成人色素性荨麻疹一例

临床资料患者,女,34岁。主因躯干、四肢褐色斑8年,于2012年9月17日就诊。8年前患者无明显诱因躯干、四肢出现红色粟粒至黄豆大小红色斑疹、斑丘疹,伴轻度瘙痒,消退后遗留褐色斑,持续存在,不能消退。遇冷后出现红色风团。曾就诊于当地医院,具体诊断及治疗     

大疱性色素性荨麻疹1例

目的　介绍 1例大疱性色素性荨麻疹的临床资料并探讨该病的最新研究进展。方法　取臀部新发皮损做皮肤组织病理和Giemsa染色检查真皮内肥大细胞。结果　表皮大致正常 ,表皮下水疱形成 ,真皮内有大量大小一致的、圆形、胞浆淡染的、单一核细胞 ,Giemsa染色示胞浆内有紫红色颗粒 ,证明是肥大细胞。结论　大疱性色素性荨麻疹是肥大细胞增生症的一个特殊类型。     

大疱性色素性荨麻疹1例

患儿女,4个半月。全身色素斑4个月,起水疱10天。查体:全身弥漫性灰褐色色素斑,表面见较多水疱、大疱及糜烂结痂,尼氏征阴性,Darier征阳性。皮损组织病理检查显示真皮浅层及血管周围见较多单一核细胞浸润,G iem sa染色阳性。诊断为大疱性色素性荨麻疹。     

大疱性色素性荨麻疹1例

患儿女,1岁,躯干、四肢反复起斑丘疹、水疱1年伴痒。全身可见褐色斑丘疹,部分斑丘疹上见水疱,摩擦斑丘疹后局部出现风团样损害,即Darier征(+)。皮损组织病理示:表皮下大疱形成,真皮浅层可见大量肥大细胞浸润,Gimsa染色阳性,直接免疫荧光阴性。     

成人色素性荨麻疹一例

患者,女,23岁。全身浅褐色斑片4年,皮肤划痕征(+),Darier征(-)。皮损组织病理示:真皮乳头层和真皮浅层血管周围可见灶状分布的圆形和梭形肥大细胞。甲苯胺蓝染色(+)。诊断:色素性荨麻疹。     

成人色素性荨麻疹1例

色素性荨麻疹（Urticaria pigmentosa,UP）属于皮肤肥大细胞增生症的一种,病因不明,其发病率为1：8000～1：1000之间,男女发病率相当。大多数患者为儿童,成人发病不常见。本文报道1例典型的成人色素性荨麻疹。     

色素性荨麻疹

患儿男,3岁。 主诉:全身出现色素性斑疹、斑丘疹,伴轻度瘙痒2年余。 现病史:患儿于1岁时躯干部开始出现棕褐色斑疹、斑丘疹,黄豆至蚕豆大,伴轻度瘙痒,搔抓或摩擦皮肤后局部可出现皮肤发红或风团,偶有水疱、血疱,皮疹消退后遗留色素沉着斑。患儿发病以来无恶心、呕吐、腹痛、腹泻、关节肿痛等不适。对皮疹未予以特殊诊治,皮疹呈进行性增多,并逐渐扩展至四肢、颜面部。 个人史:患儿系足月顺产,母乳喂养,平素体健,无特殊不良嗜好。     

色素性荨麻疹1例

<正>1临床资料患儿男,3岁。全身反复起红斑、风团伴瘙痒两年半。其父代诉患儿自8个月大时胸背部起红疹,约3～4 d后自行消退,但原皮损处留有皮疹大小褐色斑。继之患儿头部、颈部、四肢等部位先后出现相似症状。皮损处抓挠或摩擦后出现明显抓痕、风团或红斑,可自行消退。发病时有烦躁不安、哭闹、搔抓等。多次就诊,间断口服抗过敏药物,静脉滴注维生素C及葡萄糖酸钙等治疗,无明显效果。自发病以来,无腹痛和腹     

色素性荨麻疹38例临床及组织病理分析

回顾性分析38例色素性荨麻疹患者的临床表现及组织病理.患者皮损多表现为躯干、四肢圆形或类圆形的色素性斑丘疹.真皮内可见肥大细胞不同程度浸润,Giemsa染色阳性.色素性荨麻疹的临床和组织病理具特征性,Giemsa染色可帮助确诊.     

