150例皮肤纤维瘤组织病理与临床分析

目的 分析皮肤纤维瘤（DF）的组织病理与临床特征,探讨两者的相互联系。方法 回顾分析2017年9月至2018年8月在上海市皮肤病医院病理科经皮肤组织病理检查确诊的150例DF患者的临床和组织病理资料。结果 150例患者中,男65例,女85例,年龄（42 ± 13.8）岁,病程3个月至 > 30年,部分有伴随症状,主要表现为瘙痒,有自发痛、轻压痛,18例皮损有外伤、虫咬或感染病史。皮损主要位于四肢（107例,71.3%）,以单发皮损为主（105例,70%）。病理检查前拟诊为“DF”102例,“表皮样囊肿”16例,“色痣”13例,“瘢痕疙瘩”3例,“皮肤肿物”12例,“恶性黑素瘤”1例,“黄色肉芽肿”1例,“结节性痒疹”1例,“神经纤维瘤”1例。在这些患者的169张苏木精-伊红染色切片中,66张（39.1%）为细胞性,36张（21.3%）硬化性,25张（14.8%）表现为动脉瘤样型DF,22张（13.0%）上皮样型。12张切片内可见两种或两种以上亚型并存的现象。还可见少数新的变异型,如DF伴汗腺导管增生（1例）、深在型DF（3例）、上皮样细胞与增生胶原相间的DF（1例）等。动脉瘤样型DF病程长短不一,7个月至> 30年,多表现为发生于下肢的皮肤肿物样损害。细胞性DF病程相对较短,常发现数月后就诊,好发于四肢,常伴痒痛。硬化性或萎缩性DF病程长,为数年或数十年,好发于上肢,多无伴随症状。上皮样型DF病程长短不一,临床表现多样,多发于下肢,无伴随症状。结论 DF的临床表现及病理表现均具有多样性,不同的DF皮损可有相似的典型组织病理学表现,不典型的病理表现可干扰疾病诊断。     

Variants of dermatofibroma - a histopathological study

Several variants of dermatofibroma have been described. They are essentially distinguished by their clinical and histopathological features. To review the mainfeaturesof these variants, a retrospective study of skin biopsies and tissue excisions of dermatofibromasperformed in the dermatology and venereology service at the Hospital Garcia de Orta between May 2007 and April 2012 was carried out. During that period, 192 dermatofibromas were diagnosed in 181 patients, the lesions being more common in women. Median age of the study population was 48 years. The most common lesion site was the limbs (74% of patients). The histopathological types found were common fibrous histiocytoma (80%) and the aneurysmal (5.7%),hemosiderotic (5.7%), epithelioid (2.6%), cellular (2.1%), lipidized (2.1%), atrophic (1.0) and clear cell (0.5%) variants. Based on these findings, this review focuses on the clinical and histological features of the various variants of dermatofibroma in terms of their clinical presentation, distinct histopathological features, differential diagnosis and prognosis.     

Aneurysmal Benign Fibrous Histiocytoma with Atrophic Features

Aneurysmal benign fibrous histiocytoma is an uncommon pathologic variant of dermatofibroma. In addition to the features of a typical dermatofibroma, it has large cleft-like or cavernous blood-filled spaces with numerous hemosiderin pigments. It should be differentiated from angiomatoid malignant fibrous histiocytoma, malignant melanoma, and vascular tumors such as Kaposi''s sarcoma and angiosarcoma. Atrophic dermatofibroma is also a rare variant of dermatofibroma, and the combination of aneurysmal and atrophic features is rarer still. We report a case of aneurysmal benign fibrous histiocytoma with atrophic features in a 27-year-old male who had a grayish-brown atrophic patchy lesion on his back for 2 years.     

动脉瘤样纤维组织细胞瘤五例临床及病理特征分析

目的 探讨动脉瘤样纤维组织细胞瘤的临床特征及组织病理学诊断原则.方法 回顾性分析5例动脉瘤样纤维组织细胞瘤患者的临床及病理资料.结果 5例患者中,男3例,女2例.皮损为暗红色或棕色结节,3例皮损近期突然增大；3例位于四肢,2例位于胸部和下颌.皮损组织病理学检查:均具有典型皮肤纤维瘤的组织学特征,同时见多数不规则出血性裂隙和囊腔结构,伴有含铁血黄素沉积；免疫组化示波形蛋白和CD68阳性,血管性标记(CD34和CD31)均阴性.结论 鉴于AFH临床上具有近期快速增大的特点,组织学上显示出血性裂隙及囊腔形成,该肿瘤需要与血管肉瘤和血管瘤样纤维组织细胞瘤鉴别.     

