Paraneoplastic erythroderma: an insight on the existing data

Exfoliative erythroderma is a severe dermatologic condition with variable underlying causes. The association of erythroderma with an occult visceral malignancy is a relatively uncommon finding, which, however, can be accompanied by devastating consequences. This report is the first updated overview regarding the latest data on paraneoplastic erythroderma and was conducted according to the PRISMA Guidelines for systematic reviews. Articles derived from the databases PubMed, EMBASE, and SCOPUS were analyzed for this study. Patients with paraneoplastic erythroderma share certain clinical characteristics that physicians should be aware of, so as to avoid potential sequeale of an erroneous management as a result of misdiagnosis. The reporting of such cases is crucial in order to raise awareness of this condition and to facilitate an earlier diagnosis.     

副肿瘤性天疱疮1例

报告1例副肿瘤性天疱疮。患者男,50岁。反复口腔黏膜糜烂,全身有皮损、瘙痒,伴淋巴结增大1个月余。依据病史、组织病理和免疫病理改变,确诊为副肿瘤性天疱疮伴慢性淋巴细胞白血病。     

副肿瘤性天疱疮1例

副肿瘤性天疱疮（paraneoplastic pemphigns,PNP）是一种与肿瘤有关的自身免疫性皮肤病,国内近几年陆续有该病的个例报告,现将笔者诊治的1例PNP报告如下。     

副肿瘤性天疱疮1例

报告1例副肿瘤性天疱疮。患者男,31岁。因口腔黏膜疼痛性、难治性糜烂6个月,全身皮肤红斑、丘疹,伴脱发1个月就诊。体格检查:头部呈虫蚀状及斑片状脱发,口唇及外阴黏膜弥漫性肿胀、糜烂,全身皮肤散在多形性红斑、丘疹,偶见水疱,手、足部可见点状或片状水肿性红斑伴鳞屑。增强CT检查示右肾上腺区肿物,考虑Castleman瘤。外科手术切除一9cm×6cm大肿瘤,组织病理检查示透明血管型巨大淋巴结增生,伴局灶钙化;免疫组化CD3(+)、CD20(+)、CD21(+)、CD68(+)。诊断为副肿瘤性天疱疮。术后应用糖皮质激素治疗皮损好转。     

副肿瘤性天疱疮1例

报告1例副肿瘤性天疱疮。患者女性，48岁，因多形皮肤粘膜损害1月伴发非霍奇金淋巴瘤2年余入院。皮肤病理活检示棘刺松解性大疱，直接免疫荧光棘细胞间及基底膜区免疫反应物沉积。其免疫组化示T细胞侵入表皮现象。     

Omenn syndrome: a rare case of neonatal erythroderma

Omenn syndrome is a severe combined immunodeficiency characterized by erythroderma, hepatosplenomegaly, lymphadenopathy and failure to thrive, with activated oligoclonal T lymphocytes and an absence of circulating B cells.A 3 day-old boy presented with a congenital erythroderma. Investigations revealed a marked neutropenia and lymphopenia and the absence of a thymus. Genetic studies showed RAG 1 mutations. He was successfully treated with an HLA identical bone marrow transplantation. Omenn syndrome is a rare severe combined immunodeficiency. Most cases are due to mutations in the RAG genes with autosomal recessive transmission. Our observation is original because of an incomplete clinical presentation. During the course of the disease, the child had no failure to thrive, no organomegaly and no recurrent infection. Immunodeficiency must be excluded in every case of neonatal erythroderma and an immunological assessment should be performed without delay.     

挪威疥误诊为红皮病一例

患者,女,75岁。周身红斑、丘疹6个月伴糜烂、渗出4天,曾于当地诊所予以“西替利嗪片、糠酸模米松乳膏”治疗,症状无好转,皮疹逐渐泛发,我院门诊以“红皮病并皮肤感染”收入院。入院后第二天追问病史,既往曾有周身剧烈瘙痒,以腹部、指缝等部位为著,夜间加重,其家人有典型疥疮样皮损,为患者完善直接镜检见多个疥虫及虫卵。     

表现为红皮病的挪威疥一例

患者,女,53岁。双上肢红斑、丘疹伴瘙痒1年,泛发全身3个月,曾误诊为"过敏",予抗组胺药、糖皮质激素软膏治疗,皮损未见明显好转。皮肤活检及显微镜镜检发现疥螨,给予氧化锌硫软膏外用。     

Tubular apocrine adenoma: apropos of a case

Tubular apocrine adenoma is a rare, benign apocrine tumour of the skin, histologically distinct from other epithelial proliferations, showing apocrine differentiation. A new case of this uncommon lesion is reported herein and a review of the literature concerning this tumour is made.     

