A Severe Case of Netherton Syndrome in a Filipino Child

Abstract

Objective. To present a rare case of Netherton Syndrome (NS) in a Filipino child.

Case. This is a case of an 11-year-old girl with elevated immunoglobulin E, trichorrhexis nodosa, and ichthyosis linearis circumflexa. She had dry skin with fine scaling at birth with recurrent pruritic, erythematous papules coalescing to plaques mainly on the face and extensors. The skin dryness turned to generalized redness with fine scaling, and the skin started getting tight. She could no longer completely extend both knees, but she was still able to walk. She also started having difficulty closing both eyes. She developed migratory serpiginous erythematous plaques with peripheral double-edged scaling. At six years old, she developed ulcers in the scalp, trunk, and extremities, which resulted in admission to our institution. She was managed for multiple skin infections, pneumonia, sepsis, seizure, severe malnutrition, joint contracture, atopy, and bilateral cicatricial ectropion.

Conclusion. Management of NS remains challenging. Common management options include emollients, topical corticosteroids, calcineurin, and protease inhibitor, and phototherapy while newer ones that need further validation include intravenous immunoglobulins and biologics such as infliximab. However, until specific recommendations are made, overall management for NS remains challenging. Regular multidisciplinary monitoring of the manifestations of NS is central to its management.