Autoinflammatorische Syndrome in der Dermatologie

Engels, Laura; Ruzicka, Thomas; Giehl, Kathrin

doi:10.1007/s15012-019-2886-y

Cited by 1 publication

(4 citation statements)

References 12 publications

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“…It has been established that PG is a type of neutrophilic dermatitis. In combination with other symptoms, including pyogenic arthritis (PA), acne (A) or suppurative hidradenitis (SH), PG can be a symptom of distinct autoinflammatory syndromes [ 5 ].…”

Section: Methodsmentioning

confidence: 99%

“…Another group of autoinflammatory diseases not associated with PG includes cryopyrin-associated periodic syndromes (CAPS), familial Mediterranean fever (FMF), hyper-IgD syndrome (hypergammaglobulinemia D and HIDS, also known as mevalonate kinase deficiency), tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and Schnitzler’s syndrome (urticarial vasculitis) [ 5 , 6 ]. CAPS, FMF, HIDS and TRAPS are distinct entities of hereditary periodic fever syndrome (HPFS), usually seen in children [ 6 ].…”

Section: Methodsmentioning

confidence: 99%

“…The basic shared pathomechanism of autoinflammatory syndromes is the dysfunction of inflammasomes [ 5 ].…”

Section: Methodsmentioning

confidence: 99%

“…Inflammasomes comprise protein complexes located within the cytosol that are in a group involved in the production of important proinflammatory cytokines such as interleukin-1β (IL-1β) and IL-18 via caspase 1 activation [ 5 , 7 ]. Inflammasomes also play key roles in recognizing conserved pathogen-associated molecular patterns (PAMPs) and damage-associated molecular patterns (DAMPs) [ 5 , 7 ].…”

Section: Methodsmentioning

confidence: 99%

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Ancient friends, revisited: Systematic review and case report of pyoderma gangrenosum-associated autoinflammatory syndromes

Saternus

Schwingel

Müller

et al. 2020

Journal of Translational Autoimmunity

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In the last decade, new scientific findings significantly improved our understanding of the molecular pathogenesis of autoinflammation and have resulted in the identification and definition of several pyoderma gangrenosum-associated autoinflammatory syndromes (PGAAIS) as new and distinct clinical entities. These different clinical entities include PAPA (pyogenic arthritis, pyoderma gangrenosum and acne conglobata), PASH (pyoderma gangrenosum, acne and suppurative hidradenitis), PAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and pyogenic arthritis), PsAPASH (pyoderma gangrenosum, acne, suppurative hidradenitis and psoriatic arthritis), PASS (pyoderma gangrenosum, acne conglobata, suppurative hidradenitis, and axial spondyloarthritis) and PAC (pyoderma gangrenosum, acne and ulcerative colitis), which can be distinguished by their clinical presentation and the presence or absence of mutations in several genes, such as the genes encoding proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1), nicastrin (NCSTN), Mediterranean fever (MEFV) and nucleotide-binding oligomerization domain-containing protein (NOD). In this systematic review, we summarize the present knowledge of this rapidly developing hot topic and provide a guide to enable the easy diagnosis of these syndromes in everyday clinical practice. Moreover, we report a rare case of PASS syndrome demonstrating successful treatment with adalimumab and another case of a previously unreported combination of symptoms, including psoriatic arthritis, pyoderma gangrenosum, suppurative hidradenitis and Crohn’s disease (newly coined PsAPSC), as examples. Because of the identification of similar genetic and pathogenic mechanisms of PGAAIS, we think the wide variety of seemingly different syndromes may represent distinct phenotypes of one disease.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

“…The basic shared pathomechanism of autoinflammatory syndromes is the dysfunction of inflammasomes [ 5 ].…”

Section: Methodsmentioning

confidence: 99%