Prognosis and Management of Congenital Hair Shaft Disorders with Fragility-Part I

Pediatr Dermatol. 2016 Sep;33(5):473-80. doi: 10.1111/pde.12894. Epub 2016 Jun 13.


Hair shaft disorders are characterized by congenital or acquired abnormalities of the hair shaft. The objective was to review the literature regarding the prognosis and treatment options of hair shaft disorders. We used keywords in the search engines PubMed and Medline to identify all publications in the English language related to the prognosis and management of hair shaft disorders. Data were extracted from 96 articles that met search criteria. Findings were limited to case reports and small case series, as no studies were found. Disorders that improve in childhood include pili torti, trichorrhexis invaginata, wooly hair, and pili trianguli et canaliculi. Others, such as trichorrhexis nodosa, monilethrix, pili annulati, and pili bifurcati improve with minoxidil. Oral retinoids have improved hair abnormalities in trichorrhexis invaginata and monilethrix. There is no specific treatment for congenital hair shaft abnormalities. Gentle hair care is the mainstay of care for hair shaft disorders associated with fragility. Practices for gentle care include no brushing, backcombing, chemical products, tight braids, heat exposure, or mechanical grooming. Any inherited or congenital disorder requires genetic counseling as part of management.

Publication types

  • Review

MeSH terms

  • Alopecia Areata / congenital
  • Alopecia Areata / diagnosis
  • Alopecia Areata / epidemiology
  • Alopecia Areata / therapy
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Disease Management
  • Female
  • Hair / abnormalities*
  • Hair Diseases / congenital*
  • Hair Diseases / diagnosis
  • Hair Diseases / epidemiology
  • Hair Diseases / therapy*
  • Humans
  • Incidence
  • Male
  • Prognosis
  • Risk Assessment