前臂结节性筋膜炎一例

患者,男,31岁。右前臂皮下结节5天,伴轻度压痛不适。高频超声检查示：表皮连续微隆起,真皮深层及皮下组织可见一类椭圆形低回声结节,边界清楚,体积约6.30 mm×6.23 mm×3.20 mm。皮损组织病理示：真皮及皮下组织大量梭形细胞呈束状排列,胶原纤维增生,间质黏液样变,伴少量淋巴细胞浸润及红细胞渗出。诊断：结节性筋膜炎。     

Hyalinized collagen in a dermatofibrosarcoma protuberans after treatment with imatinib mesylate

We report a novel histological finding in a dermatofibrosarcoma protuberans (DFSP) after treatment with imatinib mesylate: copious amounts of hyalinized collagen. A 57-year-old female was referred for evaluation and treatment of a 7 x 8 x 10 cm tumor on the right anterior shoulder. Histological evaluation of the incisional biopsy showed a highly cellular dermal neoplasm composed of spindle cells arranged in a storiform pattern with minimal collagen. A CD34 immunohistochemical stain was strongly positive, highlighting atypical spindle cells in the dermis. A diagnosis of DFSP was made and the patient was enrolled in the Southwest Oncology Group trial. She received imatinib mesylate 400 mg per day for 1 year. At the end of therapy, the DFSP decreased in size to 5.5 x 4 x 4 cm. The tumor was excised. Histological evaluation showed a residual dermal neoplasm composed of atypical spindle cells and a copious amount of hyalinized collagen. Areas of necrosis were not seen. A CD34 stain confirmed the presence of residual DFSP, highlighting atypical spindle cells. A procollagen I stain was strongly positive, confirming the presence of abundant collagen in the dermis. A similar finding has been reported in gastrointestinal stromal tumor, which shows deposition of myxohyaline stroma after treatment with imatinib mesylate.     

CD1A Rich Inflammatory Stage of Lichen Sclerosus Et Atrophicans Mimicking Mycosis Fungoides and Extramammary Paget''S Disease

Infantile digital fibromatosis (IDF), also called Reye tumor or inclusion body fibromatosis, is a benign proliferation of myofibroblasts, with characteristic perinuclear eosinophilic cytoplasmic inclusion bodies. The inclusions stain pink with hematoxylin‐eosin, red with Masson's trichrome, and purple with phosphotungstic acid‐hematoxylin. This intradermal neoplasm is formed by interlacing bundles of collagen and spindle cells. Positive immunohistochemical staining with vimentin, cytokeratin, desmin, and actin indicate myofibroblastic cell origin. Clinically, patients present at birth or infancy with asymptomatic smooth, flesh colored nodules, typically on lateral or dorsal digits. IDF is a benign neoplasm that may spontaneously regress and treatment is observation. Lesions recur in 60% of excisions; surgery is only recommended in cases of functional impairment or aggressive growth. We report three cases of IDF. The patients, ranging 8 months to 8 years old, presented with pink to flesh colored nodules on the feet and fingers. A spectrum of histological features was observed. All cases revealed dermal proliferation of spindle cells with blunt nuclei and eosinophilic intracytoplasmic inclusions. Pattern of spindle cells ranged from loose haphazard arrangement to well‐organized interlacing bundles. The quantity of eosinophilic inclusions varied. For diagnostic and treatment purposes, all three patients underwent excision, with subsequent recurrence in two cases.     

