suppurativa (HS) that prevents relapses is 'in toto' excision of the infected glandular tissue. This way a deficiency emerges, which needs to be restored. Reconstruction with split skin graft (SSG) is a most common way of recovery, but thoracodorsal artery perforator (TDAP) fasciocutneous flap delivers better functional and aesthetic results. METHOD: Between May 2014 and July 2016, 14 patients underwent reconstructive surgery after excision of axillary HS, 2 of them had bilateral lesion. In 15 cases TDAP was used, in 1 case we used thoracodorsal artery capillar perforator flap (TAPcp). RESULTS: In all but 2 cases 1 dominant perforator was found. 1 flap had 2 dominant perforators and 1 flap was supplied by capillary perforators. Size of the flaps spread between 6 × 8 and 10 × 15 cm. 15 reconstructions were successful, 1 flap necrotised because of the lack of compliance of the patient. CONCLUSION: As a result of the glandular tissue excision carried out because of a chronic HS, a deficiency emerges. TDAP flap is an ideal solution for surgical reconstruction of axillar deficiencies, and a great alternative to SSG.

[Acne inversa/hidradenitis suppurativa: A challenge for the future]. / Acne inversa/Hidradenitis suppurativa: Eine Herausforderung für die Zukunft.

Acne inversa (AI)/hidradenitis suppurativa is a chronic recurrent inflammatory dermatosis with signs of a systemic disease. AI is characterized by typical skin alterations in body areas bearing apocrine glands. The care of the AI patients in Germany is still inadequate. This situation might be significantly improved through the following efforts: (i) shortening of the time between the disease onset and the diagnosis/start of therapy; (ii) the in-depth investigation of AI pathogenesis with the aim of identifying innovative therapeutic targets and blood biomarkers; (iii) establishing a method for quantifying the severity of the disease, which takes into account both the clinical assessment and objective laboratory parameters and the self-assessment of the patient (e.g., Dermatology Life Quality Index [DLQI]); (iv) the elaboration of a clear algorithm for the interdisciplinary treatment of patients, which, in addition to the therapy of skin lesions, also includes lifestyle-modification measures and takes into consideration the systemic character of AI. This article describes the current problems of medical care for AI patients and outlines how we can achieve the predetermined goals.

[Diagnosis and treatment of hidradenitis suppurativa].

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease defined by recurrent nodules, tunnels and scarring involving the intertriginous regions. Although patients with HS present a distinct symptomatology with a subsequent impairment of quality of life, an overall diagnostic time of seven years has been reported. A better and broader understanding of this disease may potentially reduce the diagnostic delay. This review provides an updated overview of diagnosis, monitoring tools as well as early treatment interventions.

The role of interleukin-17 in the pathogenesis of hidradenitis suppurativa.

Hidradenitis suppurativa is a chronic inflammatory skin condition affecting primarily the axillary, perianal, and inguinal areas. Patients with hidradenitis suppurativa present with occlusion and subsequent rupture of follicular ducts, profound abscesses, fistulae, odorous discharge, fibrosis, and scar formation, causing significant morbidity. Knowledge of the pathogenesis of hidradenitis suppurativa is limited and treatment with antimicrobial drugs, immunosuppressants, and surgical procedures have shown varying results. The pathogenic role of the interleukin-17 cytokine family in chronic inflammatory skin conditions has been described. Interleukin-17A and interleukin-17F have similar properties and induce the production of cytokines, chemokines, antimicrobial peptides, and metalloproteinases, all of which take part in the inflammatory response. The efficacy of anti-interleukin-17A therapy in psoriasis has also been proven and anti- interleukin-17A drugs are already in use for this condition. There is currently no consensus on the role of interleukin-17 in the pathogenesis of hidradenitis suppurativa. Studies have demonstrated increased interleukin-17 mRNA expression in lesional hidradenitis suppurativa skin, whereas the protein concentrations of interleukin-17 were found to be normal compared to healthy control skin in one other study. A phase II clinical trial on anti- interleukin-17 therapy in hidradenitis suppurativa is ongoing.