Uncombable hair syndrome

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Classification according to ICD-10
Q84.1 Congenital morphological disorders of hair, not elsewhere classified
ICD-10 online (WHO version 2019)
The Struwwelpeter , best known literary figure with the uncombable hair syndrome.
Heinrich Hoffmann (1858): He didn't let his hair be combed.

The syndrome of uncombable hair ( Latin: Pili trianguli et canaliculi ), also known as the symptom of hair that cannot be styled , Struwwelpeter syndrome or glass wool hair , is a rare structural abnormality of the scalp hair . As the name of the syndrome already describes, it means that the scalp hair cannot be styled.

Description and diagnosis

Uncombable hair syndrome is characterized by dry, frizzy scalp hair that cannot be combed. The hair is basically disheveled ("struwwelig"), but not brittle, mostly silver-blonde and slowly growing. The syndrome can manifest itself in the period from the first few weeks of life to puberty . In most cases, it occurs in early childhood.

The non-combability is caused by an arbitrary arrangement of the tufts of hair, which in turn is due to the structural hair structure. The pili trianguli et canaliculi (differently deformed hair shafts, partly deformed by two to three deep elongated notches), which are typical for this syndrome, can best be seen in cross-section under a light microscope . To do this, the hair embedded in paraffin is cut across. The clinical diagnosis can be safely confirmed with a scanning electron microscope image. While "normal" hair has a round cross-section, the hair on the head in the syndrome of uncombable hair is triangular to kidney-shaped over its entire length. At least 50% of the scalp hair shows this deformation and only the scalp hair is affected.

Glass wool hair usually appears as an independent syndrome. However, it can also be associated with other physical anomalies, such as ectodermal dysplasia , retinopathia pigmentosa , juvenile cataract , polydactyly , anomalies in tooth enamel .

In contrast to these syndromes, the uncombable hair syndrome is not associated with any other physical, neurological or psychological abnormality.

In the Anglo-Saxon specialist literature, the syndrome of uncombable hair is usually referred to as spun glass hair ("glass wool hair ") or uncombable hair syndrome ("non-manageable hair syndrome") or with the French term cheveux incoiffables ("not manageable hair") .

etiology

Hereditary factors as well as sporadic forms have been described as the cause of the glass wool hair. The hereditary forms are mainly autosomal - dominant , but also autosomal recessive inheritance - each with different penetrance .

Initial description

The uncombable hair syndrome was first described in 1973 by A. Dupré and colleagues as Cheveux incoiffables and in the same year by J. Stroud and A. Mehregan as Spun glass hair .

Individual evidence

  1. ↑ GN Pereira: Do you know this syndrome? (PDF; 257 kB) In: Anais Brasileiros de Dermatologia 81, 2006, pp. 185–188.
  2. ↑ B. Korge: New and proven in the diagnosis of hair diseases. In: Der Hautarzt 54, 2003, pp. 699–702. doi : 10.1007 / s00105-003-0571-9
  3. ↑ J. Hicks et al.: Uncombable hair (cheveux incoiffables, pili trianguli et canaliculi) syndrome: brief review and role of scanning electron microscopy in diagnosis. In: Ultrastruct Pathol 25, 2001, pp. 99-103. PMID 11407534
  4. ↑ a b C. Rieubland et al.: Uncombable hair syndrome: a clinical report. In: Eur J Med Genet 50, 2007, pp. 309-314. PMID 17526443
  5. ↑ A. Zanca include: Ancient observations of "Uncombable hair syndrome". In: International Journal of Dermatology 32, 1993, p. 707.
  6. ↑ M. Zegpi and I. Roa: The Uncombable hair syndrome. In: Arch Pathol Lab Med 111, 1987, pp. 754-755. PMID 3632290
  7. ^ A. Dupré et al .: Cheveux incoiffables: anomalie congénitale des cheveux. In: Bull. Soc. Ms. Dermatol. Syphiligr. 80, 1973, pp. 111-112.
  8. ^ J. Stroud, and A. Mehregan, "Spun glass hair": a clinico-pathologic study of an unusual hair defect. In: The First Human Hair Symposium 1973, pp. 103-107.

further reading

  • A. Potthoff and T. Jansen: Uncombable hair syndrome. In: Current trends in pediatric dermatology Berlin Medical Publishing House (BMV), Berlin, 2004, pp. 17–22.
  • G. Goerz and Peter Busch: The glass wool hair (“Spun Glass” Hair). In: Archives of Dermatological Research 270, 1981, pp. 353-359. doi : 10.1007 / BF00403940
  • AJ Lee et al .: A girl with loose anagen hair syndrome and uncombable, spun-glass hair. In: Pediatric Dermatology 22, 2005, pp. 230-233. PMID 15916571
  • RM Trüeb et al: Pili torti et canaliculi in ectodermal dysplasia. In: dermatologist 45, 1994, pp 372-377. PMID 8071068
  • JD Boyer et al .: Loose anagen hair syndrome mimicking the uncombable hair syndrome. In: Cutis 57, 1996, pp. 111-112. PMID 15916571
  • P. Ang and YK Tay: What Syndrome is this? Uncombable hair syndrome (Pili trianguli et canaliculi). In: Pediatric Dermatology 15, 1998, pp. 475-476. PMID 9875973
  • WB Shelley and ED Shelley: Uncombable hair syndrome: Observations on response to biotin and occurrence in siblings with ectodermal dysplasia. In: J Am Acad Dermatol 13, 1985, pp. 97-102. PMID 4031156
  • J. Ferrando and others: Pili canaliculi ("Cheveux incoiffables" or "Cheveux en fiber de verre"). In: Annales de Dermatologie et de Vénéréologie 107, 1980, pp. 243-248.
  • PH Itin et al .: Pili trianguli et canaliculi: a distinctive hair shaft defect leading to uncombable hair. In: Dermatology 187, 1993, pp. 296-298.
  • R. Laurent et al .: Syndrome des cheveux incoiffables. In: Annales de Dermatologie et de Vénéréologie 105, 1978, pp. 633-635.

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