Moussaid L, Benchikhi H, Boukind E H, Sqalli S, Mouaki N, Kadiri F, Lakhdar H
Service de Dermatologie-Vénéréologie, CHU Ibn Rochd, Casablanca, Maroc.
Ann Dermatol Venereol. 2004 Jan;131(1 Pt 1):29-33. doi: 10.1016/s0151-9638(04)93538-7.
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.
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.
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.
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.
着色性干皮病是一种罕见的常染色体隐性遗传性皮肤病。其皮肤表现以皮肤癌为主。本研究旨在探讨着色性干皮病皮肤肿瘤的流行病学、临床、组织学、治疗及演变情况。
在摩洛哥卡萨布兰卡伊本·罗赫德大学医院皮肤科和性病科进行了一项回顾性单中心研究。研究对象为1990年至2000年期间入院或接受随访的所有着色性干皮病患者。纳入了所有病例档案。对所有皮肤肿瘤病例进行了组织病理学研究。
10年间共收治120例着色性干皮病患者。其中54%为女性，46%为男性。首个肿瘤出现的平均年龄为7.7岁。96例患者（80%）诊断出153个皮肤肿瘤。这些肿瘤中，基底细胞癌占32.6%，鳞状细胞癌占33.9%，黑色素瘤占11%。31例患者（25.8%）发现眼部肿瘤，8例患者发现颊部肿瘤。治疗方面，所有病例均对一个或多个肿瘤进行了手术切除。42%的病例联合了电凝治疗，52%的病例进行了皮肤移植。55例患者（57.2%）术后出现皮肤复发。25例患者死亡，31例失访。
与最大规模的文献综述相比，我们的系列研究中主要为皮肤肿瘤的发生率较高（80%）（文献综述中为45%）。这些肿瘤主要为基底细胞癌和鳞状细胞癌，发病年龄较早（7.7岁）。我们的系列研究还具有术后皮肤复发率高的特点（51.6%）。忽视光防护建议以及缺乏定期复诊导致大尺寸肿瘤增多，使治疗策略变得困难甚至无法实施。
