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Monilethrix Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 21, 2016
 

History

Monilethrix is a lifelong disease. Internodal fragility leads to breakage of hair and varying degrees of alopecia. Symptoms spontaneously regress during puberty and pregnancy, but the condition never disappears completely. Affected persons are generally healthy.

Keratosis pilaris is almost invariably associated with monilethrix. Sites of predilection are the scalp, nape of the neck, and extensor surfaces of the upper arm and thigh. No evidence suggests that keratosis pilaris can lead to the formation of beaded hair. Koilonychia is commonly associated with monilethrix. A wide variety of concomitant ectodermal defects are reported in the literature. Examples include dental abnormalities, juvenile cataracts, and some neurologic defects that appear as a cognitive deficiency or epilepsy.

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Physical

Lanugo hair usually appears normal at birth. Several months later, it is replaced by moniliform hair, which is dry, brittle, fragile, and lusterless. The hair breaks spontaneously to 0.5-2.5 cm. Broken short hair looks as if it has been burnt.

Monilethrix occurs mainly on the scalp, predominantly on the occiput and nape and occasionally on other scalp areas. Scalp involvement can be widespread or circumscribed. The eyelashes, eyebrows, pubic, axillary, and limb hair are occasionally involved.

Monilethrix is associated with follicular keratosis (horny follicular papules). Patients with monilethrix and a prolonged clinical course of several years' duration may have cutaneous atrophy (aplasia pilorum moniliformes [Latin]).

The absence of macroscopic beading should prompt repeated hair microscopy and not an automatic rejection of this diagnosis.[16] Dermatoscopy can be helping in making the diagnosis, especially with adequate hair sampling.

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Causes

See Pathophysiology.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Malgorzata Alexiewicz-Slowinska, MD Consulting Dermatologist, Group Medical Practice, Poznan, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Carrie L Kovarik, MD Assistant Professor of Dermatology, Dermatopathology, and Infectious Diseases, University of Pennsylvania School of Medicine

Carrie L Kovarik, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Dr. Marek Kazmierowski and Dr. Gosiunia Alexiewicz-Slowinsk, to the development and writing of this article.

References
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Lusterless, dry, brittle hair with alopecia and keratosis pilaris mainly on the occiput. The hair of this 4-year-old boy was never cut.
Photomicrograph shows the regular nodes and internodes of the hair shaft in monilethrix, or pili moniliformes (original magnification X200).
Photomicrograph shows the regular nodes and internodes of the hair shaft in monilethrix, or pili moniliformes (original magnification X200).
Monilethrix that affects the entire scalp.
Monilethrix that affects the entire scalp.
 
 
 
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