甲母质瘤一例并文献复习

报道一例甲母质瘤并对相关文献进行复习。患者，男，54岁。右手食指指甲增厚、变黄10年。组织病理检查：甲板显著增厚，甲母质细胞V形增生，底端呈条索状伸向真皮，上端呈乳头瘤样伸向甲板，上覆嗜酸性角质增生带，部分乳头瘤顶端见渗出结痂。真皮浅层梭形细胞增生，排列杂乱，间有散在肥大细胞，深部纤维及纤维细胞增生，排列与表皮平行。免疫组化染色：梭形细胞CD34阳性，S-100阴性，FⅩIIIa阴性，CD68阴性，CK7阴性。诊断：甲母质瘤。手术切除肿瘤后随访10个月无复发。     

黏液性皮肤纤维瘤

报告1例黏液性皮肤纤维瘤.患者女,43岁.右前臂丘疹2周余.皮肤科检查:右前臂一直径约0.8 cm结节.组织病理检查示表皮不规则增生,真皮内可见大量梭形细胞增生,局部胶原排列疏松,阿新蓝染色阳性.免疫组化染色结果示波形蛋白(vimentin)阳性,CD31、CD34、平滑肌肌动蛋白(SMA)和S-100蛋白阴性.诊断为黏液性皮肤纤维瘤.     

肿胀性红斑狼疮1例

患者男，36岁。因面部、双上肢和背部反复起水肿性红班2年。实验室检查免疫学指标均正常，皮损直接免疫荧光检查阴性。组织病理检查示真皮浅层和深层血管周围以及附属器周围有淋巴细胞、组织细胞浸润。阿新蓝染色在真皮胶原纤维束间有大量黏蛋白沉积。     

Annular atrophic plaques of the skin (Christianson's disease)

A 69-year-old Caucasian man presented to the Gainesville Veterans Administration Medical Center for evaluation of several asymptomatic enlarging lesions on the face and forearms that had been present for 10 to 15 years. They were initially small but had progressively enlarged, especially during the previous 5 years. He reported having sustained a concussive grenade blast injury of the left temple and right forearm during the Korean Conflict in 1951. The injured areas healed uneventfully in 2–3 weeks. The patient was otherwise healthy, and the review of systems was noncontributory. Laboratory work-up (complete blood count, chemical profile, erythrocyte sedimentation rate, rheumatoid factor, hepatitis profile, antinuclear antibodies, urinalysis, and chest X-ray) was within normal limits. Lyme antibody titers were negative. Physical examination revealed similar-appearing lesions located variously on both temples, left preauricular and infraauricular areas, and the right forearm (Fig. 1). The lesions were large (ranging from 4 to 12 cm in diameter), centrally atrophic patches with ivory-colored thickened edges, and had the distinct appearance of healed skin grafts. Blood vessels were easily seen through the atrophic skin. All lesions were located on sun-exposed areas (predominantly on the patient's left side). No similar lesions elsewhere on the body or skin graft donor sites were found. The patient was treated with doxycycline 100 mg by mouth twice a day for 21 days to no avail, Intralesional steroids and clobetasol propionate ointment under occlusion were used without any change in the lesions. At the submission of this paper, the lesions remain stable. Skin biopsies were taken from the edge and center of the lesions, Histologic examination (Fig. 2) of the center of a lesion showed a flattened epidermis without interface changes. Sclerosis of superficial and mid, but not deep, dermal collagen was present, particularly within the specialized connective tissue surrounding eccrine structures. A focus of lymphocytic inflammation with rare plasma cells and edema was present at the dermal-subcutaneous interface. Histologic examination of the edge of the lesion showed a flattened epidermis overlying a perivascular and periadnexal lymphohistiocytic inflammatory infiltrate. The papillary dermis was sclerotic centrally, and sclerosis was present around one eccrine unit. Increased interstitial mucin was noted. Direct immunofluorescence using antibodies to IgG, IgM, IgA, and C3 was negative, A silver stain for borrelia organisms was negative. Electron microscopy of a biopsy from the advancing border of a lesion revealed foci of complete cytoiysis of the basilar epidermis above an interrupted basal lamina (Fig, 3a). Cellular debris was evident even within the subajacent papillary dermis (Fig. 3b). Abundant colloid-like material associated with occasional 8–10-nm straight tubules was also present within the upper papillary dermis. A solitary lymphocyte lay closely apposed to a basilar keratinocyte having large cytoplasmic vacuoles. Electron microscopy of a biopsy from the center of the same lesion revealed a flattened basilar epidermis and focal reduplication and interruption of the basal lamina (Fig. 3c). Abundantly interspersed among the collagen bundles of the upper and lower papillary dermis were aggregates that appeared amorphous at low magnification. Upon higher magnification, however, the aggregates could be seen to be comprised of straight and wavy tubules embedded in a finely granular matrix (Fig. 3d). A dermal blood vessel was surrounded by several basal laminae, just outside of which were presumptive lymphocytes with cerebritorm nuclei and histiocytes. No dermal elastic fibers could be identified. Collagen fibrils appeared normal.     

