网状红斑黏蛋白病1例

患者男,26岁。面颈部、躯干、四肢皮疹1年。皮损组织病理示轻度角化过度,表皮水肿,真皮浅至中层毛细血管扩张充血,周围有较多单一核细胞浸润。阿新蓝染色见真皮乳头层及网状层大量黏蛋白样物质沉积。诊断:网状红斑黏蛋白病。     

网状红斑性黏蛋白病1例

患者,男,40岁。躯干网状红斑、丘疹3年伴轻微瘙痒,日晒后加重。组织病理示:真皮胶原束间有黏蛋白沉积,阿新蓝染色阳性。     

网状红斑黏蛋白病1例

患者女,28岁,双侧乳房境界清楚的网状红斑3年,月经期间发红加重.皮损组织病理示:真皮中上层血管周围淋巴细胞浸润,间质胶原纤维束间有阿辛兰染色阳性的黏蛋白沉积.诊断:网状红斑黏蛋白病.外用0.1％他克莫司软膏2次/d,治疗1.5个月后无明显改善,口服羟氯喹200 mg,2次/d,0.5个月后皮损显著改善,1.5个月后完...     

网状红斑性黏蛋白病一例

患者男,27岁,颈背部片状浸润性红斑伴轻度瘙痒2年余。2012年6月29日来我科就诊。患者2年前日晒后自颈部出现浸润性红斑,渐累及肩部,皮疹缓慢增多增大,自觉瘙痒,曾在当地医院就诊（诊断治疗不详）,皮疹无好转,且逐渐扩展至背部两侧。既往体健,无甲状腺疾病、糖尿病、自身免疫性疾病及其他疾病史,家族中无类似疾病患者……     

网状红斑黏蛋白病并发蕈样肉芽肿1例

报告网状红斑黏蛋白病并发蕈样肉芽肿1例。患者女。42岁。因躯干、上肢红斑14年,皮损增多,加重5年就诊。6年前行皮损组织病理学检查示真皮乳头层及网状层有大量黏蛋白样物质沉积,诊断为网状红斑黏蛋白病,入院皮肤科检查：头部、面部、颈部、躯干、四肢见片状轻度浸润性红斑,部分融合成网状,腹部红斑上可见紫红色浸润性斑块。皮损组织病理检查：真皮肉淋巴细胞苔藓浸润,部分细胞异形,少量浸润细胞移入表皮,形成Pautrier样微脓种,免疫组化染色结果示：LCA（＋＋＋）,CD45RO（＋＋＋）,诊断。蕈样内芽肿。     

网状红斑性黏蛋白沉积症1例并文献复习

报告1例网状红斑性黏蛋白沉积症并复习相关文献。患者女，20岁，以面、颈、胸、背、双上臂持久性光加重性网状红斑为主要临床表现，无系统损害，相关实验室检查无异常。组织病理学示：真皮中上层较大量阿辛蓝染色阳性的黏蛋白沉积，血管周围少量淋巴细胞浸润，直接免疫荧光示基膜区IgM呈线状沉积：根据临床表现、组织病理及阿辛蓝染色等诊断为网状红斑性黏蛋白沉积症。     

网状红斑粘蛋白病1例

患者女,４６岁。１２年前无明显诱因于上背部出现暗红色斑点及毛囊性丘疹,并逐渐扩大,红斑互相融合成网状或片状,有轻微痒感,日光照射后加重。后皮疹范围逐渐扩大,９年前开始波及整个背部、面颈部,并逐渐累及面部、耳后、胸腹部、双上肢。无肌痛及肌无力,无明显季节性。曾就诊于多家医院,诊断为毛发红糠疹、脂溢性皮炎、光敏性皮肤病等。经避光、口服维生素Ａ、外用皮质类固醇激素、硫磺软膏等治疗,效果不佳。体检：系统检查无异常。皮肤科情况：面部、耳后、颈、背、胸、腹、双上肢皮肤片状或网状暗红斑,有浸润感,并见粟粒、绿…     

网状红斑性粘蛋白病1例

报告１例网状红斑性粘蛋白病。患者中年男性，皮损主要发生于面，胸及背部，为网状红斑基础上出现褐色丘疹。病理检查显示真皮上部胶原纤维束间有粘蛋白沉积。该例患者尿酸水平明显增加，是否与本病有关需进一步观察。作者对网状红斑性粘蛋白病的临床病理特点，发病机制及治疗进行了文献复习。     

