肿胀性红斑狼疮
Lupus erythematosus tumidus
ORPHA:90283疾病
定义 英文原文（暂无中文）
A rare form of chronic cutaneous lupus erythematosus characterized by extreme photosensitivity with intermittent formation of erythematous, edematous, urticarial-like, smooth plaques on sun-exposed skin areas. The lesions heal without scarring. The course of the disease is benign, and development of systemic lupus erythematosus is infrequent. Most patients do not have lupus-related autoantibodies. Skin biopsy shows a perivascular and periadnexal lymphocytic infiltrate and increased dermal mucin deposition without involvement of the dermoepidermal junction.
别名
间歇性皮肤狼疮
基本事实
- 发病年龄
- 青少年期、成年期、儿童期、老年期
临床表型 12
极常见 99–80%3
- 光感性皮肤 HP:0000992
- 真皮深层血管周围炎性浸润 HP:0031191
- 红斑 HP:0025474
偶见 29–5%5
- 环状皮肤病变 HP:0025528
- 抗 La/SS-B 抗体阳性 HP:0032235
- 抗核抗体阳性 HP:0003493
- 自身免疫性抗体阳性 HP:0030057
- 荨麻疹性斑块 HP:0030351
罕见 <4–1%1
- 抗双链DNA 抗体阳性 HP:0020151
排除 0%3
- 皮肤色素脱失/色素沉着 HP:0007483
- 皮肤脱屑 HP:0040189
- 瘢痕形成 HP:0100699
外部标识与链接
发现这一页有错误？告诉我 · 邮件主题会自动带上本页的 ORPHA 编号
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）