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系统性肉样瘤病患者的特定皮肤损伤:与疾病严重度和慢性度的关系。

Specific cutaneous lesions in patients with systemic sarcoidosis: relationship to severity and chronicity of disease.

机构信息

Department of Dermatology, Bellvitge University Hospital, IDIBELL, University of Barcelona, Barcelona, Spain.

出版信息

Clin Exp Dermatol. 2011 Oct;36(7):739-44. doi: 10.1111/j.1365-2230.2011.04128.x. Epub 2011 Jul 25.

DOI:10.1111/j.1365-2230.2011.04128.x
PMID:21790725
Abstract

BACKGROUND

Specific (granulomatous) cutaneous lesions are seen in 9-37% of cases of systemic sarcoidosis, and are usually classified into maculopapules, plaques, lupus pernio (LP), scar sarcoidosis, and subcutaneous sarcoidosis. Their prognostic significance has not been fully established.

AIM

To analyse the relationship between the clinical type of granulomatous cutaneous lesions and the systemic features and prognosis of systemic sarcoidosis.

METHODS

The clinical charts of 86 patients (19 men, 67 women, mean age 46.82 years) with systemic sarcoidosis and granulomatous cutaneous involvement followed up for > 2 years at Bellvitge University Hospital were reviewed.

RESULTS

Cutaneous lesions developed before or at the time of diagnosis of systemic sarcoidosis in 80.23% of patients. The main cutaneous lesions were classified as maculopapules (28 patients), plaques (31), LP (6), scar sarcoidosis (7) and subcutaneous sarcoidosis (14). Erythema nodosum (EN) was seen in 30 patients. Radiological stage was 0 for 8 patients, I for 48, II for 24, III for 5 and IV for 1. Systemic sarcoidosis activity persisted for > 2 years in 47 patients, and 42 received systemic corticosteroid treatment for their disease. Maculopapular and subcutaneous sarcoidosis were mainly seen in patients with EN and radiological stage I. Plaques and LP were associated with chronic disease and requirement for systemic corticosteroids.

CONCLUSIONS

Cutaneous granulomatous lesions are usually present at the diagnosis of systemic sarcoidosis, and the type of cutaneous involvement may have prognostic significance.

摘要

背景

在系统性肉样瘤病的病例中,有 9-37%可见特异性(肉芽肿性)皮肤损害,通常分为斑丘疹、斑块、冻疮样狼疮(LP)、瘢痕肉样瘤和皮下肉样瘤。但其预后意义尚未完全确定。

目的

分析肉芽肿性皮肤损害的临床类型与系统性肉样瘤病的系统特征和预后之间的关系。

方法

回顾了在 Bellvitge 大学医院接受随访>2 年的 86 例(男 19 例,女 67 例,平均年龄 46.82 岁)系统性肉样瘤病伴肉芽肿性皮肤受累患者的临床病历。

结果

80.23%的患者在诊断系统性肉样瘤病之前或同时出现皮肤损害。主要的皮肤损害分为斑丘疹(28 例)、斑块(31 例)、LP(6 例)、瘢痕肉样瘤(7 例)和皮下肉样瘤(14 例)。30 例患者出现结节性红斑(EN)。8 例患者的放射学分期为 0 期,48 例为 I 期,24 例为 II 期,5 例为 III 期,1 例为 IV 期。47 例患者的系统性肉样瘤病活动持续>2 年,42 例患者因疾病接受了全身皮质类固醇治疗。EN 和 I 期放射学表现患者主要出现斑丘疹和皮下肉样瘤,斑块和 LP 与慢性疾病和全身皮质类固醇治疗相关。

结论

皮肤肉芽肿性损害通常在系统性肉样瘤病的诊断时出现,皮肤受累的类型可能具有预后意义。

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