Remission of refractory pyoderma gangrenosum, severe acne, and hidradenitis suppurativa (PASH) syndrome using targeted antibiotic therapy in 4 patients

Join‐Lambert, Olivier; Duchatelet, Sabine; Delage, M.; Miskinyte, S.; Coignard, H.; Lemarchand, Nicolas; Alemy-Carreau, Murielle; Lortholary, Olivier; Nassif, Xavier; Hovnanian, Alain; Nassif, Aude

doi:10.1016/j.jaad.2015.07.040

Cited by 31 publications

(44 citation statements)

References 12 publications

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“…Hidradenitis suppurativa in association with autoinflammatory syndromes is often of severe type (Hurley II, III) unresponsive to many of the standard regimens (Fig. ) . The TNF blocking agents adalimumab and infliximab have obtained good control of autoinflammatory HS lesions, supporting the role of a dysregulated innate immune response in HS pathogenesis.…”

Section: Characteristics Of Nonsyndromic and Syndromic Acne And Hidramentioning

confidence: 99%

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Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

2017

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Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)-1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence-based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.

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Section: Characteristics Of Nonsyndromic and Syndromic Acne And Hidramentioning

confidence: 99%

“…Disease symptoms are often severe and can be unresponsive to standard regimens. Acute stabilizing in‐hospital treatment followed by long‐term specialized multimodal treatment is required . If not managed sufficiently, these syndromes can have devastating physical and psychological consequences for the affected patients .…”

Section: Introductionmentioning

confidence: 99%

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

2017

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“…Mutations in PSTPIP1, via increased binding affinity to pyrin, are responsible for the activation of an inflammasome. [1][2][3] In turn, caspase-1 is activated and cleaves pro-interleukin (IL)-1ß to its active isoform IL-1ß. The overproduction of IL-1ß leads to uncontrolled release of pro-inflammatory cytokines, particularly IL-17, which direct recruitment and activation of neutrophils.…”

Section: Discussionmentioning

confidence: 99%

“…Aseptic abscess syndrome shares the abnormality in the PSTPIP1 promoter region seen in PASH syndrome, specifically the increased number of the CCTG microsatellite repeat. [1][2][3] None of these are noted to have vasculitis as a feature, despite the predominant neutrophilic infiltrates these all share.…”

Section: Jaad Case Reports Volume 3 Numbermentioning

confidence: 97%