Extra nuchal-type fibroma associated with elastosis, traumatic neuroma, a rare APC gene missense mutation, and a very rare MUTYH gene polymorphism: a case report and review of the literature*

We report a case of an extra nuchal-type fibroma in a 51-year-old male suspected to have attenuated familial adenomatous polyposis (Gardner's syndrome), who presented with a longstanding buttock mass excised due to enlargement and pain. Histopathologically, lobules of haphazard, hypocellular, hyalinized collagen bundles replaced the dermis and subcutis and entrapped nerve bundles, mimicking Morton neuroma. Ramifying nerve twigs found around larger nerve fascicles showed the co-existence of traumatic neuroma. Elastic tissue stain revealed elastosis characterized by large, arborizing fibers lying between and within the hyalinized collagen bundles. Modified Masson's trichrome stain showed light blue staining of collagen bundles producing the hyalinized nodules with irregular, light red staining of collagen bundles at their periphery and within tumor collagen. Compression and/or degeneration of collagen and secondary elastosis with later entrapment by tumor collagen could explain this microscopic phenotype. By immunohistochemistry, tumor spindle cells expressed nuclear β-catenin and cyclin D1, mostly within regions of fibrosis implicating activation of the adenomatous polyposis coli (APC)-Wnt pathway. Genetic analysis showed a missense mutation in APC gene (c.7504G>A, p.G2502S in exon 15) and a functional homozygous polymorphism in the MUTYH gene (c.36+325G>C, (IVS1+5G/C)). Nuchal-type fibroma has been associated with Gardner's syndrome and trauma. In this patient, genetic predisposition coupled with repetitive, localized trauma and collagen degeneration may have provided the stimulus for the development of extra nuchal-type fibroma.     

以斑状脱发为首发症状的弥漫性神经纤维瘤一例

患者男,42岁,因头部斑状脱发伴头皮肿胀2年余就诊。皮肤科检查：左侧头皮见一10cm×8cm的肿块,境界清楚,略高起皮面,表面毛发脱落,触之柔软。皮损组织病理检查：肿瘤位于真皮深部和皮下脂肪组织,主要由短梭形细胞构成,可见特征性Meissner小体样结构;肿瘤基质疏松,散在肥大细胞。免疫组化：50％肿瘤细胞表达S-100。诊断：弥漫性神经纤维瘤。确诊后,鉴于皮损累及头部面积较大,患者拒绝手术治疗,目前仍在随访中。     

Nodular morphea in a linear pattern.

A 9-year-old black girl presented with papules and nodules in a linear distribution on her right inner arm, right axilla, and right chest ( Fig. 1 ). The lesions were hyperpigmented, indurated, and had been present for 3 years. She was otherwise healthy. A biopsy of one of the lesions revealed a sparse superficial and deep perivascular infiltrate of lymphocytes, compactly arranged collagen bundles in the upper part of the dermis, and abundant mucin throughout the reticular dermis, which was consistent with morphea with dermal mucinosis ( Fig. 2 ). Biopsy of another lesion revealed classic histologic features of morphea: thickened bundles of collagen crowded in the reticular dermis aligned parallel to the skin surface.

