Uncombable hair syndrome

The syndrome of unkämmbaren hair (Latin Pili trianguli et canaliculi ), also referred to as a symptom of not frisierbaren hair, Struwwelpeter syndrome or glass wool hair, is a rare structural anomaly of the hair. As the name of the syndrome it describes already, it means that the head hairs are not manageable.

Description and Diagnosis

The syndrome of unkämmbaren hair is characterized by dry frizzy head hair that is not easy to comb. The hair is always disheveled ( " Tangled " ), but not brittle, usually silver - blond and slow growing. The syndrome can manifest itself in the period of the first few weeks of life until puberty. In most cases, it occurs already in early childhood.

The Nichtkämmbarkeit is caused by a random arrangement of the tufts of hair, which in turn is due to the structural hair structure. Under a light microscope can be typical of this syndrome pili trianguli et canaliculi ( different deformed hair shafts, in part, by two or three deep elongate grooves deformed ) best seen in cross section. For this, the paraffin-embedded hairs are cut transversely. The clinical diagnosis can be confirmed safe with a scanning electron micrograph. During "normal" hair has a round cross section, the head hair when the syndrome unkämmbaren hair on the entire length is triangular and kidney-shaped deformed. At least 50 % of scalp hair have this deformation, and only the head hair are affected.

Usually occurs the glass wool hair on as a separate syndrome. But it can also with other physical anomalies, such as ectodermal dysplasia, retinitis pigmentosa, juvenile cataracts, polydactyly, abnormalities in tooth enamel, be connected.

In contrast to this, the syndrome syndromes unkämmbaren the hair is not associated with a different physical, or with neurological or mental abnormalities.

Referred to in the Anglo-Saxon literature the syndrome of unkämmbaren hair is mostly as spun glass hair ( " glass wool hair " ) or uncombable hair syndrome ( " not - to style hair syndrome " ) or the French term cheveux incoiffables ( " not to style hair " ).

Etiology

The cause of the glass wool hair, both hereditary causes, as well as sporadic forms are described. The hereditary forms are essentially autosomal dominant, but autosomal recessive modes of inheritance - each with different penetrance - known.

First description

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