Immunohistochemical analysis of S100-positive epidermal Langerhans cells in dermatofibroma

Dermatofibroma is a dermal fibrohistiocytic neoplasm. The Langerhans cells are the immunocompetent cells of the epidermis, and they represent the first defense barrier of the immune system towards the environment. The objective was to immunohistologically compare the densities of S100-positive Langerhans cells in the healthy peritumoral epidermis against those in the epidermis overlying dermatofibroma (20 cases), using antibodies against the S100 molecule (the immunophenotypic hallmark of Langerhans cells). The control group (normal, healthy skin) included ten healthy age and sex-matched individuals who underwent skin biopsies for benign skin lesions. A significantly high density of Langerhans cells was observed both in the epidermis of the healthy skin (6.00 ± 0.29) and the peritumoral epidermis (6.44 ± 0.41) vs. those in the epidermis overlying the tumor (1.44 ± 0.33, p < 0.05). The quantitative deficit of Langerhans cells in the epidermis overlying dermatofibroma may be a possible factor in its development.     

Case for diagnosis

We report the case of an 11-year-old male patient with a histopathological and immunohistochemical diagnosis of dermatofibroma with an atypical clinical presentation on the right forearm. Although dermatofibroma is considered a benign skin tumor, some of its differential diagnoses, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, are truly aggressive. Lesions with atypical clinical aspects and topology associated with specific histopathological variants are some of the criteria for complete tumor excision.     

Dermatofibroma in a black tattoo: report of a case

Tattooing has been associated with a variety of complications including inflammatory and granulomatous reactions, transmission of infections, and neoplasms. We report a case of a 24-year-old male who presented with a 2-month history of an erythematous nodule involving a newly made tattoo on the right leg. An excisional biopsy was performed and the histopathological evaluation was consistent with dermatofibroma. Only three cases of dermatofibroma associated with tatooing were reported in litetature. We report an additional case and review the literature regarding cutaneous reactions to tattoos.     

Congenital solitary reticulohistiocytosis (Hashimoto - Pritzker)

Congenital and self-healing Hashimoto-Pritzker reticulohistiocytosis is the benign variant of the Langerhans cell histiocytosis (LCH) group. It is characterized by multiple skin lesions (congenital or appearing during the first days after birth), without systemic manifestations and spontaneous resolution in days to months. The authors report the case of a boy with a single congenital leg skin lesion, a rare disease variant. Through histopathology, a dense skin infiltration of S100 protein-, CD1a-, CD207-immunomarked cells was found. KI67 index was high (62%). A complete spontaneous resolution occurred 07 days after the biopsy (25 days after birth). Monolesional disease, distal limb lesion, absence of lesions in the mucous membrane or seborrheic area, and less than 25 percent of LCs with Birbeck granules were said to be possible clues for a favorable prognosis in LCs histiocytosis. But, as a precautionary measure, the child will be followed up until at least 2 years of age.     

Acrosyringeal variant of extragenital lichen sclerosus et atrophicus

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis of the anogenital area, and approximately 15% to 20% of patients with LSA have extragenital lesions. Here, we report the case of an 18-year-old Korean man presenting with multiple asymptomatic punctated hypopigmented atrophic macules on the dorsa of both feet. Dermoscopic examination revealed hypopigmented atrophic macules with several central keratotic plugs. The histopathologic findings indicated LSA but were confined to the acrosyringium. Based on the clinical and histopathological findings, the patient was diagnosed with an acrosyringeal variant of extragenital LSA. The patient in this case showed a unique histopathological finding in which the typical features of LSA were confined to the acrosyringium, as well as an unusual clinical presentation of non-coalescing atrophic punctate macules on the dorsum of the feet.     

白色纤维性丘疹病

报告1例白色纤维性丘疹病。患者女，44岁。躯干四肢丘疹3年，无自觉症状。丘疹直径3-5mm，象牙白色或肤色，部分皮损萎缩。皮损组织病理检查显示真皮乳头层及网层上方胶原纤维增生，呈均质化改变。弹性纤维染色显示真皮浅层带状弱弹性纤维消失。     

Annular atrophic lichen planus

We describe a new case of annular atrophic lichen planus. Annular atrophic lichen planus is a rare variant of lichen planus that is clinically characterized by violaceous plaques of annular morphology with central atrophy and histopathologic findings of annular and atrophic lichen planus, respectively. There have been 4 cases of this entity reported previously.     

Atrophic dermatofibrosarcoma protuberans: a case report and reappraisal of the literature

Dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous malignancy. Unusual presentations described as atrophic have been documented. A case of DFSP with both clinical and histologic atrophy is presented, and all cases purporting atrophy with this tumor are reviewed. Meaningful trends are extracted from this data. In addition, the imprecise use of the term atrophic in regard to DFSP is clarified. We maintain that the variant of atrophic DFSP that mimics atrophoderma or anetoderma, as in this case, is the rarest variant of atrophic DFSP. Atrophic DFSP should be in the differential for depressed lesions on the trunks of women or on the lower extremities of children.     

