Abstract
Purpose: :
To describe the ocular manifestations found in two cases of Xeroderma Pigmentosum and the utility of de impression cytology in these cases.
Methods: :
Case I. A 19 year–old male presented to us with red eye, scarring, fotofobia and foreign body sensation. He had previous history of basal cell carcinoma in the face. On ophthalmic examination his visual acuity was 20/50(.)20 /30 both eyes, with an intraocular pressure of 14 mm Hg. The right eye with conjuntival redness, perilimbic pigmentation and punctate queratitis. Left eye with same characteristics, but with deposit of pigment in cornea at estromal level without vascularization. both caruncules with pigment. Ultrabiomicroscopy reported neoformation in the nasal sector compatible with pterigión, without infiltration. The impression cytology reports in left eye proteinaceous and fibrilar material with inflamatory cells, pleomorfism and hypercromasia with atypia. Case II. A 17 year–old patient who referred fotofobia, tearing and hyperemia of both eyes. He had history of basal cell carcinoma of conjuntiva of the left eye. In the opthalmologic examination we found a visual acuity of 20/20 in both eyes, with a normal intraocular pressure. Right eye with hyperemia and nasal pseudopterygium that invade 2 mms of the cornea. Left eye without changes. Normal ultrabiomicroscopy. The impression cytology reports in both eyes epithelial cells with discreet variability in form and nuclear size, with granular cromatine and some with small nuclei.
Results: :
In both patients the impression citology reported changes compatible with cronic and acute inflamatory reactions. The changes observed in the ophthalmologic exploration do not justify the surgical boarding, but the narrow observation mainly at caruncular level. Both patients presented changes compatible with actinic prurigo.
Conclusions: :
The XP is a rare disease (1 case by 500.000 new born) that is inherited in a recesive autosómic form. In these patients the two types of conjuntival cancer found with frecuency are the basal cell carcinoma and the squamous cell carcinoma. The impression cytology is one of the methods of early diagnosis in these cases, and displasia can be detected with 80 % of certainly. In spite of the opportune detection, 2/3 of the cases die before arriving at the third decade of the life.
Keywords: radiation damage: light/UV • conjunctiva • cytology