Inter-rater agreement and reliability of outcome measurement instruments and staging systems used in hidradenitis suppurativa.

BACKGROUND: Monitoring disease activity over time is a prerequisite for clinical practice and research. Valid and reliable outcome measurement instruments (OMIs) and staging systems provide researchers and clinicians with benchmark tools to assess the primary and secondary outcomes of interventional trials and to guide treatment selection properly. OBJECTIVES: To investigate inter-rater reliability and agreement in instruments currently used in hidradenitis suppurativa (HS), with dermatologists experienced in HS as the rater population of interest. METHODS: In a prospective completely balanced design, 24 patients with HS underwent a physical examination by 12 raters (288 assessments) using nine instruments. The results were analysed using generalized linear mixed models. RESULTS: For the staging systems, the study found good inter-rater reliability for Hurley staging in the axillae and gluteal region, moderate inter-rater reliability for Hurley staging in the groin and for Physician's Global Assessment, and fair inter-rater reliability for refined Hurley staging and the International HS Severity Scoring System. For all the tested OMIs, the observed intervals for limits of agreement were very wide relative to the ranges of the scales. CONCLUSIONS: The very wide intervals for limits of agreement imply that substantial changes are needed in clinical research in order to rule out measurement error. The results illustrate a difficulty, even for experienced HS experts, to agree on the type and number of lesions when evaluating disease severity. The apparent caveats call for global efforts, such as the HIdradenitis SuppuraTiva cORe outcomes set International Collaboration (HISTORIC) to reach consensus on how best to measure physical signs of HS reliably in randomized trials. What's already known about this topic? Without valid and reliable instruments to measure outcomes, researchers and clinicians lack the necessary benchmarks to assess primary and secondary end points of interventional trials properly. Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. Several outcome measure instruments exist for HS, but their validation is generally incomplete or of relatively low methodological quality. What does this study add? Using a prospective completely balanced design this study examined inter-rater reliability with HS-experienced dermatologists as the rater population of interest. The study did not find very good reliability for any included instrument or lesion counts. This study illustrates the difficulty in finding agreement on the type and number of HS lesions, even among experts. The results question whether physical signs are best measured by a traditional physician lesion count instrument. What are the clinical implications of this work? For staging, Hurley staging and physician global visual analogue scale proved to be acceptable instruments in terms of inter-rater reliability. For the instruments designed to measure changes in health status, our study illustrates how difficult it is, even for experts, to measure the physical signs of HS using a simple rater counting. Consequently, other assessment methods of physicals signs, such as ultrasound evaluation, require consideration.

Pilonidal sinus disease: an intergluteal localization of hidradenitis suppurativa/acne inversa: a cross-sectional study among 2465 patients.

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.

Could isotretinoin flare hidradenitis suppurativa? A case series.

We report eight cases of patients with severe acne who were treated with isotretinoin and developed painful nodules in the axillae and groin, consistent with hidradenitis suppurativa (HS). The pathogenesis of HS is still not completely understood; recent research from a study in 2011 of biopsies from HS lesions showed a reduction or absence of sebaceous glands compared with normal skin in patients with HS, with the report suggesting that this contributes to the pathogenesis of the disease. Interestingly, the main effect of isotretinoin is to decrease the size and action of sebaceous glands, so hypothetically, as isotretinoin acts by reducing the sebaceous glands further it could potentially aggravate HS. Our experience has instilled caution in our prescribing of isotretinoin, and we question patients, particularly those with acne conglobata, about symptoms of HS prior to and during treatment.

[Acne flare on isotretinoin: A pointer to diagnosis of hidradenitis suppurativa]. / « Poussées inflammatoires d'acné ¼ sous isotrétinoïne révélant une hidradénite suppurée : 4 cas.

BACKGROUND: Isotretinoin is the most potent treatment for acne but paradoxical flares can occur. HS lesions of the face may be mistaken for acne. We report on 4 patients in whom an "acne" flare on isotretinoin led to the correct diagnosis of HS. PATIENTS AND METHODS: Four young male patients aged 15 to 28 years were referred to us because of an acne flare on isotretinoin. Three of them had clinical features considered unusual in acne : involvement of the nape of the neck (2), retroauricular cysts (2), a rope-like pre-sternal lesion (1), a large bag-like sinus on the face (1), large deep depressed U-type scars on the back (3). Questioning revealed that all three had previously experienced several attacks of inflammatory nodules in the axillae and/or inguinal folds; they had not mentioned these lesions since they seemed so minor. The fourth patient had lesions typical of HS comprising nodules, sinus, rope-like hypertrophic scars on the face mistaken for acne, epidermal cysts on the scrotum and pubic folliculitis. Treatment with systemic antibiotics resulted in regression of lesions in all 4 patients. DISCUSSION: An acne flare on isotretinoin requires investigations with a view to potential diagnosis of HS. Patients presenting "acne" and atypical features such as involvement of the neck, large U scars and cord-like structures should be questioned about the presence of nodules in the axillae and groin since patients with mild HS may not spontaneously acknowledge such typical symptoms.

