Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany

Abstract

Background Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available. Patients and methods We analysed data of 1.7 million children insured in the largest German health insurance company based on the ICD-10-GM classification for the year 2015. Data were adjusted to the general German population based on the data of the Federal Statistical Office for the year 2015. Results The prevalence of AIBD was calculated to 101.1/million children in 2015, resulting in about 1351 patients below the age of 18 years in Germany. The highest prevalence of all AIBD was seen for pemphigus vulgaris (30.5/million children) followed by linear IgA disease (24.5/million children) and bullous pemphigoid (4.9/million children). Conclusion Autoimmune bullous diseases in minors are scarce but should be taken into consideration in patients with pruritus and/or blisters and erosions on the skin and/or mucous membranes. Treatment is challenging, and due to the rarity of AIBD in minors, the management of these disorders in this patient population is best performed in specialized centres in a multidisciplinary approach, including paediatric dermatologists or dermatologists and paediatricians.

Spectrum of autoimmune bullous diseases among children in Kuwait

“…In a recent age-adjusted one-year (2015) population-based report from Germany from the database created by a German health insurance plan using ICD-10 codes, the prevalence rate of the AIBD among children was observed to be 101.1 cases per million populations and was interestingly highest for pemphigus variants (30.5/million/year) followed by LABD (24.5/million/year), and BP (4.9/million/year). 19 We had only two cases of rare pemphigus variants, including neonatal pemphigus and PSV-PDV and no cases of PV, PF, or EBA. Whether these differences are related to ethnic, genetic or environmental factors, is uncertain.…”

Epidemiology of Pemphigus

“…Pemphigus can arise in any age group with most patients aged between 45 and 65 years at the time of diagnosis ( Table 1 ). Outside the endemic areas, where up to 30% of patients have been reported to be younger than 20 years ( Aoki et al., 2015 ; Diaz et al., 1989 ), pemphigus is rare aged below 18 years ( Hübner et al., 2020 ; Mintz and Morel, 2011 ). In a recent study from Germany, only 0.6% of patients with PV were minors ( Hübner et al., 2020 , 2016 ).…”

“…Outside the endemic areas, where up to 30% of patients have been reported to be younger than 20 years ( Aoki et al., 2015 ; Diaz et al., 1989 ), pemphigus is rare aged below 18 years ( Hübner et al., 2020 ; Mintz and Morel, 2011 ). In a recent study from Germany, only 0.6% of patients with PV were minors ( Hübner et al., 2020 , 2016 ). The mean age of presentation in PV ranges between 36.5 years in Kuwait ( Nanda et al., 2004 ) and 72.4 years in Bulgaria ( Tsankov et al., 2000 ).…”

A rare case of childhood mucous membrane pemphigoid involving the oral and genital mucosa

“…20 Clinical and immunopathologic findings of various autoimmune blistering diseases in children are summarized in Table S1. 18,[21][22][23][24][25] Ocular involvement occurred in 9 of the 25 published cases of pediatric MMP, and may present clinically as chronic conjunctivitis, fibrosis on the conjunctival fornix, symblepharon, meibomian duct obstruction, keratoconjunctivitis sicca, and entropion that may be associated with corneal ulcers and trichiasis. 1,3,5,7 Continued ophthalmologic surveillance, even in the absence of ocular symptoms on disease onset, is necessary, since ocular lesions may manifest years later.…”