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

Abstract

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.

“My Daughter Has Thin and Short Hair”

“…In our patient, only the scalp was affected, but in extensive cases, there may be eyebrow, eyelash, and nail involvement. There is usually intrafamilial variability and the disease may range from almost normal scalp to total alopecia [3]. When performed, as in our case, trichoscopy shows regular variations in the diameter of the hair shaft with elliptical dilations (nodes) and constrictions (internodes) [4].…”

“…Monilethrix is a rare autosomal dominant disease of the hair shaft characterized by alopecia as a result of hair breakage [2]. Clinically, there is normal hair at birth but, within the first months of life, a characteristic moniliform aspect of the hair develops [3]. Perifollicular erythema and follicular hyperkeratosis are also common.…”

Unusual hair findings in a child with cardiofaciocutaneous syndrome

“…Trichorrhexis nodosa is a common hair shaft disorder that presents as sparse, thin hair diagnosed with an appearance suggestive of the ends of two brushes pushed together on light microscopy. In general it accompanies a genetic or sporadic disease such as Tay syndrome, arginine‐succinic aciduria, Menkes disease, biotin deficiency . To the best of our knowledge, this is the first report of a CFCS case having trichorrhexis nodosa and trichoptilosis as ectodermal findings.…”

Evaluation of Patients with Alopecia

“…Hair shaft disorders have a particular aspect: beaded shaft in monilethrix, brush fractures in trichorrhexis nodosa, shaft nodes in trichorrhexis invaginata, twisted shafts in pili torti [35,47,48].…”

“…Trichonodosis is characterized by knotted hair on the distal portion of the shaft, sometimes due to mechanical factors such as scratching or combing. In trichoptilosis, hairshafts become dry and exhibit splitting or fraying of the hair due to excessive exposure to chemical, thermal or hairdressing procedures [48]. If the hair shaft is apparently normal in microscopy, other tests can be performed, such as: KOH mount or root exam [59].…”