Acquired ichthyosis is very likely to be associated with a malignancy, especially with Hodgkins disease. The association of congenital ichthyosis with malignant tumors has been so far reported only in two instances. Herein we report on a third case. Due to the small number of cases, no conclusion ca
Congenital ichthyosis in a Libyan child with ophthalmic manifestations
โ Scribed by Mahmoud Elshtewi; Desmond B. Archer
- Publisher
- Springer Netherlands
- Year
- 1991
- Tongue
- English
- Weight
- 480 KB
- Volume
- 15
- Category
- Article
- ISSN
- 0165-5701
No coin nor oath required. For personal study only.
โฆ Synopsis
This report presents a Libyan child with congenital ichthyosis of the recessive lamellar variety complicated by severe ectropion of both upper and lower eyelids. Over a period of 12 months the degree of ectropion diminished and the anterior segments of the eyes were preserved using regular lubricating drops, vitamin A ointment and intermittent topical antibiotic therapy. Despite the pronounced initial upper eyelid ectropion no surgery was required to relieve the ectropion or prevent corneal exposure.
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A female patient with the karyotype 45,X/46, X, r(X)(p11.2 q13) and severe developmental delay, prominent fingertip pads, long palpebral fissures, short stature, and history of hypotonia had a phenotype reminiscent of Kabuki syndrome. We hypothesized that overexpression of X chromosome-derived seque