Observations on hair shaft morphology in mucopolysaccharidoses.

Scalp hair samples were obtained from 11 children with mucopolysaccharidosis (MPS, types I, II and III) in order to study the micromorphological structure of the hair shafts. To evaluate the morphological variation longitudinal hair preparations and cross-sections were investigated by light microscopy and additionally scanning electron microscopy was employed to demonstrate the surface structure. It is shown that the mean hair shaft diameter in MPS-patients, separated into two age groups, is not significantly different from the mean value in normal children of the same age, whereas the wider range of diameters and, in connection with it, the increased medulla content seems to be a characteristic feature of MPS. The most striking deviations were observed in the cross-sections, particularly in MPS III/A patients: varying polygonal shapes and an abnormal pigment distribution. According to these cross-sections the hair surface exhibited severe deformities in the scanning electron microscope, i.e. distortions of hair, irregular nodes and longitudinal grooves. In MPS I and II similar abnormalities were found.