PsAPASH: A new syndrome associated with hidradenitis suppurativa with response to tumor necrosis factor inhibition

Saraceno, Rosita; Babino, Graziella; Chiricozzi, Andrea; Zangrilli, Arianna; Chimenti, Sergio

doi:10.1016/j.jaad.2014.10.002

Cited by 56 publications

(33 citation statements)

References 5 publications

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“…In addition to the above‐mentioned syndromes, other less well‐described constellations have been described in single case reports (Table ). The dermatologic manifestations of PASH syndrome can be accompanied by either pyogenic arthritis in PAPASH syndrome or psoriatic arthritis (PsA) in PsAPASH syndrome . PAC syndrome consisting of PG, acne, and ulcerative colitis have also been described .…”

Section: Other Autoinflammatory Syndromes Associated With Hidradenitimentioning

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: Other Autoinflammatory Syndromes Associated With Hidradenitimentioning

confidence: 99%

“…PAC syndrome consisting of PG, acne, and ulcerative colitis have also been described . PSTPIP1 mutations have been identified in PAPASH and PAC syndrome but not yet in PsAPASH . Both anakinra and adalimumab have led to good clinical control of symptoms in these syndromes (Table ) …”

Section: Other Autoinflammatory Syndromes Associated With Hidradenitimentioning

confidence: 99%

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

2017

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“…They include PASS (pyoderma gangrenosum, acne, suppurative hidradenitis, and axial spondyloarthritis), PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and HS) and PsAPASH (psoriatic arthritis, pyoderma gangrenosum, acne, and HS) (Bruzzese, 2012; Marzano et al, 2013; Saraceno et al, 2015). Recently, a PASS patient treated with 100 mg/day Anakinra and improvement of all symptoms was reported, after Anakinra discontinuation relapse occurred within 3 days (Leuenberger et al, 2016).…”

Section: Skin Diseases With Il-1 Involvementmentioning

confidence: 99%

Potential of IL-1, IL-18 and Inflammasome Inhibition for the Treatment of Inflammatory Skin Diseases

2017

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In 2002, intracellular protein complexes known as the inflammasomes were discovered and were shown to have a crucial role in the sensing of intracellular pathogen- and danger-associated molecular patterns (PAMPs and DAMPs). Activation of the inflammasomes results in the processing and subsequent secretion of the pro-inflammatory cytokines IL-1β and IL-18. Several autoinflammatory disorders such as cryopyrin-associated periodic syndromes and Familial Mediterranean Fever have been associated with mutations of genes encoding inflammasome components. Moreover, the importance of IL-1 has been reported for an increasing number of autoinflammatory skin diseases including but not limited to deficiency of IL-1 receptor antagonist, mevalonate kinase deficiency and PAPA syndrome. Recent findings have revealed that excessive IL-1 release induced by harmful stimuli likely contributes to the pathogenesis of common dermatological diseases such as acne vulgaris or seborrheic dermatitis. A key pathogenic feature of these diseases is IL-1β-induced neutrophil recruitment to the skin. IL-1β blockade may therefore represent a promising therapeutic approach. Several case reports and clinical trials have demonstrated the efficacy of IL-1 inhibition in the treatment of these skin disorders. Next to the recombinant IL-1 receptor antagonist (IL-1Ra) Anakinra and the soluble decoy Rilonacept, the anti-IL-1α monoclonal antibody MABp1 and anti-IL-1β Canakinumab but also Gevokizumab, LY2189102 and P2D7KK, offer valid alternatives to target IL-1. Although less thoroughly investigated, an involvement of IL-18 in the development of cutaneous inflammatory disorders is also suspected. The present review describes the role of IL-1 in diseases with skin involvement and gives an overview of the relevant studies discussing the therapeutic potential of modulating the secretion and activity of IL-1 and IL-18 in such diseases.

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“…19 An entity called PAPASH syndrome pyogenic (inflammatory) arthritis, pyoderma gangrenosum, acne, hidradenitis suppurativa has been discussed in the literature. 21 …”

Section: Background and Discussionmentioning

confidence: 99%

A Case of Relapsing Polyarthritis Associated With Hidradenitis Suppurativa

Adiga

2016

Journal of Investigative Medicine High Impact Case Reports

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Hidradenitis suppurativa (HS) is a chronic, inflammatory follicular skin disease with recurrent skin nodules, sinus tracts, and scarring. We observed a case of HS associated with relapsing polyarthritis. On presentation the patient had a flare of polyarthritis with an increase in the number and size of pustular nodules. He has had similar episodes 1 to 2 times yearly subsiding with antibiotic treatment. Radiographs revealed erosions and demineralization. Symptoms improved following institution of anti-inflammatory and antibiotic therapy. HS is associated with several inflammatory conditions, and dysregulation in innate immunity may play an important role in etiopathogenesis. Spondyloarthritis/sacroiliitis is the most common joint manifestation in HS and mechanism(s) underlying arthropathy is unknown. Treatment of arthritis in HS is anecdotal.

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PsAPASH: A new syndrome associated with hidradenitis suppurativa with response to tumor necrosis factor inhibition

Abstract: N/

Cited by 56 publications

References 5 publications

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Potential of IL-1, IL-18 and Inflammasome Inhibition for the Treatment of Inflammatory Skin Diseases

A Case of Relapsing Polyarthritis Associated With Hidradenitis Suppurativa

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