儿童色素减退型蕈样肉芽肿一例

患儿,女,5岁。全身出现散在分布的色素减退斑、淡红色斑1年。组织病理检查示：表皮轻度萎缩,可见淋巴细胞移入表皮,真皮浅层血管周围淋巴组织细胞浸润。免疫组化示：CD3(+++),CD4(表皮内散在阳性,真皮内+),CD8(表皮内++,真皮内+),CD20(-),CD79a(-),Ki-67(index约10%)。诊断：色素减退型蕈样肉芽肿。     

皮肤上皮样血管瘤性结节一例

皮肤上皮样血管瘤性结节是一种少见的良性皮肤血管增生性疾病,临床表现为单发或多发的红色或紫红色的斑块、结节,好发于躯干及四肢,本文报道一例。患者,女,47岁。背部红色结节3个月伴轻微瘙痒,组织病理示结节位于真皮浅层,周围无包膜,由上皮样血管内皮细胞增生形成,无明显异型性,大量管腔样结构形成,免疫组化示：CD31(+), CD34(+), D2-40(+), Fli-1(+), KP-1(+), Ki-67(20%+),溶菌酶(+)。诊断：皮肤上皮样血管瘤性结节。治疗：行手术切除,随访至今2年未复发。     

上皮样肉瘤1例

患者男,61岁。左上肢多发性结节2年。皮损组织病理示:瘤组织位于真皮层,呈结节状,结节中心坏死,坏死周边肿瘤细胞呈上皮样或梭形,核分裂相易见。免疫组化染色示:CK(2+),EMA(2+),Vim(+),CD34(3+),CD31(+)。诊断:上皮样肉瘤。     

大疱性类天疱疮合并原发性腹膜后弥漫大B细胞淋巴瘤1例

患者男,77岁。躯干、四肢反复红斑、水疱1年余,加重10d,确诊为大疱性类天疱疮。患者发病4年后体检时发现腹膜后肿物,外科手术切除肿物后组织病理显示淋巴样细胞弥漫性浸润,免疫组化示CD20(+),PAX-5(+),Bcl-6(灶+),MUM-1(+),Ki-67(90%+)。诊断:原发性腹膜后弥漫大B细胞淋巴瘤。     

下唇部血管平滑肌瘤

报告1例下唇部血管平滑肌瘤。患者男,30岁。左下唇结节3年,渐增大,偶伴有疼痛。皮损组织病理检查示平滑肌细胞增生,肿瘤团块内伴有大量不规则血管腔。免疫组化示血管内皮细胞CD31及CD34阳性,平滑肌细胞的平滑肌肌动蛋白(SMA)阳性。诊断:血管平滑肌瘤。予手术治疗后至今未复发。     

皮脂腺痣合并毛母细胞瘤1例

 报告1例皮脂腺痣合并毛母细胞瘤。患者男,39岁。左面部皮疹30余年,无自觉症状。皮肤专科情况：左面部见一个4 cm× 2 cm大小的皮色斑块,表面粗糙。斑块基础上可见两个约黄豆大小、表面光滑的蓝灰色半球形肿物和一个黄豆大小的乳头状淡红色肿物,质中。浅表淋巴结未触及肿大。皮肤镜下可见蓝灰色均质结构、分枝状血管、黄白色叶状结构。皮损组织病理示：真皮浅层内可见大量皮脂腺、由基底样细胞构成的上皮性肿瘤,伴色素沉着,肿瘤间质纤维组织增生,基底样细胞具有毛囊特征。免疫组化示AR(-), Ki-67（15%+）, BCL-2（+）,CD10(灶性+),CD34(-),CK20（散在+）,Ber-EP4（+）。结合临床表现及病理检查,诊断为皮脂腺痣合并毛母细胞瘤。手术完整切除皮损,随访半年无复发。     

上皮样肉瘤1例

患者男,26岁。左上肢多发性结节、溃疡4年。皮肤组织病理检查示:真皮肿瘤团块呈结节状分布,结节由多数上皮细胞样细胞和少数梭形细胞组成,中央有大片状坏死区。免疫组化染色结果示:EMA(2+),波形蛋白(2+),CD34(+),CK(2+)。诊断为上皮样肉瘤     

