PASH Syndrome: The First Case Report from Iran

Mansouri, Parvin; Hesami, Zeinab; Hajiha, Nima; Chalangari, Alireza; Chalangari, Katalin Martits; Azizian, Zahra

doi:10.5812/jssc.86096

Cited by 2 publications

(1 citation statement)

References 13 publications

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“…Oral retinoids did not result in favorable effects in the majority of the reported cases (80%). In a single PASH case, isotretinoin in combination with oral steroids induced a 2-year remission [71].…”

Section: Resultsmentioning

confidence: 86%

Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review

et al. 2020

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Background: There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients. Methods: A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Results: Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission. Conclusion: The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist’s role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.

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Section: Resultsmentioning

confidence: 86%

Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review

et al. 2020

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PASH Syndrome in Iran

Yasri¹,

Wiwanitkit

2019

J Skin Stem Cell

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We read a paper entitled "PASH syndrome: The first case report from Iran," by Mansouri et al. with a great interest (1). Mansouri et al. mentioned that "PASH syndrome despite rarity should be considered as a possible cause of complex skin manifestations" (1).PASH syndrome is an uncommon problem. It is a clinical syndrome that consists of pyoderma gangrenosum, acne, and hidradenitis suppurativa (2). Cutaneous inflammation is the main pathology of PASH. The underlying pathogenesis of this syndrome is over-activation of the innate immune system that results in the increased production of the interleukin (IL)-1 family (2). Genetic heterogenicity is reported in PASH syndrome (3). The NCSTN gene is reported to have an association with PASH syndrome (4). The PASH syndrome is sporadically reported from several countries around the world, such as the USA, Spain, China,. A novel multimodal treatment with infliximab, cyclosporine, and dapsone is recommended for PASH treatment (8). The present report by Mansouri et al. ( 1) is not the first case report from Iran. Faraji Zonooz et al. reported a similar case from Iran (3). The genetic variation in that case is well described (data available at https://infevers.umaimontpellier.fr/web/detail_mutation.php?n=35&Id_muta-tion=1875&page=sequence&seq=cDNA&ancre=1875&prot=0). FootnotesAuthors' Contribution: Sora Yasri and Viroj Wiwanitkit made an equal contribution.

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PASH Syndrome: The First Case Report from Iran

Cited by 2 publications

References 13 publications

Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review

Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review

PASH Syndrome in Iran

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