Hidradenitis suppurativa in a prepubertal case series: a call for specific guidelines

Abstract

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.

Hidradenitis suppurativa in paediatric patients: a retrospective monocentric study in Germany and review of the literature

“…This is highlighted in several studies, including our own, with the axilla and groin regions being most commonly involved. 1,4,25,26 Furthermore, the disease course seems to be similar in paediatric patients and adults, with the exception of the Danish case series where all five patients experienced mild HS. [1][2][3]11,25,26 Obesity is likely associated with a more serious disease progress as well as precocious puberty.…”

“…Genetic susceptibility, obesity/metabolic syndrome and smoking are considered as promoting factors. 1,3,4 A positive family history has been associated with an early-onset disease. Mutations in the csecretase gene as well as aberrations in the Notch signalling cascade have been demonstrated to be implicated in the inflammatory process.…”

“…5 In paediatric patients an association with hormonal dysregulation, premature adrenarche and adrenal hyperplasia have also been documented. [4][5][6] The disease presents usually after puberty, typically in the second and third decade of life, with a slight female predominance. 1,[3][4][5] Prevalence of HS varies among different older studies from 0.00033 to 4%, [7][8][9] and a mean disease incidence of 6.0 per 100 000 person-years has been reported.…”

“…[4][5][6] The disease presents usually after puberty, typically in the second and third decade of life, with a slight female predominance. 1,[3][4][5] Prevalence of HS varies among different older studies from 0.00033 to 4%, [7][8][9] and a mean disease incidence of 6.0 per 100 000 person-years has been reported. 1 Recent large epidemiological analysis in Germany and the USA revealed a HS prevalence of 0.03% and 0.053%, respectively, 7,10 and an incidence of 0.03% in 2015 with highest rates among women and in the age group of 30-40 years.…”

“…3,6,11,12 A disease onset at prepubertal patients (10 years old or younger) with or without signs of precocious puberty or premature adrenarche has also been presented in case reports or small case series. 4,[13][14][15][16][17] In this study, we evaluated the clinical characteristics of HS among children and adolescents in a large monocentric patients group in Germany.…”