成人种痘样水疱病样淋巴瘤一例并文献复习

本文报道1例种痘样水疱病样淋巴瘤并对WHO最新分类、EBV感染相关发病机制、鉴别诊断及治疗方面的相关文献进行复习。患者,男,38岁。全身反复皮疹30年,加重1年,患者病程中伴有发热,皮损预后遗留凹陷性瘢痕。血清EB病毒抗体IgG阳性。皮损组织病理示：真皮全层及皮下脂肪小叶内大量淋巴细胞浸润。免疫组化染色示：CD2(+++),CD3(++),CD8(++),TIA-1(++),CD56(少量+),CD20(灶状+) ,Ki-67＞30%。EB病毒原位杂交：阳性。诊断：种痘样水疱病样淋巴瘤。     

具有多种表现的蕈样肉芽肿一例

患者,男,80岁。手足反复出现多种皮损伴瘙痒、疼痛4年,加重1年。皮肤活检和组织病理示：表皮内及真皮浅层血管周围灶状淋巴样细胞浸润。免疫组化示：CD3（+）、CD4（+）、CD5（-）、CD7（+）、CD8（+）、CD20（+）,Ki67（10％）。诊断：蕈样肉芽肿。予以抗组胺药物、糖皮质激素药膏、窄谱中波紫外线照射治疗,随访。     

外阴疣状黄瘤

报告1例外阴疣状黄瘤.患者女,47岁.外阴丘疹6个月.皮损组织病理学检查示:表皮呈乳头瘤样增生,真皮乳头较多泡沫细胞;免疫组化染色示CD68阳性.手术切除后随诊9个月无复发.     

幼年隆突性皮肤纤维肉瘤

报告1例幼年隆突性皮肤纤维肉瘤。患儿男,8岁。左侧腹股沟皮肤红色结节5个月。皮肤科检查：左侧腹股沟皮肤见一花生米大的半球形粉红色结节,表面光滑,境界清楚,结节质中等,与皮肤粘连,浸润感明显。皮损组织病理检查：表皮基本正常,真皮有增生的梭形细胞组成的细胞团块,部分梭形细胞呈特征性席纹状排列,未见明显细胞异形及核分裂象。免疫组化：增殖核抗原（Ki-67）(5%+),CD34(+),S-100蛋白（-）。分子生物学检测：COL1A1-PDGFB融合基因比例70%。诊断：隆突性皮肤纤维肉瘤。治疗上予扩大切除术辅以术后放疗,随访无复发。     

Phenotypic heterogeneity of lymphoma of the skin

We studied surface markers present in 56 cases of lymphoma of the skin by immunohistochemical staining, using the ABC (avidin-biotin-peroxidase complex) and PAP (peroxidase-antiperoxidase complex) methods. Of these cases, 49 were T-cell lymphoma and 7 were B-cell lymphoma. Ten of the 49 cases of T-cell lymphoma were adult T-cell leukemia/lymphoma (ATL). Twenty-five of 31 cases of T-cell lymphoma except ATL analyzed by the ABC method showed a helper/inducer phenotype (Leu2a-,Leu3a+), two cases showed a suppressor/cytotoxic phenotype (Leu2a+, Leu3a-), one case showed Leu2a+Leu3a+, one case showed an inducer phenotype (Leu2a-, Leu3a+, Leu9+), and one case showed OKT11+, Leu2a-, Leu3a-, Leu1-, Leu9+, CD25+, Leu10+, CD30+. One CD8+ lymphoma was Pagetoid reticulosis, and a CD4+, CD8+ lymphoma was lymphomatoid papulosis with erythematous plaque. Cutaneous T-cell lymphoma (CTCL), previously described by Edelson et al., is defined as a helper T-cell lymphoma with marked affinity for the skin. In our study, 5 cases of T-cell lymphoma of the skin were not CTCL as described by Edelson et al. These results show that T-cell lymphoma of the skin is heterogeneous in nature. In other words, CTCL is one type but represents a major proportion of T-cell lymphomas of the skin.     