180-kDa bullous pemphigoid antigen defective generalized atrophic benign epidermolysis bullosa: report of four cases with an unusually mild phenotype

Generalized atrophic benign epidermolysis bullosa (GABEB) is a rare variant of non-lethal junctional epidermolysis bullosa characterized by generalized skin blistering healing with atrophy and by atrophic alopecia with onset in childhood. Other features include mild mucosal blistering, dental abnormalities and nail dystrophy. We report four additional cases of GABEB from two families originating from the same isolated village. The patients shared an unusually mild clinical phenotype with cutaneous blisters strictly limited to trauma sites and rare occurrence of oral mucosal lesions. Scalp, eyelash and eyebrow alopecia was present in only two cases. Immunofluorescence studies showed a markedly reduced expression of the 180-kDa bullous pemphigoid antigen (BP180), and northern analysis of cultured keratinocytes indicated that the gene encoding for BP180 is affected in these GABEB patients.     

Atrophic dermatofibrosarcoma protuberans: case report, review, and proposed molecular mechanisms

We report a case of atrophic dermatofibrosarcoma protuberans, an uncommon clinical variant. We discuss molecular and genetic mechanisms of dermatofibrosarcoma protuberans tumorigenesis, review the literature on atrophic dermatofibrosarcoma protuberans, and pose a molecular mechanism for the atrophic variant's phenotype. We also describe using Mohs micrographic excision with CD34 immunostains on the frozen sections to definitively treat the tumor.     

Papulosis maligna atrophicans Degos Nicht immer maligne!

A patient developed malignant atrophic papulosis with only cutaneous manifestation. Repeated coloscopy uncovered no malignant papules in the colon. Referring to the literature, the value of permanent anticoagulant therapy is discussed. In contrast to the term "malignant" atrophic papulosis, there also seems to be a variant with a benign clinical course.     

Guttate morphoea in human T-cell lymphoma/lymphotrophic virus type-1 (HTLV-1) infection

A 62-year-old Japanese man presented with multiple small atrophic macules on the trunk and extremities. The lesions were discrete, oval in shape and enclosed by lilac ring. They were distributed in a Christmas tree distribution, reminiscent of pityriasis rosea. Skin biopsy showed increased collagen fibres in the dermis and invading subcutaneous tissue. The clinico-pathological features were consistent with guttate morphoea, a rare variant of localized scleroderma. Serological tests revealed a positive reaction to human T-cell lymphoma/lymphotropic virus type-1 infection.     

Multibranched acquired periungual fibrokeratomas with confounding histopathologic findings resembling papillomavirus infection: a report of two cases

Acquired periungual fibrokeratomas are benign fibrous tissue tumors and are considered as the topographical variant of acquired digital fibrokeratoma. They usually present as solitary tumors. In some instance, the entity may appear in multibranched fashion. The main histopathologic features consist of acanthosis, thick collagen bundles mainly oriented in a vertical axis forming a central core, and numerous proliferating fibroblasts. In this article, we present two cases of acquired multibranched periungual fibrokeratoma and depict their varying clinical features over time. Binucleation and perinuclear halos of keratinocytes mimicking human papillomavirus (HPV) infection were detected microscopically, but there was no reactivity with HPV immunostaining. In context, anti‐HPV immunostaining may be helpful in the differentiation of fibrokeratomas from HPV infection. On the other hand, it should be kept in mind that these histopathologic findings may be found in acral biopsies independent of viral effects.     

进行性结节性组织细胞瘤1例

患者男,20岁。全身弥漫性黄瘤样皮疹、结节6年余。皮肤科情况:全身弥漫性、对称性分布圆形、光滑的黄褐色丘疹、结节,丘疹直径2～8mm,结节直径7～40mm,口腔黏膜、生殖器部位可见数个类似皮损。皮损组织病理:病灶浸润真皮全层,细胞团灶状排列,可见泡沫细胞团,组织细胞及Touton样多核巨细胞,周边有圆形细胞浸润及成纤维细胞免疫组化CD68(+),S-100(-)。诊断:进行性结节性组织细胞瘤。     

A Case of Fibrous Histiocytoma Associated with Xanthogranulomatous Alteration

We report here an unusual case of fibrous histiocytoma in which an area of xanthogranuloma was observed histologically. Our case suggests that adult xanthogranuloma is a variant of fibrous histiocytoma.     

Scleromyxedema histopathologically mimicking hypercellular fibrous papules (angiofibomas): Case report of an unusual histopathological presentation

Scleromyxedema (SMX) is an inflammatory condition of unknown etiology strongly associated with monoclonal gammopathy. Classical histopathology of SMX is characterized with the triad of diffuse mucin deposits, increased amount of collagen, and presence of stellate fibroblasts. Herein, we report an unusual histopathological variant of SMX in a 41-year-old female with lesions of the nose histopathologically mimicking cellular angiofibromas. The dome-shaped papules were characterized by increased collagen bundles and fascicles of spindle cells. Widened vessels were seen at the periphery of the proliferation. Cells expressed CD68. Factor XIIIa was expressed only by dendritic cells. The mucin was highlighted with colloidal iron. In sum, we draw attention to this unusual variant of SMX, which should be suspected in a setting of multiple “angiofibromas/fibrous papules” on the face with presence of mucin.