Figure 1 Open in figure viewer PowerPoint Discrete nodules in a linear distribution on the right arm     

甲状腺乳头状癌合并嗜酸性蜂窝织炎1例

患者女,14岁。甲状腺乳头状癌次全切术后7周,双小腿红肿2周。体检:双下肢可见大片水肿性红斑,界限不清,触诊有浸润感,无压痛,皮温高。外周血嗜酸性粒细胞计数升高。皮损组织病理示:表皮灶性海绵水肿,真皮浅层水肿,真皮全层可见血管周围及胶原束间多数嗜酸性粒细胞浸润,血管周围见中等密度组织细胞、淋巴细胞浸润。诊断:嗜酸性蜂窝织炎。     

Two cases of male nipple leiomyoma: idiopathic leiomyoma and gynecomastia-associated leiomyoma

We describe 2 cases of male nipple leiomyoma. A 70-year-old man had a painful subcutaneous tumor on his left nipple of 6 months duration. Histopathology disclosed dermal spindle cells with oval-shaped nuclei forming interlacing bundles with irregular pattern. Glandular elements were absent. The spindle cells were positive to α-smooth muscle actin, desmin, and vimentin. Estrogen receptor (ER) and progesterone receptor (PrR) were negative. We diagnosed this case as male leiomyoma of the nipple. Another patient was a 61-year-old man with gynecomastia induced by spironolactone of 6 months duration. He also had a painful nodule on his left nipple and histopathology disclosed spindle-shaped tumor cells as in the previous patient. The tumor was accompanied by glandular elements in the deep dermis and subcutaneous tissue, which showed apocrine secretion and were positive for α-smooth muscle actin, ER, and PrR. These glandular elements were interpreted as mammary gland. But ER and PrR stain did not show positive results for leiomyoma in the upper dermis. To the best of our knowledge, this is the first report of male idiopathic and gynecomastia-induced leiomyoma with ER and PrR staining.     

席纹状胶原瘤1例

患者男,20岁,左侧鼻翼发疹7年,渐增大。皮肤科情况:左侧鼻翼有一枚直径约0.9 cm×0.9 cm大小皮色结节,表面光滑,质地坚韧。皮损组织病理示:肿瘤占据真皮全层,边界尚清,无包膜。瘤体主要由席纹状或旋涡状排列的胶原纤维束组成,结构致密,散布星状或梭形纤维母细胞。诊断:席纹状胶原瘤。     

Focal dermal hypoplasia: ultrastructural abnormalities of the connective tissue

We followed over 10 years three girls with focal dermal hypoplasia syndrome. The histopathological changes demonstrated at the optical level an hypoplastic dermis with thin and scarce collagen bundles and a marked diminution of elastic fibers. Mature adipose tissue was found scattered within the papillary and reticular dermis. No alterations in the basal membrane were observed by immunocytochemical or ultrastructural techniques. Ultrastructurally, in the skin-affected areas, loosely arranged collagen bundles composed of few fibrils were seen scattered in the extracellular matrix. Scarce elastic fibers of normal morphology were also observed. Fibroblasts were smaller, oval-shaped, and diminished in number with a poorly developed cytoplasm. In these fibroblasts, the most conspicuous feature was a remarkable and irregular thickening of the nuclear fibrous lamina. Taking into account that a common link between all laminopaties may be a failure of stem cells to regenerate mesenchymal tissue, this failure would induce the dermal hypoplasia observed in our patients presenting Goltz syndrome.     

Giant dermatofibroma with monster cells.

We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor XIIIa was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.     

Ultrastructure of skin and hair of an Egyptian mummy

The skin of a female Egyptian mummy, embalmed 100–300 A.D., was studied by transmission and scanning electron microscopy.
After rehydration with cacodylate buffer containing sucrose, the ordinary technique for transmission electron microscopy was performed on skin of the right shoulder. In epidermal cells, mitochondria and their cristae were identified. Between epidermal cells, desmosomes, half the normal size, were found. In the dermis, collagen bundles and elastic fibres were observed. The collagen fibres had a diameter of 67–91 nm, and the band pattern showed 10–12 sub-bands per one period of 55–61 nm length. The elastic fibres had less amorphous substance than normal and a fibrillar beaded pattern. The dermal components of the skin were well preserved in spite of the mummification.
Throughout the dermis and the subcutaneous fatty tissue, a number of bacterial spores were found showing a characteristic appearance.
Scanning electron microscopy of hairs showed a layer of the preserving material surrounding hairs, and under it a normal cuticle was seen.     

