ABSTRACT

Observation:

Olmsted syndrome (OS) is a very rare genetic disorder, which includes diffuse alopecia, leukokeratose of oral mucosa, onychodystrophy , hyperkeratotic linear streaks, follicular keratosis and anhidrosis. Pseudomonilethrix shaft anomaly is characterized by hair shafts with elliptical nodes at variable intervals and separated by narrower internodes. To the best of our knowledge, we present here the first documented case of OS associated with pseudomonilethrix hair shaft abnormality.