REM SYNDROME - RETICULAR ERYTHEMATOUS MUCINOSIS (ROUND-CELL ERYTHEMATOSIS), A NEW ENTITY

REM SYNDROME - RETICULAR ERYTHEMATOUS MUCINOSIS (ROUND-CELL ERYTHEMATOSIS), A NEW ENTITY
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DOI:
10.1111/j.1365-2133.1974.tb15865.x
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发表时间:
1974-01-01
影响因子:
10.3
通讯作者:
LINKER, U
LINKER, U
中科院分区:
医学1区
文献类型:
--
作者:
STEIGLEDER, GK;GARTMANN, H;LINKER, U

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在科隆观察到四名患者,三名女性和一名男性,上胸和背部出现片状或网状红斑,并有扩散到腹部的趋势。除了一例发痒和另一例晒伤后发痒外,患者没有其他症状。组织学上,除轻度水样变性和一定程度的海绵样变性外,表皮正常。其主要特征是:真皮血管扩张,血管周围有明显的圆形细胞浸润,此外,还可见阿尔辛蓝染色的物质沉积。口服大黄有一定的好处,否则包括皮质类固醇在内的治疗都不成功。其他抗疟疾药物没有使用。这与阳光照射有一定关系,但氙光的全光谱没有引起任何异常反应。由于不可能将这些发现与任何已知的综合征联系起来,因此使用了网状红斑和粘蛋白沉着症这一主要体征的组合来命名这种实体REM综合征。
Four patients, three females and one male, were observed in Cologne with sheet‐ or net‐like erythema of the upper chest and of the back, with a tendency to spread to the abdomen. The patients had no symptoms with the exception of itching in one case and itching after sunburn in another. Histo‐logically the epidermis was normal, apart from slight hydropic degeneration and some minor degree of spongiosis. The main features were: dilated blood vessels in the dermis, with a more or less pronounced perivascular infiltrate of round cells, and, in addition, deposits of a substance which stained with alcian blue. Oral treatment with Rhetis was of some benefit, otherwise therapy, including corticosteroids, was not successful. Other antimalarial drugs were not used. There was some relation to sun exposure, but the full spectrum of xenon‐light did not cause any abnormal reactions. Since it was not possible to relate these findings to any known syndrome, a combination of the main signs, reticular erythema and mucinosis, was used to name this entity REM syndrome.