Pseudoxantoma elasticum (PXE), also known as Groenblad-Strandberg syndrome, is a rare heritable disease with an estimated prevalence of 1:50,000 in the general population. PXE is considered a prototype of multisystem ectopic mineralization disorders and it is characterized by aberrant mineralization of soft connective tissue with degeneration of the elastic fibers, involving primarily the eyes, the cardiovascular system, and the skin. Cutaneous lesions consist of small, asymptomatic, yellowish papules or larger coalescent plaques, typically located on the neck and the flexural areas. PXE is caused by mutations in the ABCC6 (ATP-binding cassette subfamily C member 6) gene that encodes a transmembrane ATP binding efflux transporter, normally expressed in the liver and the kidney; however, the exact mechanism of ectopic mineralization remains largely unknown. The histological examination of cutaneous lesions, revealing accumulation of pleomorphic elastic structures in middermis, is essential for the definitive diagnosis of PXE, excluding PXE-like conditions. PXE is currently an intractable disease; although the cutaneous findings primarily present a cosmetic problem, they signify the risk for development of ocular and cardiovascular complications associated with considerable morbidity and mortality. The purpose of this review is to present a comprehensive overview of this rare form of hereditary connective tissue disorders, focus on the pathogenesis, the clinical manifestation, and the differential diagnosis of PXE. Emphasis is also placed on the management of cutaneous lesions and treatment perspectives of PXE.

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Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease associated with a high physical and psychological burden. It is a disorder of the infundibular segment of the pilosebaceous unit, characterized by subcutaneous nodules, abscesses, sinus tracts and scar formation on the intertriginous and apocrine‐bearing areas. HS is quite rare in young and prepubertal children. It usually begins after puberty, but several reports of prepubertal HS onset have been described. These cases are strongly linked to hormonal disorders and genetic susceptibility. Specific guidelines for prepubertal patients are still lacking, so further studies are warranted to better delineate a tailored approach. This paper aims to summarize the most significant aspects, as well as the most recent information about the epidemiology, pathogenesis, clinical features, diagnosis, comorbidities and treatment of paediatric HS. In addition, we report our clinical experience in managing HS in a group of eight prepubertal patients based on systemic antibiotics (azithromycin) and zinc oral supplementation.

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until 1 week, in four patients (10%) until 2 weeks, and in two patients (5%) until 3 weeks after the patient was cured. The mean mite DNA load during these periods was only 4Á20% (95% confidence interval 0Á93-7Á47) of the peak DNA load, and none of these patients had clinical or microscopic recurrence of infestation. Therefore, scabies mite DNA may be detectable during the follow-up period after cure, but its presence may be the result of epidermal turnover to liberate mite DNA rather than relapse or treatment failure, especially if the DNA load is less than 10% of the peak load In conclusion, our study demonstrated that a nested realtime quantitative PCR assay targeting the cox1 gene of S. scabiei is useful not only for the diagnosis of scabies but also for post-treatment monitoring. This PCR assay may not completely replace traditional skin scraping and microscopic examinations in terms of time and cost, but it may help physicians who have not previously diagnosed scabies or used dermoscopy to diagnose scabies accurately. The assay may also be helpful in the diagnosis of patients with atypical symptoms and signs, and may be helpful in identifying cure or recurrence during post-treatment monitoring.