HLA-B*51 allelic and carrier frequency in Kazakhstan: insights into Behçet's disease prevalence in Central Asia.

Behçet's disease (BD) is a systemic vasculitis whose prevalence varies according to the ethnicity. HLA-B*51 is a well-known genetic factor predisposing to BD: populations with higher HLA-B*51 allelic frequencies are also those with a greater disease prevalence, as occurs in the Silk Road countries. However, the BD prevalence is unknown in many countries, including Kazakhstan and, in general, Central Asia, despite the location on or near the Silk Road. Due to the lack of clinical studies from this area, we analyzed the bone marrow donors registry in Kazakhstan, in order to assess the HLA-B*51 allelic and carrier frequencies, which resulted to be 8.14 and 15.28%, respectively. Through a comparison with the same parameters in other countries (by performing a data search in the medical literature and in the Allele Frequency Net Database), we might speculate a BD prevalence in Kazakhstan of 10-15/100.000 at least. This result should prompt a greater clinical consideration of this disease in Central Asia, as well as the publication of clinical data from this geographic area.

Adalimumab in the treatment of pediatric Behçet's disease: case-based review.

Behçet's disease (BD) is a systemic vasculitis affecting prominently the veins, which is usually diagnosed in adulthood, but can occur in children younger than 16 years in about 4-26% of cases. The therapy is based on several immune-suppressive drugs; in case of inadequate control and/or complications, the biologic therapy with anti-TNF drugs has been successfully used in adults. Here, we reported one pediatric case of BD with systemic (persistent/recurrent high fever), skin and mucosal manifestations (recurrent aphthous stomatitis, anal/penile ulcers, erythema nodosum and papulo-pustules), that were unresponsive to the conventional treatment with steroids and colchicine; however, he was successfully treated with adalimumab. Compared to adult patients, the experience with adalimumab in the treatment of pediatric BD is very limited. Indeed, through a systematic search in the medical literature, we retrieved 4 case reports and 2 case series, describing BD pediatric patients treated with adalimumab, in addition to three clinical studies including some BD children. The analysis and discussion of these available clinical experiences may indicate adalimumab as an effective and safe option to treat several forms of BD, in addition to BD-related chronic uveitis.

Pruritus in patients with amyopathic dermatomyositis.

Amyopathic dermatomyositis has been associated with the exposure to several drugs: the article by Jeimy et al. described the onset of this uncommon disease in a patient treated with omalizumab. Paradoxically, this patient complained of an intense pruritus and this finding has been reported by several authors observing patients with amyopathic dermatomyositis.