Uncombable Hair Syndrome

Overview:

Uncombable hair syndrome, also known as pili trianguli et canaliculi or ptychogenic dwarfism, is a rare genetic disorder that affects the hair of the scalp. It is characterized by the presence of dry, brittle, and unmanageable hair that is difficult to comb or style. The hair shafts are twisted and misshapen, and may have a triangular cross-section, giving them a spiky or fluffy appearance.

Causes:

The exact cause of uncombable hair syndrome is not fully understood, but it is thought to be inherited in an autosomal dominant manner. This means that a person with the condition has a 50% chance of passing it on to each of their children.

Symptoms:

Symptoms of uncombable hair syndrome may include:

Dry, brittle, and unmanageable hair that is difficult to comb or style

Hair shafts that are twisted and misshapen, with a triangular cross-section

Spiky or fluffy appearance of the hair

Hair that is lighter in color than the surrounding hair

Diagnosis:

Diagnosis of uncombable hair syndrome is typically made based on the appearance of the hair and a thorough medical history. A dermatologist or other healthcare professional may also perform a scalp biopsy to confirm the diagnosis.

Treatment:

There is no specific treatment for uncombable hair syndrome, and management of the condition is largely supportive. This may include the use of moisturizing shampoos and conditioners, gentle combing techniques, and regular trims to remove split ends. In severe cases, a wig or hairpiece may be recommended.

Prognosis:

The prognosis for individuals with uncombable hair syndrome is generally good, and most people with the condition lead normal, healthy lives. However, the condition may cause social and psychological difficulties, as the affected person may feel self-conscious about their appearance. It is important for individuals with uncombable hair syndrome to receive supportive care from their healthcare team and loved ones.

 FAQs

  • Patients with rare hair and scalp disorders are often referred to dermatologists for help in diagnosis and management recommendations. There are over 3000 different disorders of the skin, hair, and nails and dermatologists uniquely have the training and expertise to diagnose these conditions. While uncombable hair syndrome is generally an isolated finding that improves after adolescence, what may appear to be uncombable hair syndrome could also be woolly hair nevus which can be associated with ocular, auditory, dental, skeletal, and kidney anomalies. A dermatologist can reassure patients and parents when appropriate or help patients identify other specialists for a complete evaluation.

  • There is no widely established therapy for uncombable hair syndrome. Dermatologists typically recommend gentle hair care practices. Patients should avoid perms, chemical relaxers, excessive brushing, and heat. Conditioners, detangling creams, soft brushes may help improve the manageability of hair. One case report has shown biotin to be helpful, however, this has not been replicated and is not widely accepted as an effective treatment.

  • While the condition generally improves after adolescence and is not associated with any other medical complications, young children may experience psychosocial problems from bullying or teasing from other children given the different appearance of hair. Parents can play an important role in helping children cope and improve their self esteem.

  • One case report has shown biotin to be helpful, however, this has not been replicated and is not widely accepted as an effective treatment. There may be an opportunity for cosmetic (non-medical) therapies to help improve the manageability of hair.