By Gary M. White, MD
Pili torti (PT) is a rare structural abnormality of the hair in which the hair is flattened and twisted at regular intervals along the long axis. It may occur in isolation or in conjunction with various conditions outlined below [An Bras Dermatol. 2015 May-Jun; 90(3 Suppl 1): 29–31]:
- Beare syndrome (scarce beard and body hair, alopecia after puberty in African descent individuals)
- Ronchese syndrome (leukonychia, ichthyosis, keratosis pilaris, nail dystrophy, and dental abnormalities)
- Björnstad Syndrome (sensorineural deafness)
- Menkes syndrome (growth retardation of early onset, epilepsy and focal brain degeneration, low serum copper and ceruloplasm)
- Rapp-Hodgkin syndrome (anhidrotic ectodermal dysplasia, cleft lip, cleft palate, hypodontia, nail abnormalities, hypospadias in males)
- Trichodysplasia-xeroderma (skin xerosis, alopecia, hypotrichosis, trichorrhexis nodosa)
- Trichothiodystrophy (ichthyosis, developmental delay, brittle hair and photosensitivity)
The hair is sparse, fine and fragile. It may shimmer in reflected light. The eyebrows and eyelashes may be involved. In isolated PT, the onset of abnormal hair is usually between 2 months and 2 years of age, but may be present at birth. Patchy alopecia is seen, especially in areas of increased friction. A girl with blond hair, lighter than their unaffected siblings is typical. On microscopic exam, the hair appears twisted.
If the trichogram shows pili torti then workup should include among other things physical exam and documentation of normal development, hearing test, serum copper, and ceruloplasmin.
See fragile hair.
There is no specific treatment, although the condition may improve over time, particularly after puberty. The parents should be counseled to avoid excessive brushing, to wash the hair with a gentle shampoo, and to avoid the use of heat.
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