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Monilethrix - Hair and Scalp
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Monilethrix - Hair and Scalp

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Contributors: Mary Gail Mercurio MD, Jeffrey D. Bernhard MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Monilethrix is a hair shaft disorder characterized by nodes along the hair shaft. The elliptical nodes occur at regular intervals and are connected by nonmedullated tapered constrictions (internodes). Internodal fragility leads to hair breakage and alopecia that can be dramatic. The extent of involvement can vary widely; monilethrix most commonly affects the nape of the neck and occiput but may involve the entire scalp as well as facial and body hair. Inheritance can be autosomal dominant (due to a keratin 81, 83, or 86 mutation) or autosomal recessive (due to a desmoglein 4 mutation). 

Monilethrix usually occurs in early childhood but can occur after puberty. Affected individuals are usually born with normal hair, which is replaced with short, fragile, brittle hair in the first months of life.

For more information, see OMIM.

Codes

ICD10CM:
Q84.1 – Congenital morphological disturbances of hair, not elsewhere classified

SNOMEDCT:
69488000 – Beaded hair

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Trichorrhexis nodosa – absence of recurrent nodes on microscopic evaluation
  • Trichorrhexis invaginata – absence of recurrent nodes on microscopic evaluation
  • Pili torti – absence of recurrent nodes on microscopic evaluation
  • Pseudomonilethrix – nodes along the hair shaft are at irregular intervals, and because usually due to artifact, findings are not typically present with a repeat specimen

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 03/29/2017
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Monilethrix - Hair and Scalp
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Monilethrix : Brittle hair, Short broken hairs, Widespread non-scarring alopecia, Normal hair at birth
Clinical image of Monilethrix
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