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Pathogenesis in trichorrhexis invaginata (bamboo hair)
The Journal of Investigative Dermatology
|July 1, 1984
Summary
Netherton's syndrome causes hair anomalies like trichorrhexis invaginata due to a soft hair cortex. This softness, linked to incomplete protein cross-linking, leads to hair shaft invagination.
Area of Science:
- Dermatology
- Genetics
- Biochemistry
Background:
- Netherton's syndrome is characterized by specific hair anomalies.
- Trichorrhexis invaginata is a hallmark hair abnormality in Netherton's syndrome.
Purpose of the Study:
- To investigate the formation mechanism of trichorrhexis invaginata in Netherton's syndrome.
- To analyze hair shaft structural changes at the microscopic and ultrastructural levels.
Main Methods:
- Scanning electron microscopy (SEM) and light microscopy (LM) of plucked hairs and biopsy specimens.
- Transmission electron microscopy (TEM) to examine hair follicle and shaft ultrastructure.
- Histochemical staining for sulfhydryl (-SH) and disulfide (S-S) groups using DACM.
Main Results:
- SEM revealed trichorrhexis invaginata, pili torti, and other hair abnormalities.
- LM and TEM showed invaginated hair cuticle and cortical changes, including cleavages and electron-dense deposits.
- Histochemical analysis indicated increased -SH groups and incomplete S-S bond formation in affected hair cortex.
Conclusions:
- Trichorrhexis invaginata results from a soft hair cortex in the keratogenous zone.
- Cortical softness is attributed to incomplete conversion of -SH to S-S linkages in hair proteins.
- These findings elucidate thePathogenesis of hair shaft abnormalities in Netherton's syndrome.