[Cutaneous tumors during xeroderma pigmentosum in Morocco: study of 120 patients].

INTRODUCTION

Xeroderma pigmentosum is a rare recessive and autosomically transmitted genodermatosis. Its cutaneous manifestations are dominated by skin cancers. This investigation aims at studying the epidemiologic, clinic, histologic, therapeutic and evolutive aspects of the skin tumors during xeroderma pigmentosum.

PATIENTS AND METHODS

A retrospective monocentric study was carried out in the Dermatology and Veneorology Department of Ibn Rochd University Hospital of Casablanca, Morocco. It included all the xeroderma pigmentosum admitted to hospital or followed-up from 1990 to 2000. All the dossiers were included. The anatomopathologic study was carried out in all the skin tumor cases.

RESULTS

One hundred and twenty xeroderma pigmentosum were admitted in 10 Years. Fifty-four percent of the cases were females and 46 p. 100 were males. The mean apparition of the first tumor was 7.7 Years. One hundred and fifty-three skin tumors were diagnosed in 96 patients (80 p. 100). These tumors were of basocellular carcinoma type in 32.6 p. 100, of squamous cell carcinoma type in 33.9 p. 100 and of melanoma type in 11 p. 100. Ocular tumors were found in 31 cases (25.8 p. 100) and buccal in 8 cases. Therapeutically, the surgical exeresis of one or many tumors was performed in all cases. Electrocoagulation was associated in 42 p. 100 of the cases and skin graft in 52 p. 100. Cutaneous relapses after surgery were noticed in 55 cases (57.2 p. 100). Twenty-five patients died and 31 were lost to follow-up.

DISCUSSION

Our series is characterized by a large frequency of mainly cutaneous tumors (80 p. 100) in comparison with the largest review of literature (45 p. 100). These tumors were mainly represented by basocellular and squamous cell carcinoma with onset at an early age (7.7 Years). Our series is also characterized by a large frequency of cutaneous relapses after surgery (51.6 p. 100). Neglecting advice on photoprotection and the lack of regular control visits lead to the proliferation of large size tumors, making therapeutic strategies difficult or even impossible.