|Year : 1984 | Volume
| Issue : 2 | Page : 99-100
Keratoma malignum, a skin disorder with ocular lesions
Department of Ophthalmology, Silchar Medical College, Silchar, India
Department of Ophthalmology, Silchar Medical College, Silchar
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Bhattacharjee H. Keratoma malignum, a skin disorder with ocular lesions. Indian J Ophthalmol 1984;32:99-100
Keratoma malignum is a variety of congenital Ichthyosis. Such a rare case with different congenital ocular lesions is documented.
| Case report|| |
A baby boy of two months old was examined for the complaint of progressively increasing fleshy mass one each in both the upper eye lids since birth. On detail interview it was learnt that the boy was out come of an uneventful gestation of 37 weeks duration. There was no relevant family history.
According to parents, at birth the baby was encased in a thick whitish armour which after a few hours became hard and the mother subsequently removed the same after repeated bath.
On examination the baby was found to have grotesquely deformed face and claw like hands. The skin of the whole body is hyperkeratotic, wrinkled, hard, dry, and non elastic with deep red fissures which tend to follow Langer's line of cleavage. Crust like horny plates of skin are found on the back and sides of the neck, around the umbilicus and the back, extensor surface of the limbs particularly around the adjoining area of knee and elbow joints and on the face. The whole body looks as if is covered with cellophane paper [Figure - 1].
Ocular findings are (a) evertion of upper and lower lids in both eyes with conjunctival hyperplasia in the upper lids and more marked in the left side, (b) telecanthus and hypertelorism [Figure 2), (c) superficial punctate corneal infiltrations. The crystalline lens is found transparent with normal fundus.
| Discussion|| |
Ichthyosis is a rare disorder of keratinization and keratoma malignum is a variety of congenital ichthyosis which in severity of' manifestation lies between ichthyosis foetalis and ichthyosiform erythroderma and its incidence (including both the type) is 1:300,000 and is inherited by an autosomal recessive gene with variable expressivity. Various ocular involvements like ectropion of lids, keratitis, and cataracts are reported in the literature along with different types of congenital ichthyosis,,,
The described case had associated congenital ectropion of all the four lids with conjunctival hyperplasia, telecanthus and hypertelorism. Last two associated congenital abnormalities are observed for the first time along with congenital ichthyosis.
| Summary|| |
A case of keratoma malignum with congenital ectropion of all the eye lids with conjunctival hyperplasia, keratitis, telecanthus and hypertelorism is described.
| References|| |
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[Figure - 1], [Figure - 2]