Wound care, adalimumab, and multidisciplinary approach in a patient affected by <scp>PASH</scp> syndrome

Zagaria, Orlando; Ruggiero, Angelo; Fabbrocini, Gabriella; Gallo, Lucia; Romanelli, Marco; Marasca, Claudio

doi:10.1111/iwj.13403

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…Autoinflammatory syndromes (AIS) of HS are PASH, PAPASH, pyoderma gangrenosum (PG)—acne vulgaris—HS—ankylosing spondylitis (PASS) and psoriatic arthritis—pyoderma gangrenosum—acne—HS (PsAPASH). PASH 87–118 was predominant, with 68 total patients and five overlapping synovitis‐acne‐pustulosis‐hyperostosis‐osteitis (SAPHO) forms. Phenotyping in AIS is ill‐defined, but despite heterogeneity, there are similar features.…”

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%

Hidradenitis suppurativa: Detangling phenotypes and identifying common denominators

Vișan,

Căruntu,

Costache

et al. 2023

Acad Dermatol Venereol

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“…Our results provide potential insights into why ADA administration could lead to improved efficacy in conjunction with wide-excision surgery for patients with HS who require surgical intervention as was reported in the HS SHARPS clinical study ( Bechara et al, 2021 ). Additional studies have shown that ADA promotes HS excisional wound healing and skin manifestations ( Saint-Georges et al, 2018 ; Zagaria et al, 2020 ). Moreover, a pilot study, presented by Gefion Girbig at the 2020 European Academy of Dermatology and Venereology congress, showed that in conjunction with wide-excision surgery, ADA enhances primary wound closure after HS surgery and reduces secondary wound healing disorders and complications.…”

Section: Discussionmentioning

confidence: 99%

“…A previous study has shown that ADA but neither ETN nor CZP induces a wound-healing profile in vitro by regulating macrophage differentiation and matrix metalloproteinase expression, which may underlie the difference in efficacy among various anti–TNF-α agents in impaired wound healing ( Cao et al, 2021 ). Although several studies have suggested that ADA can have a substantial positive impact on HS excisional wound healing and skin manifestations ( Saint-Georges et al, 2018 ; Zagaria et al, 2020 ), wound healing and secondary wound reconstruction were not assessed in the SHARPS study ( Bechara et al, 2021 ). In addition to HS, it has been reported in case studies that anti–TNF-α agents may reduce ulcerated necrobiosis lipoidica plaques in patients with type 2 diabetes ( Sandhu and Alavi, 2019 ).…”

Section: Introductionmentioning

confidence: 99%

Therapeutic TNF Inhibitors Exhibit Differential Levels of Efficacy in Accelerating Cutaneous Wound Healing

Cao,

Harvey,

Jin

et al. 2024

JID Innovations

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“…), traditional immunosuppressants (corticosteroids, cyclosporine, sulfasalazine, etc. ), immunomodulators (thalidomide and dapsone), biologics (anti-TNF, anti-IL-1, anti-IL-17, and anti-IL-23), and surgical procedures ( 3 , 20 – 27 ). Moreover, any proposed therapeutic strategy should address all three comprising entities as effectively as possible, and combined therapies are strongly recommended.…”

Section: Discussionmentioning

confidence: 99%

Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa Syndrome: A Case Report and Literature Review

et al. 2022

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Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome is a rare inflammatory disease characterized by pyoderma gangrenosum (PG), mild to severe facial acne, and hidradenitis suppurativa (HS). It only affects the skin and represents cutaneous characteristics of a spectrum of autoinflammation. Lack of pyogenic sterile arthritis (PA) distinguishes the pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome from pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PA-PASH), pyoderma gangrenosum, acne, hidradenitis suppurtiva, and ankylosing spondylitis (PASS), and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndromes. The exact etiology and pathogenesis of PASH syndrome remain unknown. Both PG and HS are contained in the spectrum of neutrophilic dermatitis, which is considered as an autoinflammatory syndrome. From a pathophysiological point of view, they show similar mechanisms, including neutrophil-rich cutaneous infiltration and overexpression of the interleukin-1 (IL-1) family. These findings provide guidance for these intractable diseases. In this review, we described a case of PASH syndrome in a patient who initially failed to respond to immunosuppressive treatment but responded to a combination of colchicine and thalidomide. We reviewed the relevant literature that focuses on PASH syndrome management.

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Wound care, adalimumab, and multidisciplinary approach in a patient affected by PASH syndrome

Cited by 5 publications

References 14 publications

Hidradenitis suppurativa: Detangling phenotypes and identifying common denominators

Hidradenitis suppurativa: Detangling phenotypes and identifying common denominators

Therapeutic TNF Inhibitors Exhibit Differential Levels of Efficacy in Accelerating Cutaneous Wound Healing

Pyoderma Gangrenosum, Acne, and Hidradenitis Suppurativa Syndrome: A Case Report and Literature Review

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