Pili torti Q84.12

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 24.11.2024

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Synonym(s)

Beare Syndrome; Galewsky disease; Torsion Hair; Trichokinesis; Trichotortosis; twisted hair; Twisted hair

History
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Schulz, 1900; Ronchese, 1932

Definition
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Autosomal dominant or autosomal recessive, but apparently also acquired form of hair malformation with mostly flattened hairs that rotate at angles of 90°, 180° or 360° around their longitudinal axis. It is usually first observed in childhood.

Etiopathogenesis
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Unknown; occurring in the context of ectodermal syndromes.

Manifestation
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Occurs mainly in girls with fine light hair, aged 2 to 5 years. A postpubescent variant, in which mental retardation occurs simultaneously, is described as Beare's syndrome.

Localization
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In childhood on the entire scalp hair, later mainly in the occipital and temporal region.

Clinical features
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Diagnosis
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Microscopic: rotation of the hairs by 90 ° or 180 ° around their longitudinal axis at intervals of 2-10 mm; band-shaped flattening of the hairs.

Therapy
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A causal therapy is not known. Extensive regression until the hair malformation disappears completely after puberty is possible.

Note(s)
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Most people experience sporadic hair twists, increasingly as a result of hair cosmetic procedures. Physiologically, they are found in underarm and genital hair.

Literature
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  1. Beare JM (1952) Congenital pilar defect showing features of pili tori. Br J Dermatol 64:366-372.
  2. Dishop MK et al (2009) Pathologic changes of skin and hair in ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome. Am J Med Genet A.149A:1935-1941
  3. Pietrzak A et al (2015) Steatocystoma multiplex with hair shaft abnormalities. J Dermatol doi: 10.1111/1346-8138.12837
  4. Ronchese F (1932) Twisted hairs (pili torti), Arch Derm Syph 26: 98-108
  5. Srinivas SM et al.(2013) Netherton syndromes with pili torti. Int J Trichology 5:225-226

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 24.11.2024