Uzun Soner, Durdu Murat, Akman Ayse, Gunasti Suhan, Uslular Canan, Memisoglu Hamdi R, Alpsoy Erkan
Department of Dermatology, Cukurova University School of Medicine, Adana, Turkey.
Int J Dermatol. 2006 May;45(5):523-8. doi: 10.1111/j.1365-4632.2004.02533.x.
The purpose of this study was to describe the epidemiological and clinical features, course, response to treatment, and prognosis of pemphigus in the Mediterranean region of Turkey.
All patients with confirmed pemphigus were prospectively enrolled in two major dermatology departments in the cities of Adana and Antalya in the Mediterranean region between March 1998 and March 2004. Details including demography, findings of clinical examinations, treatment, course, and prognosis were recorded.
One hundred and forty-eight patients with pemphigus were diagnosed during the 6-year period, with a prevalence of 1.46 and an annual incidence of 0.24 per 100,000 in this region. There was a female predominance with a male to female ratio of 1:1.4. Pemphigus vulgaris (PV) was the most common clinical subtype, identified in 123 patients (83%). The mean age of onset was 43. In 101 (82%) patients with PV, disease began as persistent oral ulcers. The majority of the patients with PV could be managed with middle or high-dose steroids (60-140 mg/day). Complete clinical remission was obtained in 41 (39.4%) patients. The mortality rate was 4.8%.
A moderately high incidence of pemphigus was found in the Mediterranean region of Turkey as compared with that encountered in other countries. The commonest clinical subtype was PV with a 9.5-fold higher incidence than pemphigus foliaceus. It is more frequent in middle-aged people and has a female predominance. Although a relatively higher dose of steroid was needed to control the PV, the disease completely remitted in a significant proportion of the patients.
本研究旨在描述土耳其地中海地区天疱疮的流行病学和临床特征、病程、治疗反应及预后。
1998年3月至2004年3月期间，所有确诊为天疱疮的患者均前瞻性纳入地中海地区阿达纳和安塔利亚两市的两个主要皮肤科科室。记录了包括人口统计学、临床检查结果、治疗、病程和预后等详细信息。
在这6年期间共诊断出148例天疱疮患者，该地区患病率为1.46，年发病率为每10万人0.24。女性占优势，男女比例为1:1.4。寻常型天疱疮（PV）是最常见的临床亚型，123例患者（83%）确诊为此型。发病的平均年龄为43岁。101例（82%）PV患者疾病始于持续性口腔溃疡。大多数PV患者可用中或高剂量类固醇（60 - 140毫克/天）治疗。41例（39.4%）患者实现了完全临床缓解。死亡率为4.8%。
与其他国家相比，土耳其地中海地区天疱疮发病率中等偏高。最常见的临床亚型是PV，其发病率比落叶型天疱疮高9.5倍。在中年人中更常见，且女性占优势。虽然控制PV需要相对较高剂量的类固醇，但相当一部分患者疾病完全缓解。
