The Association among Pyoderma Gangrenosum, Ulcerative Colitis, and Hidradenitis Suppurativa and the Syndromic Hidradenitis Suppurativa Network: A Case Report

Bettoli, Vincenzo; Schettini, Natale; Libanore, Marco; Scuderi, Valeria; Zedde, Pierantonia; Forconi, Riccardo; Pacetti, Lucrezia; Ceccherini, Isabella; Gattorno, Marco; Poggioli, Gilberto; Corazza, Monica

doi:10.1159/000513639

Cited by 1 publication

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References 10 publications

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“…Saternus et al 130 highlighted new combinations of AIS with other disorders, such as the tetrad of pyoderma gangrenosum—acne—HS—leukocytoclastic vasculitis coined as VPASH, PASCD (PASH and Crohn's disease), PASC (PASH and ulcerative colitis) or PsAPSC (psoriatic arthritis, PG, SH and Crohn's disease). Another form 134 included pyoderma gangrenosum, ulcerative colitis and HS in a 27‐year‐old female with symptoms of HS and PG that almost completely disappeared after colectomy. These new possible forms contour the contribution of yet unidentified genetic factors leading to systemic innate immune dysregulation in syndromic HS.…”

Section: Resultsmentioning

confidence: 99%

Hidradenitis suppurativa: Detangling phenotypes and identifying common denominators

Vișan,

Căruntu,

Costache

et al. 2023

Acad Dermatol Venereol

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Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease with a severe impact on patients' quality of life through its recurrent and painful nature, as well as its comorbidity burden. The shift in the pathogenic paradigm from a condition of the apocrine glands to an autoinflammatory disease associated with follicular destruction has rendered its understanding difficult, as there are still large gaps in pinpointing the underlying mechanisms, which cannot currently explain the existing clinical variation and as a result, translate into suboptimal therapy. Multifactorial involvement is hypothesized, with an implication of genetic mutations, microbiome dysbiosis, cytokine upregulation and environmental factors. Clinical observation is fundamental for diagnosis, however, the marked heterogeneity in presentation leads to delays in detection and challenges in treatment selection, showcasing clear limits in defining the link between genetic aspects of HS, the role of epigenetic factors and its pathogenic pathways. There have been attempts to formulate phenotypes that could aid in prognostication and management, however, current classification schemata show significant overlap and no validation through longitudinal studies. In this context, nomenclature poses a great challenge due to the lack of global agreement in the definition of lesions, which should be addressed by future research to enable simplified recognition and allow for more precise severity scoring. This could be complemented by the addition of extra dermatologic findings or paraclinical assessment in constructing phenotypes. The development of valid, predictive and reliable classifications of HS may lead to an improvement in comprehending its pathophysiology, favouring a more personalized approach in management. This could be achieved through consensus in the characterization of clinical features and data gathering, as well as validation attempts for described phenotypes. Ultimately, the genotype‐endotype‐phenotype correlation in HS requires targeted, systematic inquiries and should be addressed more largely to broaden the perspective on this debilitating entity.

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Section: Resultsmentioning

confidence: 99%