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Piercing of the tongue and perioral regions is an increasingly popular expression of body art, with more patients coming in for a routine check-up with tongue and/or lip piercings. Several complications of oral piercing have been reported, some of which are life-threatening. In the present clinical survey the prevalence of both tongue and lip piercing complications in oral health was assessed in a group of 50 patients. The most common dental problem registered was chipping of the teeth, especially in association with tongue piercing. Gingival recession was seen as a result of lip piercing with studs. Postprocedural complications included oedema, haemorrhage and infection. Therefore, dentists and oral and maxillofacial surgeons should be given more authority to advise patients with oral and facial piercings or those who plan to acquire this type of body art. [ABSTRACT FROM AUTHOR]

An amendment to this paper has been published and can be accessed via a link at the top of the paper. [ABSTRACT FROM AUTHOR]

Hyperinflammatory syndromes are life-threatening disorders caused by overzealous immune cell activation and cytokine release, often resulting from defects in negative feedback mechanisms. In the quintessential hyperinflammatory syndrome familial hemophagocytic lymphohistiocytosis (HLH), inborn errors of cytotoxicity result in effector cell accumulation, immune dysregulation and, if untreated, tissue damage and death. Here, we describe a human case with a homozygous nonsense R688* RC3H1 mutation suffering from hyperinflammation, presenting as relapsing HLH. RC3H1 encodes Roquin-1, a posttranscriptional repressor of immune-regulatory proteins such as ICOS, OX40 and TNF. Comparing the R688* variant with the murine M199R variant reveals a phenotypic resemblance, both in immune cell activation, hypercytokinemia and disease development. Mechanistically, R688* Roquin-1 fails to localize to P-bodies and interact with the CCR4-NOT deadenylation complex, impeding mRNA decay and dysregulating cytokine production. The results from this unique case suggest that impaired Roquin-1 function provokes hyperinflammation by a failure to quench immune activation. Roquin-1 is a posttranscriptional regulator that controls the expression of many immune-related genes such as ICOS and TNFA. Here, the authors report a homozygous R688* loss of function mutation in Roquin-1 in a patient with syndromic uncontrolled hyperinflammation associated with immune cell activation and hypercytokinemia. [ABSTRACT FROM AUTHOR]