Keratosis lichenoides chronica: report of a case developing after erythroderma

A 66-year-old male presented with keratosis lichenoides chronica after a presumed drug-induced erythroderma. After resolution of the erythroderma, slightly scaly erythematous and violaceous papules in a reticular arrangement over the trunk and limbs developed in association with hoarseness, palmoplantar keratoderma, onycholysis and subungual keratosis. Histology from a lichenoid lesion showed pseudo-epitheliomatous hyperplasia, hyperorthokeratosis, parakeratosis, dyskeratosis, neutrophil exocytosis and focal vacuolar degeneration of the basal layer of the epidermis. There was a band-like chronic inflammatory infiltrate in the upper dermis. The skin improved with prednisone 40 mg/day for 15 days, leaving atrophic hypopigmented scars. A diagnosis of keratosis lichenoides chronica was made.     

副肿瘤性天疱疮1例

报告1例副肿瘤性天疱疮,男性,21岁。临床特点:严重疼痛性粘膜糜烂和多形性皮损。组织病理学显示棘层松解、苔藓样皮炎或角质形成细胞坏死。B超和CT示下腹部偏右侧有一11.2cm×7.6cm×8.4cm包块。术后病理证实为非何杰金淋巴瘤。     

以红皮病为首发症状的艾滋病一例

患者,男,40岁。全身皮肤潮红、脱屑伴瘙痒1个月。患者既往无湿疹、银屑病病史。查体：全身弥漫性潮红,表面覆盖黄白色细碎鳞屑,口周、眼周可见裂隙伴渗出,双足背覆盖袜套样黄色、褐色样鳞屑,可见痂样结构。HIV抗体初筛及确诊试验阳性。诊断为艾滋病、红皮病。     

伴有隆突性皮肤纤维肉瘤的副肿瘤性天疱疮1例报告

目的：报告1例伴有隆突性皮肤纤维肉瘤的副肿瘤性天疱疮。方法：对副肿瘤性天疱疮并隆突性皮肤纤维肉瘤病例的临床表现进行表述，并检查了组织病理和免疫病理。结果：经临床和实验室检查证实，为副肿瘤性天疱疮伴发隆突性皮肤纤维肉瘤。结论：副肿瘤性天疱疮不但与内脏肿瘤有关，也可伴发皮肤肿瘤。     

Ductal eccrine carcinoma with squamous differentiation: apropos a case

Sweat gland carcinomas are rare. Given this, they can pose a diagnostic challenge especially in shave biopsy specimens. We present a case of ductal eccrine carcinoma with extensive squamoid differentiation that was repeatedly misdiagnosed by multiple dermatopathologists as squamous cell carcinoma in the initial few biopsies. As the distinction between these two neoplasms is crucial to patient management, we highlight the histologic features of this uncommon entity to highlight the potential diagnostic pitfalls.     

Paraneoplastic pemphigus associated with pelvic inflammatory fibrosarcoma: a case report

A 36-year-old African-American woman presented with an extensive stomatitis and pigmented cutaneous macules on the neck, axillae and hands. Subsequently she developed violaceus papules on the dorsa of the hands, histologically consistent with an interface dermatitis. After 18 months of progressive disease, paraneoplastic pemphigus was suspected and a search for an underlying neoplasm was initiated. An exploratory laparotomy revealed a pelvic mass and the histologic examination showed an inflammatory fibrosarcoma. The evidence of acantholysis on new cutaneous lesions and the positivity of indirect immunofluorescence with rodent urinary bladder epithelium reinforced the diagnostic criteria for paraneoplastic pemphigus, which is confirmed by the identification of strong protein bands at 210, 190 and 170 kd by immunoprecipitation. Paraneoplastic pemphigus should be considered when investigating atypical mucocutaneous manifestations of pemphigus vulgaris and lichen planus. Diagnostic screening for paraneoplastic pemphigus and a search for an underlying tumor should be performed.