Hypohidrotic ectodermal dysplasia as a rare cause of chronic rhinitis in children

Monika Jabłońska-Jesionowska, *Lidia Zawadzka-Głos

Summary
Introduction. Chronic rhinitis in children may have different causes, both local – with changes being present only in the nasal cavity – or systemic, with nasal congestion as one of the symptoms of a bigger clinical picture.
Aim. The aim of this study was to draw attention to a very rare congenital cause of chronic rhinitis in children – which is hypohidrotic ectodermal dysplasia.
Material and methods. A 6-month-old boy was admitted to the Department of Pediatric Otolaryngology of Warsaw Medical University due to chronic nasal obstruction present from birth. Clinical investigation included anterior and posterior rhinoscopy and fiberoscopy of nasopharynx. The MRI was also performed before admission. Complete blood count, serum iron level, serum thyroid hormones and level of IgG, IgA, IgM were examined to exclude anaemia, ozaena and hypothyroidism. Antinuclear antibodies (ANA) and antineutrophil cytoplasmic antibodies (ANCA) tests were also ordered to exclude granulomatosis with polyangiitis. Next, a mucosal biopsy of the nasal cavity was performed to exclude primary ciliary dyskinesia. Allergic prick tests were also performed.
Results. After genetic tests, hypohidrotic ectodermal dysplasia was diagnosed.
Conclusions. 1. Every case of chronic nasal congestion in children requires not only adequate treatment, but also thorough clinical investigation. 2. Nasal obstruction may be due to local causes, systemic diseases and genetic disorders. 3. Hypohidrotic ectodermal dysplasia is a very rare genetic disorder that causes severe, even life threatening symptoms, one of which is chronic rhinitis.

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