着色性干皮病。24例利比亚病例的临床研究。

Xeroderma pigmentosum. A clinical study of 24 Libyan cases.

作者信息

Khatri M L, Shafi M, Mashina A

机构信息

Department of Dermatology, Medical Faculty, Al-Fateh University and Central Hospital, Tripoli, Libya.

出版信息

J Am Acad Dermatol. 1992 Jan;26(1):75-8. doi: 10.1016/0190-9622(92)70010-d.

DOI:10.1016/0190-9622(92)70010-d

PMID:1732340

Abstract

BACKGROUND

Despite a high incidence of xeroderma pigmentosum, there is no previous publication from Libya.

OBJECTIVE

The purpose was to study the clinical profile of Libyan cases of xeroderma pigmentosum.

METHODS

With the help of a special protocol, 24 cases of xeroderma pigmentosum treated between 1981 and 1990 were subjected to detailed analysis.

RESULTS

The age of onset of initial manifestations ranged between 6 and 18 months whereas that of malignant lesions ranged from 2 to 10 years. Malignant lesions observed were squamous cell carcinoma in 15 patients, basal cell carcinoma in 12, and basosquamous carcinoma in 2 patients; squamous cell carcinoma of the tongue, carcinoma of the thyroid, and lymphatic leukemia affected individual cases. None of our patients developed malignant melanoma. Six patients have died; the age at death ranged between 9 and 18 years. A history of consanguinity in the parents of patients was recorded in all but two patients.

CONCLUSION

We observed early onset of severe ophthalmic lesions affecting a higher percentage of the patients.

摘要

背景

尽管着色性干皮病发病率很高，但利比亚此前尚无相关报道。

目的

旨在研究利比亚着色性干皮病患者的临床特征。

方法

借助特定方案，对1981年至1990年间接受治疗的24例着色性干皮病患者进行详细分析。

结果

初始表现的发病年龄在6至18个月之间，而恶性病变的发病年龄在2至10岁之间。观察到的恶性病变中，15例为鳞状细胞癌，12例为基底细胞癌，2例为基底鳞状细胞癌；舌鳞状细胞癌、甲状腺癌和淋巴白血病各累及个别病例。我们的患者均未发生恶性黑色素瘤。6例患者已死亡，死亡年龄在9至18岁之间。除2例患者外，所有患者的父母均有近亲结婚史。