Granulocyte and monocyte apheresis can control juvenile generalised pustular psoriasis with mutation of the IL36RN gene

Abstract

Patients with 'deficiency of the interleukin-36 receptor antagonist' (DITRA), caused by mutation of the IL36RN gene, exhibit psoriatic phenotypes, typically generalised pustular psoriasis (GPP). We report a paediatric case with DITRA, whose cutaneous lesions varied from psoriasis vulgaris in infancy to annular pustular psoriasis with acute exacerbation to GPP at 13 years of age. Conventional systemic treatments for GPP, which include oral retinoids, cyclosporine and methotrexate, are controversial in paediatric cases, because of their adverse effects and uncertain long-time consequences. Granulocyte monocyte apheresis, a process associated with few adverse events, promptly controlled our paediatric case of GPP, and has potential as a suitable alternative treatment for paediatric patients with DITRA.

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