PASH Syndrome in Iran

authors:

avatar Sora Yasri 1 , * , avatar Viroj Wiwanitkit 2

KMT Primary Care Center, Bangkok, Thailand
Dr. D. Y. Patil University, Pune, India

how to cite: Yasri S, Wiwanitkit V. PASH Syndrome in Iran. J Skin Stem Cell. 2019;6(1):e98423. https://doi.org/10.5812/jssc.98423.

Dear Editor,

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, etc. (5-7). 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.umai-montpellier.fr/web/detail_mutation.php?n=35&Id_mutation=1875&page=sequence&seq=cDNA&ancre=1875&prot=0).

References

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    Mansouri P, Naraghi Z, Hesami Z, Hajiha N, Chalangari Kasir A, Azizian Z. PASH syndrome: The first case report from Iran. J Skin Stem Cell. 2019;5(4). e86096. https://doi.org/10.5812/jssc.86096.

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    Faraji Zonooz M, Sabbagh-Kermani F, Fattahi Z, Fadaee M, Akbari MR, Amiri R, et al. Whole genome linkage analysis followed by whole exome sequencing identifies nicastrin (NCSTN) as a causative gene in a multiplex family with gamma-secretase spectrum of autoinflammatory skin phenotypes. J Invest Dermatol. 2016;136(6):1283-6. [PubMed ID: 26968259]. https://doi.org/10.1016/j.jid.2016.02.801.

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    Niv D, Ramirez JA, Fivenson DP. Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis. JAAD Case Rep. 2017;3(1):70-3. [PubMed ID: 28203623]. [PubMed Central ID: PMC5294749]. https://doi.org/10.1016/j.jdcr.2016.11.006.

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    Li C, Xu H, Wang B. Is SAPHO syndrome linked to PASH syndrome and Hidradenitis suppurativa by Nicastrin mutation? A case report. J Rheumatol. 2018;45(11):1605-7. [PubMed ID: 30068763]. https://doi.org/10.3899/jrheum.171007.

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    Staub J, Pfannschmidt N, Strohal R, Braun-Falco M, Lohse P, Goerdt S, et al. Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. J Eur Acad Dermatol Venereol. 2015;29(11):2243-7. [PubMed ID: 25352307]. https://doi.org/10.1111/jdv.12765.