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BACKGROUND Hidradenitis suppurativa (HS) is characterized by periodic worsening of both clinical manifestations and symptoms. The aim was to investigate the role of flare outbreak as a possible predictive factor of response to Adalimumab. METHODS 115 HS patients in treatment with adalimumab, with moderate-severe HS, ≥3 abscesses and inflammatory-nodules (ANs) from 5 Italian centers were included in this retrospective analysis. The information about gender, ages at onset/baseline, therapeutic delay, family history, body mass index, smoking, comorbidities, phenotypes, body areas, severity indexes at baseline was collected. Baseline characteristics, total number and timeline of flares were analysed by regression and survival analysis with Hidradenitis Suppurativa Clinical Response (HiSCR). RESULTS During the observational period, 80.9% of patients developed flares, detecting 252 flares. Univariate model identified five factors associated with the absence of response: age (p-value=0.020), comorbidities (p-value=0.030), genital-perineal involvement (pvalue= 0.004), no response at week-12 (p-value=0.027), and flares outbreak (p-value=0.010). Joint analysis of recurrent and terminal events showed a positive correlation between flare recurrence and no-response (p-value

Hidradenitis suppurativa (HS) is a chronic inflammatory disease usually involving the major skin folds characterized by a multifactorial pathogenesis and a wide spectrum of clinical manifestations. It can also rarely present in association with other diseases as complex clinical syndromes, causing additional diagnostic and therapeutic challenges. Different etiopathologic factors contribute to follicular inflammation and suppurative lesions of syndromic HS, including follicular hyperkeratinization and plugging, as well as activation of autoinflammatory pathways. Patients with syndromic HS frequently have a severe disease course, presenting with atypical skin involvement, signs of systemic inflammation, and resistance to conventional treatments. Systematic classification of syndromic HS is based on clinical, pathogenetic, and genetic factors, but it is constantly evolving due to increased disease awareness. Treatment of syndromic HS is difficult and should be personalized on a case-by-case basis. Investigating syndromic HS can lead to useful insights on genetics and pathogenesis, translating into new clinical approaches for sporadic hidradenitis. We review the classification, clinical presentation, disease associations, and therapeutic management of syndromic HS, focusing mainly on its autoinflammatory syndromes PASH, PAPASH, PsAPASH, and PASS.

Introduction Les ichtyoses hereditaires sont des maladies monogeniques de la cornification qui se traduisent par une alteration de la barriere cutanee, une desquamation anormale et des degres variables d’inflammation. Au sein de ce groupe heterogene, les ichtyoses congenitales (ARCI, 11 genes identifies) se caracterisent par leur debut congenital, leur transmission autosomique recessive et leur caractere non syndromique et non bulleux. Des etudes recentes ont revele que certaines ichtyoses genetiques pourraient partager des mecanismes pathogeniques avec le psoriasis mais aucune etude ne s’est interessee a l’association de ces deux dermatoses chez un meme sujet. Materiel et methodes Cette etude retrospective monocentrique a recueilli les donnees demographiques, genotypiques, phenotypiques et therapeutiques concernant tous les cas concomitants d’ARCI et de psoriasis suivis entre 2010 et 2020. Resultats Sur 46 ARCI, 8 patients (17 %), avec confirmation moleculaire [TGM1 (1), ABCA12 (1), Ichthyin (1), ALOX3 (2), ALOX12B (2), CYP4F22 (1)] ont presente une ou plusieurs poussees de psoriasis : 7/8 patients etaient des femmes, d’âge moyen de 28,2 ans (17–43), 50 % recevaient un retinoide systemique au long cours. Le psoriasis debutait a un âge moyen de 23,8 ans (12–33), a type de psoriasis inverse des plis (3/8), de psoriasis pustuleux generalise selon les criteres ERASPEN (4/8) et d’une forme mixte dans 1 cas. La grossesse etait le facteur declenchant dans 2 cas. Le diagnostic de psoriasis etait clinique dans 4 cas, confirme histologiquement dans 4 cas ; une surinfection fongique de l’ARCI etait eliminee dans tous les cas. La recherche de mutation sur les genes de psoriasis monogenique etait negative chez les 3 patients testes. Le psoriasis etait en remission complete sous traitement local (3/8) ou sous biotherapie [anti IL-12/23 (1/8), anti IL-17 (3/8)], cette derniere permettant par ailleurs une amelioration de l’ichtyose. Discussion Notre etude suggere que le psoriasis survient plus frequemment dans la population ARCI que dans la population generale. L’absence de documentation histologique chez certains patients et une certaine similitude histologique entre psoriasis et ARCI ne permettent pas d’exclure des poussees inflammatoires psoriasiformes de l’ichtyose. Le phenotype clinique et/ou histologique pustuleux parait surrepresente. Des etudes recentes ont demontre un desequilibre des lymphocytes Th17 dans la peau ichtyosique, comparable au psoriasis, ainsi qu’un taux eleve de lymphocytes T exprimant l’IL-17 et l’IL-22 dans le sang, correle a la severite clinique. Selon ces observations, la signature immunitaire des ARCI est associee a la polarisation Th17/IL-23, ce qui pourrait expliquer egalement la frequence plus elevee de psoriasis observee dans cette population. Ces observations laissent entrevoir la possibilite de strategies ciblant l’IL-17 pour le traitement de ces ichtyoses.

Atopic dermatitis (AD) is a chronic inflammatory skin disease mediated by multiple signals including janus kinase (JAK)/signal transducer and activator of transcription (STAT) pathway. Current therapeutic armamentarium consists of a limited number of drugs which may result in the insufficient management of AD. Preclinical evidence regarding inhibition of JAK/STAT led to the development of a promising class of therapeutics, namely, JAK inhibitors. Baricitinib, a novel JAK1/JAK2 inhibitor is currently under investigation in AD clinical trials.This review offers an overview of Baricitinib and examines clinical efficacy and safety data in patients with moderate-to-severe AD.Baricitinib showed promising preliminary data in terms of efficacy in phase II and III trials, with a very rapid onset of response and great improvements of itch and sleep disturbances. These aforementioned aspects combined with the advantage of an oral formulation have reduced drug production costs compared to biologic agents and could lead to consideration of baricitinib as a first line systemic treatment. Also, in some countries, it could be a therapeutic option in the case of contraindication or failure of conventional systemic drugs prior to biologic therapies. Data related to long-term safety and efficacy will be important to refine the place-in-therapy of this drug.