Spectrum of autoimmune bullous diseases among children in Kuwait

Arti Nanda 1, Viktor Lazarevic 1, Jihan M Rajy 1, Iman M Almasry 1, Humoud AlSabah 1, Atlal AlLafi 1

Affiliations

01 January 2021

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doi: 10.1111/pde.14368

Abstract

Background: Autoimmune bullous diseases (AIBD) are rare among children. The data describing the overall spectrum and prognosis of pediatric AIBD (pAIBD) are scarce, and there are no established treatment guidelines.

Objectives: The present study examined the spectrum, clinical characteristics, and long-term prognosis of pAIBD in a tertiary care pediatric dermatology unit.

Methods: Retrospective records of all pAIBD cases (<18 years) registered over a span of 28 years were analyzed.

Results: Records of 23 cases of pAIBD, including 16 boys and 7 girls, were reviewed. They constituted 8.5% of total AIBD patients from all age groups. Ninety-one percent of patients were of Arab ethnicity. Linear IgA bullous dermatosis was the most prevalent AIBD followed by bullous pemphigoid, bullous lupus erythematosus, and pemphigus variants (pyostomatitis-pyodermatitis vegetans and neonatal pemphigus). The mean age of onset and diagnosis was 6.4 and 7.7 years, respectively. Systemic treatments, including systemic corticosteroids and dapsone, were required in most cases. Intravenous immunoglobulin G (IVIG) was also utilized as second-line therapy. The mean follow-up period was 76 months with 90% of the patients in complete remission.

Conclusions: AIBD pose a great challenge among children both in diagnosis and treatment. This study highlights the ethnic variability and underscores the need for additional similar, international studies to achieve a better understanding of the burden related to pAIBD and help establish treatment guidelines.

Keywords: autoimmune bullous diseases; children; prognosis.

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