网状红斑性黏蛋白沉积症一例

报告1例网状红斑性黏蛋白沉积症，患者以胸背部的持久性光加重性网状红斑为主要表现，伴有点片状表面萎缩的白斑和坏死结痂，不伴系统损害，常规实验室检台无异常发现。组织病理显示．真皮有单核细胞浸润和阿辛蓝染色阳性的黏蛋白沉积.根据患者皮损的临床病理特征、组织病理及阿辛蓝染色等．诊断符合网状红斑性黏蛋白沉积症，本病临床少见。     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

Reticular erythematous mucinosis

Reticular erythematous mucinosis is a rare syndrome which can be misdiagnosed as seborrhoeic dermatitis. This long-lasting skin disease, which is characterized by the histological presence of deposits of mucin in the dermis, usually responds to treatment with per os hydroxychloroquine, 400 mg/day, for 3 months.     

Reticular erythematous mucinosis syndrome: a case report

A 71-year-old man visited us with asymptomatic erythematous patches of two years' duration on the sternum and right costal arch areas. Histologically there was an appreciable amount of lymphocytic infiltration around the infundibulum of the hair follicle and the dermal capillaries, but the deposition of a colloidal iron positive material was observed around the lymphocytic infiltration and between the collagen bundles to only a mild degree. Reticular erythematous mucinosis (REM) syndrome is controversial; what is its nature as a mucinosis? This case seems to be the extreme of REM syndrome in which mucin deposition was minimal.     

Positive photobiological investigation in reticular erythematous mucinosis syndrome

BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. CASE REPORTS: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. CONCLUSIONS: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.     

Reticular erythematous mucinosis – (REM syndrome) in twins

We present female twins with reticular erythematous mucinosis (REM syndrome). Remarkably, the lesions developed in both sisters almost at the same time in the same locations after UV exposure. Reports of familial manifestations of REM syndrome are very rare and an association to a distinct HLA constellation has not been proven. Our report clearly suggests a genetic predisposition.     

Reticular erythematous mucinosis syndrome with an infiltration of factor XIIIa+ and hyaluronan synthase 2+ dermal dendrocytes

We report a patient with reticular erythematous mucinosis (REM) syndrome. Content of hyaluronan in lesional skin was approximately 2.9-fold higher than in the patient's uninvolved skin, but its synthetic activity in fibroblasts explanted from lesional skin remained unchanged. Immunohistochemical study using antifactor XIIIa (anti-FXIIIa) antibody demonstrated that the number of FXIIIa+ cells in the lesional skin was significantly increased compared with those in the patient's uninvolved skin and in normal control skin samples (P < 0.01). As hyaluronan is considered to be synthesized by hyaluronan synthase (HAS), which is composed of three genetically distinct isoforms (HAS1, HAS2 and HAS3), the cells responsible for the accumulation of hyaluronan in lesional skin were immunohistochemically examined using antibodies for HAS1, HAS2 and HAS3. The specific antibody for HAS2 was found to react with some populations of FXIIIa+ cells in the involved skin, and the number of HAS2+ cells was significantly increased in the involved skin (P < 0.01). The results suggest that accumulation of hyaluronan in REM may be related to populations of FXIIIa+/HAS2+ dermal dendrocytes rather than to dermal fibroblasts.     

Favorable response of reticular erythematous mucinosis to ultraviolet B irradiation using a 308-nm excimer lamp

Reticular erythematous mucinosis (REM) is a rare chronic mucinosis. Histologically, the presence of mucin in the upper dermis is the most specific feature. A 73-year-old woman presented to our outpatient clinic with a 4-year history of netlike macular erythema with slight edema on her left arm. She was diagnosed as having REM on the basis of the clinical picture and histological findings. She was treated with ultraviolet B irradiation using the VTRAC Excimer Lamp system with favorable response. This is the first reported case that was treated with a 308-nm excimer lamp.     

Reticular Erythematous Mucinosis Syndrome Associated with Psoriasis-like Eruptions

We report a case of reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions. Although the two skin diseases are clinically distinct, they may be related in some ways.