盘状红斑狼疮
Discoid lupus erythematosus
ORPHA:90281疾病
定义 英文原文（暂无中文）
A rare form of chronic cutaneous lupus erythematosus characterized by erythematous, scaly papules and plaques preferentially occurring on sun-exposed skin areas (scalp, face, and ears) and exhibiting follicular plugging, pigmentary changes, and central atrophy, scarring, and telangiectasia. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and involvement of the dermoepidermal junction with thickening of the basement membrane and vacuolar degeneration of the basal cells. A small percentage of patients may develop systemic lupus erythematosus.
基本事实
- 发病年龄
- 青少年期、成年期、儿童期、老年期
- 患病率
- 1-9 / 100 000（United States）
临床表型 10
常见 79–30%8
- 萎缩性瘢痕 HP:0001075
- 红色丘疹 HP:0030350
- 红斑 HP:0025474
- 克尔里病，贯穿性角化过度病 HP:0007502
- 皮肤色素减退 HP:0001010
- 狼疮带测试阳性 HP:4000107
- 头皮瘢痕性脱发 HP:0004552
- 皮肤毛细血管扩张 HP:0100585
偶见 29–5%1
- 关节疼痛 HP:0002829
罕见 <4–1%1
- 皮肤鳞状上皮细胞癌 HP:0006739
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）