Disseminate recurrent folliculitis as the presenting picture of hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) has a polymorphous clinical presentation. Herein we report two cases of HS revealed by disseminate recurrent folliculitis. PATIENTS AND METHODS CASE 1: A 31-year-old woman consulted for disseminate recurrent folliculitis on the trunk and proximal segments of the limbs that had been ongoing for several years. No other cutaneous lesions were seen, particularly in the classic area for HS lesions. Weight loss and treatment with azithromycin 500mg/day resulted in complete remission, but relapse occurred on dosage reduction. CASE 2: The elder sister of patient 1, who was also obese and 10 years her senior, presented a similar clinical history, although in addition, she was presenting lesions characteristic of HS on her armpits and inguinal folds; further, she had undergone an operation 3 years earlier for pilonidal sinus. Three months of combined treatment with rifampicin and clindamycin 900 mg/day followed by long-term doxycycline 200mg/day resulted in marked improvement. DISCUSSION: Disseminate folliculitis is not infrequent during the course of HS; it forms part of the clinical picture and is particularly common in the follicular phenotypic variant; however, it is not part of the diagnostic criteria as defined consensually by the Hidradenitis Suppurativa Foundation. The cases we report suggest that a setting of disseminate recurrent folliculitis may constitute the mode of presentation of HS, and may even represent an atypical monosymptomatic form of HS. CONCLUSION: Disseminate recurrent folliculitis constitutes part of the clinical picture of HS; if it is isolated, screening should be performed for personal or familial signs of HS.

European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa.

Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillae, inguinal and anogenital regions. A mean disease incidence of 6.0 per 100,000 person-years and an average prevalence of 1% has been reported in Europe. HS has the highest impact on patients' quality of life among all assessed dermatological diseases. HS is associated with a variety of concomitant and secondary diseases, such as obesity, metabolic syndrome, inflammatory bowel disease, e.g. Crohn's disease, spondyloarthropathy, follicular occlusion syndrome and other hyperergic diseases. The central pathogenic event in HS is believed to be the occlusion of the upper part of the hair follicle leading to a perifollicular lympho-histiocytic inflammation. A highly significant association between the prevalence of HS and current smoking (Odds ratio 12.55) and overweight (Odds ratio 1.1 for each body mass index unit) has been documented. The European S1 HS guideline suggests that the disease should be treated based on its individual subjective impact and objective severity. Locally recurring lesions can be treated by classical surgery or LASER techniques, whereas medical treatment either as monotherapy or in combination with radical surgery is more appropriate for widely spread lesions. Medical therapy may include antibiotics (clindamycin plus rifampicine, tetracyclines), acitretin and biologics (adalimumab, infliximab). A Hurley severity grade-relevant treatment of HS is recommended by the expert group following a treatment algorithm. Adjuvant measurements, such as pain management, treatment of superinfections, weight loss and tobacco abstinence have to be considered.

[Clinical evaluation of the severity of hidradenitis suppurativa]. / Évaluation clinique de la sévérité de l'hidradénite suppurée--maladie de Verneuil.

Treatment and long-term management of patients with chronic diseases require the use of instruments to measure severity and outcome. In the case of hidradenitis suppurativa, such instruments are currently poorly understood and utilised. Herein, we describe the main instruments, their scope of application, their qualities and their shortcomings. The Hurley classification, which was devised for surgical assessment and is limited to description of impairment at a given site, is too often used to assess overall severity in individual patients. The Sartorius score (and variants thereof) combines global and local measurements; it is widely used, but because of its hybrid nature, the global score, or PGA-HS, is currently used; the latter, focusing on inflammatory signs, is perfectly suitable for daily assessment of pharmaceutical treatment. The HiSCR score, derived from the foregoing grading system, is used as a global criterion in clinical trials. Patient-based instruments, such as pain or suppuration score, and, to a lesser extent, quality-of-life score constitute very useful supplements. Since the disease is extremely heterogeneous, no instruments cover the entire array of situations and the requirements of medical and surgical practitioners involved in the management of this patient population.

[Antibiotic treatment of hidradenitis suppurativa]. / Les traitements antibiotiques dans l'hidradénite suppurée-maladie de Verneuil.

Bacterial infection plays an important role in hidradenitis suppurativa (HS). This infection has a number of unique features: chronicity, absence of lymph node involvement, and absence in most cases of acute super-infection by Staphylococcus aureus and/or streptococci. Treatment is based mainly on antibiotics. Various bacteria are involved; they are often part of the resident flora and may combine in polymicrobial infections, and they consist mainly of two families: coagulase-negative staphylococci (CNS) and anaerobes. Numerous antibiotics are active against CNS: betalactamins, lincosamides, macrolides, rifampicin, tetracyclines and fluoroquinolones. Antibiotics active against anaerobes include metronidazole, betalactamins, lincosamides and one fluoroquinolone (moxifloxacine). Antibiotics are given per os and in combination. Treatment is usually long-term, with a frequent need for maintenance therapy. It has to be tailored to various clinical situations: intermittent development, in which "abortive" emergency treatment is used; major or major continuous forms, where combined antibiotics are used, most frequently rifampicin and clindamycin. The global treatment strategy involves a surgical approach, which can be aided but not replaced by antibiotics. While the risks of long-term antibiotic use are reduced in this specific population of "healthy" young adults, they are not absent.

Development and evaluation of a Global Acne Severity Scale (GEA Scale) suitable for France and Europe.

BACKGROUND AND OBJECTIVE: Many acne grading methods exist; however, there is no agreed-upon standard. Our objective was to create and validate a reproducible acne assessment scale for rating the severity of juvenile facial acne suitable for use in France and Europe. METHODS: The scale we created described the different types of acne lesions in a manner similar to global assessment scales used in clinical trials. The scale was then validated by seven expert dermatologists in the field of acne [the Global Evaluation Acne (GEA) group] first on 34 photographic cases of Caucasian acne patients and second by clinical examination of 22 acne patients. RESULTS: There was good agreement in Investigators' assessments of acne both on photographs and patients (R = 0.8057; P < 0.0001, and R = 0.8437; P = 0.0015). CONCLUSION: The GEA Scale is a global scale validated both on photographs and acne patients which can be used either in clinical research or by the dermatologist in his office.