Fc epsilon R11/CD23 receptor distribution in patch test reactions to aeroallergens in atopic dermatitis.

There is increasing evidence that exposure to organic allergens may induce or exacerbate lesional skin in patients with atopic dermatitis. In this study, patients with atopic dermatitis were patch tested to 11 common organic allergens and to control chambers containing 0.4% phenol and 50% glycerin in 0.9% saline. In biopsies from positive patch test reactions, patch test control skin, lesional eczematous and non-lesional skin from atopic individuals, and normal skin from non-atopic volunteers, the presence and distribution of macrophages (RFD7+), dendritic cells (RFD1+), and Langerhans cells, and the expression of the low-affinity receptor for IgE (CD23) were investigated. In patch test reactions and lesional skin samples, inflammatory infiltrates of diffusely distributed macrophages (RFD7+), dendritic cells (RFD1+), T lymphocytes (RFTmix+), and Langerhans cells (CD1+) were seen, the latter being present in both the epidermis and the dermis. The numbers of Langerhans cells were reduced in the epidermis and increased in the dermis in patch test reactions and lesional skin compared to their controls. Double staining revealed a change in the distribution of CD23 antigen. In patch test control and non-lesional biopsies many macrophages and only a few Langerhans cells within the dermal infiltrates expressed this antigen. In patch test reaction and lesional skin samples, however, the proportion of CD23+ dermal Langerhans cells had increased compared to macrophages. Furthermore, in these latter samples an increased proportion of dermal CD1+ cells expressed the dendritic cell (RFD1+) marker. These results show that following antigen challenge there are marked similarities between the phenotype of the cellular infiltrate in patch test reaction and lesional skin biopsies, and also demonstrate a changing distribution of CD23 on antigen-presenting cells.     

An unusual case of sarcomatoid renal cell carcinoma presenting in the skin by direct extension at a laparoscopic port site

Although renal cell carcinoma (RCC) is known for its propensity to metastasize widely throughout the body, cutaneous metastases are uncommon and seen in less than 3% of RCCs. A 56-year-old man presented with a painful red lesion with satellite nodules on his abdomen at a laparoscopic port site from a partial nephrectomy for a pT1a clear-cell RCC that was performed 28 months prior. The lesion was excised; however, after excision the lesion recurred with continued pain and drainage from the surgical site. This was treated with multiple courses of antibiotics. Because of the persistent nature of the lesion, it was re-biopsied, and an atypical, keratin-positive, spindle-cell proliferation was identified within the dermis. The patient's previous skin excision was reexamined, and the same atypical cells were identified within large areas of necrosis, granulation tissue, and fibrosis. Further workup was performed on the initial excision, and the atypical cells showed expression of CD10, CAIX, PAX8, EMA, and vimentin, consistent with cutaneous involvement by RCC. Because of the rarity of skin metastases in RCC and the location at a previous laparoscopic port site, this lesion is presumed to have resulted from direct extension of tumor at the time of surgery.     

A Case of Cutaneous B-cell Lymphoma with a Storiform Stromal Reaction

A case of cutaneous B-cell lymphoma is described. The patient was treated only by surgical excision of the skin tumors five times during a period of about two years from February of 1984 to October of 1986. After the last surgical excision, a continuous disease-free period was achieved. Biopsy samples showed dense lymphocytic infiltrations with discrete masses in the dermis and subcutis; one of them showed a storiform pattern. At the time, the infiltrating cells were composed of medium and large lymphoid cells and spindle-shaped cells. The medium and large lymphoid cells were positive for CD20, CD22 and HLA-DR and negative for CD3, CD4, CD5, CD8, CD43, and kappa and lambda light chain. The spindle-shaped cells were negative for CD20, CD43, kappa and lambda light chain, lysozyme, and S-100 protein.     

