Showing papers on "Incontinentia pigmenti published in 1980"

Incontinentia pigmenti. A four-generation study.

Abstract: • Seven individual members with incontinentia pigmenti are described from a family spanning four generations. The natural history of the cutaneous, ocular, hair, and dental anomalies is described for each involved member. In addition, a previously undescribed ectodermal defect, woolly-hair nevus, appeared in several affected members of this family. Evidence is offered to support the theory that the pattern of inheritance of incontinentia pigmenti is by linkage of the gene on the X chromosome acting as a dominant gene in females and lethal in males. ( Arch Dermatol 116:701-703, 1980)

[Congenital homolateral epidermal hyperplasia and hypoplastic hemidysplasia (splitting of the Solomon's syndrome) (author's transl)].

Abstract: The observation of a 16-year-old girl born with an ectromelia and an ipsilateral inflammatory verrucous epidermal nevus led us to a synthetic study of 17 similar cases already published since 1927: all these cases concern female patients and are characterized by a unilateral hypoplastic dysplasia, most often of limbs, and inflammatory epidermal hyperplastic lesions described as ichthyosiform, psoriasiform or verrucous, usually distributed on the same side on the skin overlying the dysplastic body areas. The skin lesions may be partly regressive after birth and their histological features are suggestive of inflammatory linear verrucous epidermal nevus (I. L. V. E. N.). These associations may be representative of a special form of Solomon's syndrome whose heterogeneity has be recently emphasized. We propose to subdivide it in 3 forms: the epidermal nevus syndrome (Solomon's syndrome)--the organoid nevus syndrome (Schimmelpenning's syndrome)--the I. L. V. E. N. syndrome, probably X-linked dominant inherited (lethal for hemizygous males), associated with ipsilateral hypoplastic body lesions and, however less frequently than in the epidermal nevus syndrome, with ocular and nervous abnormalities. The distribution of cutaneous lesions has some similarities with the pattern of skin symptoms of X-linked dominant traits such as chondrodysplasia punctata, focal dermal hypoplasia or incontinentia pigmenti. The most typical feature of this syndrome is the strong inflammatory aspect of the epidermal nevus erroneously described in previous cases as unilateral psoriasis or ichthyosiform erythroderma.

[Incontinentia pigmenti (Block-Sulzberger syndrome) associated with congenital retinal fold--a long-term 18-year observation].

Abstract: Report on a case of incontinentia pigmenti presenting with the main characteristics features: patchy hyperpigmentation of the skin, alopecia and dental anomalies. In addition to the rare hyperpigmentation of the lower eyelids, hemiatrophy of the right face and horizontal nystagmus were found. Eye involvement consisted of a congenital retinal fold with retinal vessels draen into and bordered by pigment clumps.--When the patient with 14 total retinal detachment associated with a totally liquefied vitreous occurred and surgical reattachment failed. As a rarity in this case, the full picture of retrolental fibroplasia was not present; it was possible to follow developments over a period of 18 years.