Netherton syndrome (OMIM #256500) is an autosomal recessive disorder, characterized by congenital ichthyosiform erythroderma, atopic diathesis and trichorrhexis nodosa (3).
A unique case of trichorrhexis nodosa: 'bamboo hairs'.
The hair samples also showed variation, with some hairs showing one half of an area of trichorrhexis
at one end and trichoptilosis with widely splayed fibres and simple knots.
Netherton syndrome is an autosomal recessive disorder of cornification characterized by the triad of congenital ichthyosiform erythroderma/ichthyosis linearis circumflexa, trichorrhexis
invaginata (bamboo hair), and an atopic diathesis.
Other reported hair abnormalities of trichoclasia and Trichorrhexis
nodosa by Claudio Ponticelli et al (10) were not seen in our study.
Bazex-Dupre-Christol syndrome (BDCS) is clinically characterized by multiple basal cell carcinomas of the face occurring mainly during the second and third decade of life, follicular atrophoderma predominantly of the dorsum of hands and feet and generalized hypotrichosis, sometimes with pili torti and trichorrhexis
* Microscopy of the hair: twisted hair (pili torti) with fractures of the hair shafts (trichorrhexis
Bu seklin klinik ozellikleri; mental ve motor gerilik, tekrarlayan konvulziyon, bilinc degisikligi, karaciger buyuklugu, deri lezyonlari ve "trichorrhexis
invaginati is a genetic disease characterized by a segment of bulbous, dilated hair enfolded into a concave hair terminal, recalling the appearance of a bamboo node.
These include ringed or banded hairs (pili annulati) (Figures 77 and 78), conspicuous nodes (trichorrhexis
nodosa), or regular diameter fluctuations (monilethrix).
The hair is difficult to comb and often not fragile, except when associated with trichorrhexis
On hair microscopy Trichorrhexis
nodosa was observed.