色素性荨麻疹1例

患者女，18岁，全身起疹17例，对其组织切片进行吉姆莎 染色，可见肥大细胞，结合临床符合色素性荨麻疹诊断。     

A new variant of mastocytosis: report of three cases clinicopathologically mimicking histiocytic and vasculitic disorders

Cutaneous mastocytosis (CM) or urticaria pigmentosa is characterized by abnormal proliferation and accumulation of mast cells. Clinically, CM usually presents as symmetrically distributed red-brown macules or papules that develop weals, erythema and often pruritus on stroking (Darier's sign). The histological hallmark of the disease is an increase in oval to spindle-shaped mast cells in the dermis located around blood vessels and skin appendages. We describe three patients with a new clinicopathological type of CM, which clinically mimics a histiocytic disorder and histologically mimics leucocytoclastic vasculitis (LV). Three infants (two boys and one girl) developed generalized reddish-yellow-brown macules of 3-10 cm with occasional scaling and crusting on the trunk and extremities without further symptoms or organ involvement except variable itching. Histology revealed diffuse and dense dermal infiltrates of eosinophils, neutrophils and nuclear debris with perivascular accentuation, imitating LV. This infiltrate masked large epithelioid cells, positive for macrophage markers, which by special histochemical stains for metachromatic granules turned out to be mast cells. This is the first report of this new variant of CM, which may cause considerable diagnostic difficulties both clinically and histopathologically.     

带状分布的色素性荨麻疹

报告1例带状分布的色素性荨麻疹.患者女,17岁.双侧腰腹部各见一条呈带状分布的色素沉着斑,用手摩擦后局部出现红色风团.皮损组织病理检查:表皮基底层黑素增加,甲苯胺蓝染色示真皮内毛囊及汗腺周围小片状肥大细胞浸润,胞质内含有嗜异染颗粒.     

皮肤白细胞碎裂性与淋巴细胞性血管炎某些粘附分子表达的比较研究

目的：比较细胞粘附分子ＩＣＡＭ－１、ＶＣＡＭ－１和ＥＬＡＭ－１在皮肤白细胞碎裂性和淋巴细胞性血管炎的表达及其意义。方法：采用免疫组化方法研究两型血管料的粘附分子表达情况。结果：角质形成细胞ＩＣＡＭ－１在淋巴细胞性血管炎（８５．７１％）表达明显高于白细胞碎裂性血管炎（１１．１１％（Ｐ〈０．０００１）,ＩＣＡＭ－１、ＶＣＡＭ－１、ＥＬＡＭ－１在两型血管炎血管内皮中表达无区别（Ｐ值分别为１,０．０６８,０．４６）。结论：无论在白细胞碎裂性还是淋巴细胞性血管炎、粘附分子表达均上调。两种血管炎粘附分子表达率的不同反应了局部细胞因子释放的不同,这对揭示血管炎发病机理将具有重要意义。     

The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers

Background Urticaria pigmentosa is a cutaneous disorder involving infiltration of the skin with mast cells. Histologically the papillary dermis has an increased number of mast cells with an increase in basal layer pigmentation. In addition to possible systemic symptoms, patients with urticaria pigmentosa can suffer emotionally from the cosmetic nature of this skin disease. Objective The purpose was to investigate the use of a diode‐pumped Nd:YAG laser at 532 nm for the treatment of the cosmetic comorbidity of urticaria pigmentosa lesions. Methods A 19‐year‐old white male with urticaria pigmentosa had multiple lesions on the dorsum of the hands and forearms. A test site on the right inner arm was treated with a DioLite? 532 nanometer laser. Because of satisfaction with the treatment of the test site lesions, multiple lesions on the dorsal hands and forearms were also treated with the DioLite? 532 nanometer laser. Results There was a dramatic clinical reduction in the amount of lesions on the dorsum of the hands and forearms. The test site lesions on the right inner arm had not recurred. Conclusion The diode‐pumped Nd:YAG laser at 532 nanometers should be considered part of a dermatologist's armamentarium for the treatment of a patient's cosmetic concerns with lesions of cutaneous mastocytosis.     

