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Uncombable Hair Syndrome

Pili Trianguli Et Canaliculi


Presentation

  • We report the case of a 2(1/2) year old-girl presenting isolated uncombable hair syndrome suspected clinically and confirmed by scanning electron microscopy.[ncbi.nlm.nih.gov]
Juvenile Cataract
  • The relationship to a previously described syndrome of uncombable hair in combination with retinal dystrophy, juvenile cataract, and brachydactyly, in which both hair and skeletal abnormalities are common, remains to be further elucidated.[ncbi.nlm.nih.gov]
  • Several conditions are associated with uncombable hair, such as ectodermal dysplasia, retinal dysplasia/ pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia.[doi.org]
  • Several conditions have been reported to occur with UHS such as ectodermal dysplasia, retinal dysplasia, retinal pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, phalango-epiphyseal dysplasia, alopecia[orpha.net]
Enamel Anomaly
  • Several conditions are associated with uncombable hair, such as ectodermal dysplasia, retinal dysplasia/ pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia.[doi.org]
  • Several conditions have been reported to occur with UHS such as ectodermal dysplasia, retinal dysplasia, retinal pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, phalango-epiphyseal dysplasia, alopecia[orpha.net]
  • Several conditions are associated with uncombable hair, such as ectodermal dysplasia, retinal dysplasia/pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia.[researchers.dellmed.utexas.edu]
Alopecia
  • A 7-year-old female presented with messy, difficult to manage scalp hair and mild, diffuse alopecia. Hair pull specimens, diagnostic for loose anagen hair syndrome, also showed hair shaft abnormalities described in the uncombable hair syndrome.[ncbi.nlm.nih.gov]
  • , and psoriatic alopecia, and chemotherapy-induced alopecia.[books.google.es]
  • Uncombable hair may be idiopathic or associated with ectodermal dysplasia, progressive alopecia, atopic eczema, dental dysplasia, or ichthyosis vulgaris A condition in which the hair shaft is triangular in cross-section and often has a groove; seen in[medical-dictionary.thefreedictionary.com]
  • Several conditions have been reported to occur with UHS such as ectodermal dysplasia, retinal dysplasia, retinal pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, phalango-epiphyseal dysplasia, alopecia[orpha.net]
Hirsutism
  • It was likely first described in the book 'Les Velus' (the Hirsute) where an affected individual was described as having mop hair [7] .[ijcasereportsandimages.com]
  • Coarse face and body hirsutism are additional features.[mendelian.co]
Freckles
  • Moreover, eight cafè-au-lait macules larger than 5 mm on the trunk and the limbs, and freckles in the axillary regions were observed. Neurologic examination showed some difficulties in motor organization and dyslalia.[ncbi.nlm.nih.gov]
Suggestibility
  • It has been suggested that the condition may result from premature keratinization of the inner root sheath.[en.wikipedia.org]
  • We suggest that dysmorphic hair shafts observed on our patient account for her clinically unmanageable hair. Pertinent clinical, pathologic, and diagnostic features of both syndromes are reviewed.[ncbi.nlm.nih.gov]
  • Both recessive and dominant transmission with incomplete penetrance have been suggested as modes of inheritance. We report the occurrence of this condition in a young girl, her brother, and her father.[scholars.northwestern.edu]
  • Our findings suggest that this disorder could represent a tricho-odontal subtype of ectodermal dysplasia.[unboundmedicine.com]

Treatment

  • Management and treatment UHS spontaneously regresses in late childhood and only gentle care including conditioners, soft brushes and avoidance of harsh treatments cares of the hair are recommended.[orpha.net]
  • There is no definitive treatment for uncombable hair syndrome, but the condition usually improves or resolves on its own with the onset of puberty.[rarediseases.info.nih.gov]
  • There is no definitive treatment, and most cases improve with the onset of puberty.[ncbi.nlm.nih.gov]
  • Uncombable Hair Syndrome Treatment There is no definitive treatment for uncombable hair syndrome, but the condition does improve or resolves on its own, with the onset of puberty.[medicaldaily.com]

Prognosis

  • Prognosis - Uncombable hair syndrome Not supplied. Treatment - Uncombable hair syndrome Not supplied. Resources - Uncombable hair syndrome Not supplied.[checkorphan.org]
  • Read: Boy, 10, Spends 2 Years Growing Hair He Donated to Make Wig for Friend, 12, With Alopecia “[There is] no cure for this, but the prognosis of this is really good,” Dr. Bree said.[clickondetroit.com]
  • […] growth after 4 months of supplementation. [6] Last updated: 12/19/2016 Uncombable hair syndrome (UHS) usually resolves on its own (spontaneously) by adolescence. [6] Treatment is usually not necessary. [1] In the cases, where UHS is part of syndrome, the prognosis[rarediseases.info.nih.gov]

Etiology

  • Etiology The stiffness and brightness of hair are supposed to result from a misshapen dermal papilla inducing an anomaly in the keratinization of the inner root sheath.[orpha.net]
  • Etiology The genetic basis of the disorder/etiology is unknown.[dermatologyadvisor.com]
  • McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J.[books.google.com]

Epidemiology

  • Summary Epidemiology Prevalence is unknown. Clinical description Onset is usually between ages 3 months and 12 years.[orpha.net]
  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.de]
Sex distribution
Age distribution

Pathophysiology

  • All together, these findings elucidate the molecular genetic causes of UHS and shed light on its pathophysiology and hair physiology in general. Copyright  2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.[ncbi.nlm.nih.gov]
  • Pathophysiology The irregular and changing shapes of the hair cross section prevent adjacent hairs from lying flat or forming locks, and this accounts for the stand away and spangled appearance.[dermatologyadvisor.com]

Prevention

  • Arsenic poisoning Arterial insufficiency Burns Cutaneous T-cell lymphoma TopDissecting cellulitis of the scalp Dissecting cellulitis of the scalp Exfoliative dermatitis Fungal infections Hodgkin’s disease Hypopituitarism Hypothyroidism Hypothyroidism Prevention[checkorphan.org]
  • Images: SWNS Read more: How your hair changes during your pregnancy Time-saving tricks to try with your toddler How to prevent head lice[madeformums.com]
  • Their mother can also only wash their hair once a week to prevent it becoming matted. She said: 'We hardly use any shampoo, as that makes the frizz worse. I'll use a lot of conditioner, though. We get through a bottle a week.'[dailymail.co.uk]
  • Pathophysiology The irregular and changing shapes of the hair cross section prevent adjacent hairs from lying flat or forming locks, and this accounts for the stand away and spangled appearance.[dermatologyadvisor.com]

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