Pili torti

Pili torti
Menkes disease3.jpg
Menkes disease

Alternate names

Twisted hair; Coarse, dry, lusterless hair which breaks off easily

Definition

Pili torti is a rare hair condition characterized by fragile hair. In pili torti hair has a flattened shaft with clusters of narrow twists at irregular intervals.

Cause

It can occur alone or as part of other diseases like ectodermal dysplasias, Menke disease, Bjornstand syndrome, or Bazex syndrome. Acquired cases of pili torti may be associated with anorexia nervosa, malnutrition, oral retinoid treatment, or inflammatory scalp conditions (e.g., cutaneous lupus erythematousus).

Inheritance

Some cases may be inherited in autosomal dominant or autosomal recessive patterns, while others are acquired.

Signs and symptoms

• This condition is characterized by fragile hair.
• In pili torti hair has a flattened shaft with clusters of narrow twists at irregular intervals.
• In the inherited form, symptoms tend to be present from early childhood.
• If pili torti is detected, it is necessary to investigate possible neurological disorders, hearing loss, and defects in the hair, nails, sweat glands and teeth.

Treatment

• In acquired pili torti, treatment involves stopping the exposure to the causative agent (e.g., to oral retinoids) or condition (e.g., improving diet).
• There is no specific treatment for the inherited form of pili torti.
• It may improve spontaneously after puberty.
• If pili torti is detected, further evaluation to investigate possible neurological disorders, problems with hair, teeth or nails (ectodermal disturbances) and hearing loss is mandatory.
• It is generally recommended that people with pili torti try to avoid trauma to the hair.
• Suggestions include, sleeping on a satin pillowcase, avoiding excessive grooming, braiding, heat treatments, dying and coloring, reducing exposure to sunlight (wear a hat), using gentle shampoos diluted in warm water, adding conditioner to freshly washed hair, avoiding use of a hair dryer (or using it on cool setting), and avoiding oral retinoids (e.g., isotretinoin, acitretin) if possible.
• Some individuals with pili torti choose to wear a wig.