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

F Hübner, I R König, M M Holtsche, D Zillikens, R Linder, E Schmidt


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 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 1,351 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: AIBD 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.

Original languageEnglish
JournalJournal of the European Academy of Dermatology and Venereology : JEADV
Publication statusPublished - 2020


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