Incontinentia Pigmenti

Incontinentia Pigmenti

“…If one includes the m ottling of the skin, the strabism us and the alopecia of the scalp as part of the incontinentia pigm enti syndrome, the genetic pattern of our large family suggests th at this is an x-linked disorder with great penetration in heterozygotic females and in trauterine lethality inhem izygoticm ales.Other three-generation family histories have been recorded with similar findings [10,15,17]. It is interesting th a t in our family history, the grandm other had children by two different marriages.…”

“…Probably most patients with incontinentia pigm enti have only the cutaneous m anifestations. Mental retardation, epilepsy and spastic tetraplegia m ay be noted [4,6,8,11,14,15], as in our three institution alized patients.…”

Incontinentia Pigmenti et Achromians

“…Sex-linked inheritance was also supported by M arty el at. [12] in 1958 while reporting three generations of a family in which all females displayed cutaneous lesions.…”