Abstract :
Goltz syndrome is X linked dominant disorder of mesodermal and ectodermal tissue, occurring mostly
in females. It is usually lethal in males, although alive males have been reported probably due to
sporadic mutations or mosaicism. We hereby report a case of an 18 day old baby girl who presented with
multiple atrophic hypopigmented lesions in blaschkoid pattern along with limb defects. Histopathological
examination revealed atrophy of the dermis and was consistent with the diagnosis of Goltz syndrome. The
case is being reported for its rarity.
Keyword :
Goltz syndrome, Focal dermal hypoplasia.