A new hair shaft defect, helical hair, is presented. This hair abnorma
lity was found to accompany trichorrhexis invaginata and pill torti in
an infant with Netherton's syndrome. The patient's main clinical feat
ures included erythroderma since birth, failure to thrive, recurrent i
nfections, hepatosplenomegaly, lymphadenopathy, eosinophilia, hypergam
maglobulinemia, and high serum IgE levels, A possible mechanism for th
e formation of helical hair is reviewed.