XERODERMA PIGMENTOSUM, A PREDISPOSING FACTOR FOR CONJUNCTIVAL CANCER

Résumé

Squamous neoplasia of the ocular surface represents a spectrum of lesions ranging from dysplasia to invasive carcinoma. Squamous cell carcinoma of the conjunctiva is rare in children. The main risk factors for the disease are male sex, exposure to ultraviolet light, human papillomavirus, human immunodeficiency virus and xeroderma pigmentosum. We report the case of a sibling with xeroderma pigmentosum associated with bilateral squamous cell carcinoma of the conjunctiva.

Two brothers, aged 8 and 6, presented with xeroderma pigmentosum. The eldest presented with a bilateral ocular tumor involving the entire ocular surface as well as the cornea without palpebral involvement. Mutilating surgery was performed on both sides, and histology revealed squamous cell carcinoma. The younger brother was screened for a conjunctival lesion, and histology after excisional biopsy revealed papillomatous squamous dysplasia. A recurrence occurred one year later with malignant transformation into squamous cell carcinoma. Topical treatment with 5-fluorouracil was prescribed.

OSSNs should be sought in young children with xeroderma pigmentosum. Recurrences are most often local, but these lesions can evolve into invasive forms.