Arch. Derm. Res. 261, 217--218 (1978) Archives of

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�9 Springer-Verlag 1978

Archives for Dermatological Research Letters A N e w Type of Pilar Dysplasia

The Uncompable Hair Syndrome with Pili Trianguli et Canaliculi A. Dupre and J. L. Bonafe

Department of Dermatology, Hopital de La Grave, Place Lange, F-31052 Toulouse, Cedex, France

The scanning electron microscope has become an indispensable basic technique in the investigation of acquired or congenital diseases of the hair. This method allowed us the identification in 1973 of a new type of pilaf dysplasia: the "uncombable hair syndrome" [1]. The specificity of the syndrome was later confirmed by two studies [2, 3]. Clinically it is characterised by short, sparse, dry, dishevelled, frizzy and uncom-

Fig. 1. Triangular section with rounded angles

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218 A. Dupre and J. L. Bonafe

Fig. 2. Longitudinal groove along the entire length of the hair

bable hair. The condition is hereditary and seems to be a dominant character. In our observation there were two family cases, and in Grupper 's three family cases. In Ferrando's there were two. This new type of pilar dysplasia is not associated with general health problems, nor with neurological disorders, nor wi th changes in amino acid on copper metabolism.

The scanning electron microscopy (using silver shadowing-200 A - a n d a scanning angle of 45 ~ showed that the hair had two specific alterations (a) a triangular section with rounded angles; (b) a longitudinal groove along the entire length of the sides. These changes were identical for each hair without exception. This dysgenesy is not associated with other conditions, such as pili torti, monilethrix, etc... These changes ar therefore specific of the disease. It seems probable that these two a l te ra t ions- triangular section and lateral groove - when combined remove the suppleness of the hair and cause it to become uncornbable. We suggest the term "pili trianguli et canaliculi" to designate this pilar abnormality since the main feature are a canal-like longitudinal depression and a triangular section.

References

l. Dupre, A., Rochiccioli, P., Bonafe, J. L.: "Cheveux incoiffables": anomalie cong6nitale des che- veux. Bull. Soc. Fran~. Derm. Syph. 80, 111-112 (1973)

2. Grupper, Ch., Attal, C., Gougne, B.: Syndrome des cheveux incoiffables. Bull. Soc. Fran~. Derm. Syph. 81, 299-300 (1973)

3. Ferrando, J., Gratacos, M. R., Fontarnau, R., Castells Rodellas, A.: Sindrome de los cabellos "impeinables". Med. Cut. I. L. A. 1, 39-46 (1977)

Received January 5, 1978