CUTANEOUS FIBROUS DYSPLASIA - AN INCOMPLETE FORM OF THE MCCUNE-ALBRIGHT SYNDROME

A number of genetic disorders have been described in limited form, We report a patient with precocious puberty, large irregularly shaped caf e-au-lait spots since birth and a diffuse scalp alopecia showing the c utaneous changes of fibrous dysplasia. Histologically, the hair follic les were replaced by convolutions of fibrous tissue. This is to our kn owledge the first patient reported with an apparently localized cutane ous form of this syndrome, a pattern predicted by the recently describ ed somatic mutation of the G(s) alpha gene. This is the second patient reported to our knowledge with the cutaneous McCune-Albright syndrome and scalp alopecia, and the first with diffuse scalp alopecia, the la tter being the presenting sign. We believe that the differential diagn osis of both localized and diffuse alopecia should include the McCune- Albright syndrome.