Netherton's Syndrome in Two Sisters
June 2002
in “
PubMed
”
TLDR Two sisters with Netherton's syndrome had skin and hair issues, needing special diet and ointments.
Two sisters, aged 4.5 years and 6 months, were diagnosed with Netherton's syndrome, characterized by serious erythrodermia, poor hair growth, and poor overall growth despite a hypoallergenic diet. The diagnosis was confirmed by the presence of ichthyosis linearis circumflexa and trichorrhexis invaginata (bamboo hair) observed in a hair shaft. This autosomal recessive condition involved defective production or maintenance of the stratum corneum and was often associated with an atopic constitution. Treatment included skin ointments and a high-energy diet to compensate for protein loss through the skin.