黏蛋白痣10例临床病理分析

【摘要】 目的 探讨黏蛋白痣的临床病理特征。方法 回顾性分析2014年1月至2019年12月在中国医学科学院皮肤病医院经临床及组织病理确诊的10例黏蛋白痣患者的临床和病理资料。结果 10例黏蛋白痣均为儿童期发病,平均发病年龄6.5岁。7例皮损位于躯干,其中4例位于背部;2例位于四肢,1例躯干、四肢泛发。皮损在局部排列成线状、带状或簇集状,质地柔软至坚硬不等,颜色呈肤色、淡红色和黄色。组织病理检查：10例均表现为真皮内胶原纤维束排列紊乱,其间可见程度不等的黏蛋白沉积,沉积的位置和程度不一,6例在沉积区见胶原纤维增粗、红染,其余4例表现为胶原稀疏、减少;2例出现基底层灶状液化变性,3例出现真皮内不等量成熟脂肪组织等。结论 黏蛋白痣的病理主要表现为真皮内程度不等的黏蛋白沉积于杂乱的胶原束间,可与一些其他疾病类似,容易误诊,临床和病理紧密联系可确诊。     

间质性蕈样肉芽肿一例

患者女,36岁,因躯干、四肢红斑、鳞屑伴瘙痒3年就诊。皮肤科检查：患者胸腹部、臀部可见边界不清的淡红斑,其上覆层状白色鳞屑。皮损组织病理学检查：真皮浅层单一核细胞灶状浸润,部分细胞有亲表皮性,亲表皮细胞有轻度异形,真皮中层胶原纤维束间可见单一核细胞呈间质性浸润和少许黏蛋白沉积。真皮浅中层散在浸润的单一核细胞均阳性表达T辅助细胞免疫表型。诊断：间质性蕈样肉芽肿。     

Acquired cutaneous lymphangiectasia with mesothelial cells reflux in a patient with cirrhotic ascites

A previously undescribed case of acquired cutaneous lymphangiectasias on the abdomen in a patient with cirrhotic ascites where peritoneal mesothelial cells refluxed in the skin is discussed. A 56-year-old man previously submitted to liver transplantation presented with vesiculobullous lesions on the developed as his cirrhotic ascites progressed. Histology showed dilated lymphatic channels in the upper dermis lined by a single, discontinuous layer of flattened, monomorphous endothelial cells with endoluminal papillary projections. In the deep reticular dermis, we observed irregular thin- often jagged-walled vascular channels lined by a single layer of bland endothelial cells, dissecting the collagen bundles. Vessels in the lumen were medium to large bizarre-shaped polygonal cells with abundant eosinophilic cytoplasm and hyperchromatic and irregular nuclei, arranged in small clusters or as solitary units, focally in close contact with the endothelial lining or free floating within vessel cavities. Immunohistochemistry indicated atypical intraluminal cells to be positive for calretinin, a specific marker for mesothelial cells. Pathophysiologic mechanisms and problems of differential diagnosis of this unique clinicopathologic entity are discussed.     

Granulomatous dermatitis with enlarged histiocytes: a characteristic pattern of granulocyte colony-stimulating factor. Report of two cases and review of the literature

Two patients developed a pruritic maculopapular rash following treatment with granulocyte colony-stimulating factor (G-CSF). Histopathological examination disclosed a superficial dermal inflammatory infiltrate composed of interstitially arranged large histiocytic CD68+ cells and perivascularly disposed lymphocytes. Large histiocytes were interspersed among the collagen bundles with associated slight deposits of mucin. Elastophagocytosis was occasionally observed, and a slight reduction of dermal elastic fibers was also noted. There was no evidence of frank granuloma formation, fibrosis or significant changes of collagen bundles. This histopathological variant of granulomatous dermatitis with "enlarged histiocytes" seems to be characteristic of an unusual cutaneous reaction secondary to CSF treatment.