Expression of cutaneous lymphocyte-associated antigen and TIA-1 by lymphocytes in pityriasis lichenoides et varioliformis acuta and lymphomatoid papulosis: immunohistochemical study

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) are benign self-healing cutaneous eruptions that may be clinically and histologically similar. The purposes of this study were to evaluate immunohistological characteristics of PLEVA and LyP and to investigate whether Epstein-Barr virus (EBV) may be present in PLEVA and LyP. METHODS: We performed an immunohistochemical staining in 12 cases of PLEVA and 8 cases of LyP using nine antibodies for CD3, CD4, CD8, CD30, CD45RO, CD56, CD79, cutaneous lymphocyte-associated antigen (CLA), and TIA-1. In situ hybridization was performed using fluorescein-conjugated oligonucleotide probes for EBV early regions (EBER). RESULTS: In PLEVA, immunohistochemical studies revealed that infiltrated lymphocytes consisted of mainly CD3-positive (5+), CD8-positive (4+ to 5+), CLA-positive (4+ to 5+) T cells and partly CD79 positive (+ to 2+) B cells. CD4-positive T cells were less than 25%. In LyP, immunohistochemical studies revealed that infiltrated lymphocytes consisted of partly CD3-positive (5+), CD8-positive (2+ to 3+), CLA-positive (3+ to 4+) T cells and partly CD79-positive (2+ to 3+) B cells. CD4-positive T cells were less than 10%. CD8 and CLA were more strongly expressed in PLEVA than in LyP. CD30 was strongly expressed in LyP but not expressed in PLEVA. CD79 was more expressed in LyP than in PLEVA. TIA-1 was not expressed in any cases. In situ hybridization using antisense EBER probe showed negative reaction in all cases. CONCLUSIONS: Immunohistochemical stains for CD8, CD30, CD79 and CLA may be valuable tools in the differential diagnosis between PLEVA and LyP. TIA-1 was negative in LyP, which means cytotoxic cells may not be implicated in the pathogenesis of LyP. It was a contradictory result to the previous results. The absence of EBV in PLEVA and LyP suggests that this virus may not be operative in the pathogenesis of these diseases. These results suggest that LyP and PLEVA are separate disorders, thus accounting for their variable prognosis.     

Lymphohistiocytic and granulomatous phlebitis in penile lichen sclerosus

Lichen sclerosus (LS) is a chronic inflammatory disease of unknown etiology that may affect the genital and/or extragenital skin of individuals of either sex at all ages. In boys, the prepuce is the most common site of involvement. The diagnostic criteria of LS include the presence of inflammatory infiltrates mainly composed of T lymphocytes. We report on two cases of LS of the prepuce because of the unusual feature of lymphocytic (CD45RO+ and CD20+), histiocytic (CD68+), and granulomatous phlebitis. This lesion was not present in a group of another 18 cases of childhood penile LS. We have not been able to find any references describing and illustrating inflammatory involvement of the dermal vein walls in LS. Unlike the data reported in the literature, the dermal inflammatory infiltrates of these two cases showed a similar proportion of B and T lymphocytes in addition to frequent CD68+ histiocytes.     

Ulcerated cutaneous epithelioid hemangioendothelioma.

Epithelioid hemangioendothelioma described first by Weiss and Enzinger in 1982 is an uncommon vascular tumor usually involving soft tissue, less frequently the lung and the liver and exceptionally the skin. We herein report a 52-year-old woman who presented an isolated moderately painful persistant ulceration of the concha of her left ear. Histopathological findings showed strands and nests of epithelioid endothelial cells typical of cutaneous epithelioid hemangioendothelioma. Immunohistochemical stainings confirmed the vascular nature of the tumor. Surgical excision by ear amputation was performed. In a review of the literature, to our knowledge, this clinical presentation as ulceration has never previously been reported.     

Pedunculated Nodules as a Variant of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare disease of dermal fibroblastic origin that accounts for less than 5% of all soft tissue sarcomas in adults. DFSP grows slowly and is an asymptomatic lesion at the initial diagnosis. Herein, we report a case of multiple pedunculated nodules as a variant of DFSP. A 47-year-old man presented with a 7-month history of multiple well-circumscribed, firm, pedunculated nodules on the inguinal area. Histopathologic examination results showed densely packed uniform spindle cells with a storiform and cartwheel pattern, and positivity for CD34. Wide excision and skin graft were performed and at the 6-month follow-up, there was no evidence of recurrence or metastasis.