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CASE REPORT
Year : 1984  |  Volume : 32  |  Issue : 2  |  Page : 99-100

Keratoma malignum, a skin disorder with ocular lesions


Department of Ophthalmology, Silchar Medical College, Silchar, India

Correspondence Address:
H Bhattacharjee
Department of Ophthalmology, Silchar Medical College, Silchar
India
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Source of Support: None, Conflict of Interest: None


PMID: 6526475

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How to cite this article:
Bhattacharjee H. Keratoma malignum, a skin disorder with ocular lesions. Indian J Ophthalmol 1984;32:99-100

How to cite this URL:
Bhattacharjee H. Keratoma malignum, a skin disorder with ocular lesions. Indian J Ophthalmol [serial online] 1984 [cited 2021 Jan 26];32:99-100. Available from: https://www.ijo.in/text.asp?1984/32/2/99/27381

Keratoma malignum is a variety of con­genital Ichthyosis. Such a rare case with dif­ferent congenital ocular lesions is documented.


  Case report Top


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 Top


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.[4] Various ocular involvements like ectropion of lids, keratitis, and cataracts are reported in the literature along with different types of congenital ichthyosis[1],[2],[3],[5]

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 Top


A case of keratoma malignum with con­genital ectropion of all the eye lids with con­junctival hyperplasia, keratitis, telecanthus and hypertelorism is described.

 
  References Top

1.
Cordes, F.C. and Hogen M.J., 1939, Arch. Ophthalmol. 22: 590.  Back to cited text no. 1
    
2.
Vails D, 1940, Arch. Ophthalmol. 24: 275  Back to cited text no. 2
    
3.
Joy B., Blach R.K. and Well R.S., 1968. Brit. J. Ophthalmol. 52: 217.  Back to cited text no. 3
    
4.
Rook A., Wilkinson D.S. and Ebling FJ.G., 1972. A Text Book for Dermatology Vol II. 2nd ed. 1157. Black­well Scientific Publications. Oxford.  Back to cited text no. 4
    
5.
Cram D.L., Resneck J.S. and Jackson W.B., 1979, Arch Dermatol. 115: 467.  Back to cited text no. 5
    


    Figures

  [Figure - 1], [Figure - 2]



 

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