|LETTER TO EDITOR
|Year : 1994 | Volume
| Issue : 3 | Page : 176-177
Squamous cell carcinoma arising from limbus of eye in xeroderma pigmentosum
MM Udagani, VG Govekar
M M Udagani
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Udagani M M, Govekar V G. Squamous cell carcinoma arising from limbus of eye in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol 1994;60:176-7
|How to cite this URL:|
Udagani M M, Govekar V G. Squamous cell carcinoma arising from limbus of eye in xeroderma pigmentosum. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2020 May 31];60:176-7. Available from: http://www.ijdvl.com/text.asp?1994/60/3/176/4034
To the Editor,
Ocular neoplasms arising from the eye, excluding those of eyelids constitute 11% of xeroderma pigmentosum (XP) patients. sub They are most frequently arising from the limbus and are predominantly sqamous cell carcinomas (SCC). We are reporting this case because of rarity. Recently there was a case report of malignant melanoma of skin and SCC of the eye arising from limbus in an adult XP patient. 
A 6-year-old male, youngest child of a consanguineous parents had multiple freckles and hypopigmented atrophic macules on sun exposed parts of the body since 4 years of age. Parents have 2 male and 2 female children, 3 children developed XP, 1 male child is healthy.
Child had photophobia, blepharospasm and increased lacrimation. Developmental mile stones were normal and no neurological manifestations.
Patient developed a small nodular growth 1 month back, situated at 5 O'clock position at the limbus of left eye. During 1 month, it attained the size of 1.5 cm X 1 cm grayish brown raised growth encroached upon cornea completely and growth was protruding out about 0.5 cm [Figure - 1]. Child had pain, irritation and could not close the eye. There were no matastases.
Routine investigations were normal including LFT, X-ray chest was normal, skin biopsy confirmed the diagnosis of XP. Enucleation of eyeball was inevitable. Histopathology of the growth revealed as well differentiated SCC.
Neoplasm of the eye in XP confined almost exclusively to the conjuctiva, cornea and eyelids, those portions of the eye exposed to ultraviolet radiation. These tissues sheild the iris, lens and retina from ultraviolet radiation.
Unique review of 830 published cases of XP in a span of 108 years by Kraemer et al  revealed that neoplasms occured most frequently at the limbus followed by the cornea and conjuctiva. The most frequent histologic type reported was SCC.
| References|| |
|1.||Kraemer K H, Lee M M, Scotto J. Xeroderma pigmentosum, cutaneous, ocular and neurologic abnormalities in 830 published cases. Arch Dermatol 1989; 55: 248-50. |
|2.||Negabhushan N R, Satish D A, Sumathy T K, et al. Xeroderma pigmentosum with malignant melanoma and squamous cell carcinoma. Ind J Dermatol Venereol Leprol 1989; 55: 248-50. |
[Figure - 1]