A clinical and histological study of urticaria pigmentosa: relationships between mast cell proliferation and the clinical and histological manifestations

In 21 cases of urticaria pigmentosa (UP), clinical and histological observations and evaluation of mast cell (MC) volume density in the lesions using a morphometric point counting method were performed. The mutual correlations between clinical and histological findings were statistically assessed by a method of multiple regression analysis. Clinical items employed in the analyses were as follows: sex, the age of onset, the age of biopsy, the biopsy, the duration of lesions, the type of skin lesions, sites involved, the presence or absence of Darier's sign of Darier's sign and symptoms, and serum histamine level. Histological items included the localization and infiltration pattern of MC, the level of basal melanosis, the presence or absence of inflammatory cell infiltration, and the MC volume density in the lesions. Statistical significance of the partial regression coefficients was obtained for 6 pairs of the criteria (p = 0.05), including the age of onset and the age of biopsy, the age of onset and the level of basal melanosis, the duration of lesions and the level of basal melanosis, and the type of skin lesions and the level of basal melanosis. No significant correlations were observed between the MC volume density in the lesions and any of the other items. These results suggest that the basal melanosis in a UP lesion may not be a direct reaction to the transitory massive infiltration of MC, but rather be due to a relatively long-term effect of MC infiltration. Furthermore, the MC volume density in the lesion is not likely to be an important factor in determining the clinical manifestations of a UP lesion.     

Cutaneous mastocytosis with atypical mast cells and giant cytoplasmic granules

A 12-year-old male presented with an 8-year history of five firm cream colored papules on the right vertex of the scalp. A biopsy showed a dense infiltrate of monomorphous mast cells involving the dermis and extending into the subcutis. A relatively well-circumscribed cluster of larger cells showed pleomorphic nuclei with bilobed and multilobed morphology. Both mast cell populations had an eosinophilic cytoplasm filled with granules ranging in size from small to giant forms. By immunohistochemistry, the cells expressed CD117, tryptase and CD68, and were negative for AE1/AE3, CD1a, CD2 and CD25. S-100 staining revealed only faint cytoplasmic positivity and myeloperoxidase had an inhomogeneous patchy pattern, with an overall staining of less than 5% of the cells. A diagnosis of cutaneous mastocytosis was made and after 6 months follow-up, no progression observed. Clinical correlation and awareness of these unusual morphologic features as being part of the spectrum of cutaneous mastocytosis are important to avoid an erroneous diagnosis of malignancy. Although pleomorphic, multilobed nuclear morphology and giant cytoplasmic granules have not been associated with an aggressive behavior or systemic mastocytosis, close clinical observation is warranted in this context.     

Lack of c-kit mutation in familial urticaria pigmentosa

Somatic mutations within c-kit have been reported in individuals with mastocytoses, including urticaria pigmentosa (UP). We have identified three siblings with UP. We aimed to determine whether the c-kit proto-oncogene was playing a part in the aetiology of UP in these three siblings. Using seven microsatellite repeat markers spanning an 8-cM interval encompassing the c-kit gene we followed the transmission of the c-kit gene in this family. Furthermore, single-strand conformation polymorphism analysis was used to scan exon 17 of the c-kit gene for mutations in genomic DNA of all family members and somatic DNA extracted from skin of the eldest affected sibling, the proband. No mutations were found in exon 17 in either genomic DNA of all family members or somatic DNA of the proband. Patients with UP have been shown to possess somatic mutations of the c-kit gene. However, this locus has been excluded as playing a part in the three siblings examined here in whom a second gene locus must be determining their UP. Therefore, this study emphasizes genetic heterogeneity in UP. Future study to identify primary molecular determinants of UP should include affected sib-pair studies.     

Paraneoplastic vasculitis associated with multiple myeloma

The association between vasculitis and cancer has been widely reviewed in recent decades. The existence of malignancies in patients with vasculitis has been estimated at about 4.5-8%, haematological neoplasms being the most frequently observed. The haematological malignancies most frequently described are lymphoproliferative diseases such as hairy cell leukaemia and lymphomas. On the contrary, the incidence of paraneoplastic vasculitis in patients with myeloma is low; up to now, we have found nine cases reported on this subject. We report the case of a 73-year-old woman who in 1 year showed three outbreaks of acutely painful, purpuric and ulceronecrotic lesions, localized on the lower extremities. Histopathological study demonstrated thrombosis in the arterioles and leucocytoclastic vasculitis. Complementary tests revealed the presence of multiple myeloma.