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Last Updated: May 12th, 2008 - 03:38:23 |
Title: Change in the Color and Texture of Hair
Presenter: Scott A. Smith, D.O.; Mary Veremis-Ley, D.O.; Michelle L. Endicott, D.O.; Melissa Camouse, D.O.; Mary A. Evers, D.O.
Dermatology Program: University Hospitals of Cleveland, Richmond Heights
Program Director: Monte Fox, DO
Submitted on:
Aug 1, 2004
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CHIEF COMPLAINT:
A now,2 year-old girl, presented to the clinic at the age of 11 months. She had a 4-month history of change in the color and texture of her hair. Per the patient’s mother, her hair had gradually gotten lighter, kinkier, and more difficult to comb. Mother denies any hair loss or breakage. Patient is otherwise healthy. Growth and development have been normal. Patient takes no medicines. The patient’s 1 year-old sister is now experiencing similar complaints. Patient’s great aunt had similar hair complaints as a child, which subsequently improved with her age.
CLINICAL HISTORY:
Signs and symptoms:
Previous Treatment:
Other information:
PHYSICAL EXAM:
Involving the entire scalp, there are coarse, blonde hairs that are standing away from the scalp. The hairs are resistant to combing flat.
LABORATORY TESTS:
DERMATOHISTOPATHOLOGY:
Light microscope shows normal hair shafts, without evidence of grooves.
Scanning electron microscope, on cross sectioning, reveals triangular shaped hairs with indentation, giving the hairs a reniform to heart shape.
DIFFERENTIAL DIAGNOSIS:
1. Pili torti
2. Uncombable hair syndrome
3. Monilethrix
4. Trichorrhexis nodosa
5. Trichorrhexis invaginata
SCROLL DOWN FOR ANSWER AND DISCUSSION.
CORRECT DIAGNOSIS:
UNCOMBABLE HAIR SYNDROME (AKA Spun-glass hair, Pili trianguli et canaliculi, or Cheveux incoiffables)
DISCUSSION:
This syndrome is a rare disorder with an interesting clinical presentation. The syndrome has been reported in both inherited (autosomal dominant and recessive) and sporadic forms. The presentation is usually within the first year of life. The hair is characterized by bundles of scalp hair arranged in all different directions that resist being brushed or combed. The hair is often a silvery, blonde color with a spangled or glistening appearance. Only scalp hair is affected. There are usually no associated neurological or mental deficits. Most common association is with ectodermal dysplasia. Diagnosis is made by scanning electron microscope.
TREATMENT:
No proven effective therapy. Increased hair length may allow the hair to be more manageable. Hair quality may improve with age. There is a report of a few children who were treated with biotin, of which 3 responded
REFERENCES:
1. Hicks J, Metry DW, Barrish J, Levy M. Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: brief review and role of scanning electron microscopy in diagnosis. Ultrastruct Pathol. 2001 Mar-Apr;25(2):99-103.
2. Itin PH, Buhler U, Buchner SA, Guggenheim R. Pili trianguli et canaliculi: a distinctive hair shaft defect leading to uncombable hair. Dermatology. 1993;187(4):296-8.
3. Zegpi M, Roa I. The uncombable hair syndrome. Arch Pathol Lab Med. 1987 Aug;111(8):754-5.
Additional Comment:
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