儿童水疱型毛母质瘤16例临床病理分析

目的 分析儿童水疱型毛母质瘤的临床病理特点。方法 对2013—2017年就诊于北京儿童医院皮肤科的16例水疱型毛母质瘤患者进行临床病理分析。结果 16例患者中,男5例,女11例。发病年龄4个月至11岁,中位发病年龄8.5岁。病程2个月至4年,平均病程10个月。发病部位：上肢10例（上臂7例、肩部2例、前臂1例）,面部4例,颈部2例。皮损表现为局限性可推动的红色肿物,呈水疱样外观,部分皮损表面可见毛细血管扩张,肿物直径0.5 ~ 3 cm,触诊水疱内可及质硬结节。皮肤镜下,16例患者皮损见红色均匀背景,13例见白色无结构区,4例见蓝灰色无结构区,11例见线状或树枝状不规则血管,15例患者同时有多种皮肤镜表现。皮损均予手术切除,随访1 ~ 5年无复发。组织病理：肿瘤位于真皮中下部,主要由基底样细胞及影细胞组成,还可见介于上述两种细胞之间的过渡细胞。肿瘤间质中见不同程度炎症细胞浸润及纤维结缔组织增生和钙化,部分可见多核巨细胞。表皮和瘤体之间真皮内见不同程度的炎症细胞浸润、淋巴管扩张及弹性纤维减少或缺失。结论 儿童水疱型毛母质瘤好发于上肢及面颈部,组织病理特点为肿瘤由基底样细胞及影细胞构成,真皮内淋巴管扩张、弹性纤维减少,皮肤镜下以红色背景、白色无结构区为特征。     

Circulating Eosinophil and Neutrophil Counts Correlate with Disease Severity in Bullous Pemphigoid

BackgroundBullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by tissue-bound and circulating autoantibodies directed against BP180 and/or BP230 antigens. Various inflammatory cells are involved in the development of blister in BP.ObjectiveThe aim of this study was to evaluate the correlation between peripheral leukocyte counts and BP severity.MethodsWe retrospectively included 60 patients with BP, who had not been treated with systemic steroid at the time of blood sampling. The patients were classified into two groups, those with admission history (admission group) and those without admission history (non-admission group). Disease severity was evaluated using three parameters: admission history, initial steroid dosage, and modified version of a pemphigus scoring system. We evaluated the correlation between peripheral leukocyte counts and disease severity measured by the three parameters.ResultsThe admission group showed a significant increase in disease severity measured by initial steroid dosage and severity score compared with the non-admission group. Additionally, the admission group had increased total leukocyte, eosinophil, and neutrophil counts. In the correlation study, the peripheral eosinophil and neutrophil counts showed positive correlation with BP severity evaluated by both initial steroid dosage and the pemphigus scoring system.ConclusionPeripheral eosinophil and neutrophil counts can be used as a marker in predicting disease severity in patients with BP.     

A case of atezolizumab‐induced photodistributed bullous pemphigoid

Immunotherapy has revolutionized cancer therapy in recent years but is associated with unique immunologically mediated adverse effects. Immunotherapy‐induced bullous pemphigoid (BP) is an uncommon but established reaction that portends significant management implications as in most instances systemic treatment is required. We report a case of immunotherapy‐associated BP in a marked photodistribution, highlighting the diverse clinical presentations of this eruption.     

Mycophenolic acid in dermatology a century after its discovery

Mycophenolic acid was first discovered in 1913 and first used clinically in the 1970s as an immunosuppressant to prevent organ transplantation rejection. It was later used in the treatment of psoriasis. However due to its side‐effect profile and fears over its carcinogenic potential it was abandoned. From the late 1990s a prodrug, mycophenolate mofetil (MMF), was developed and more recently, enteric‐coated mycophenolate sodium (EC‐MPS), both of which have gained increasing use in the field of dermatology for a variety of skin conditions. This review discusses the pharmacology, mechanisms of action, side‐effects and current clinical applications in dermatology of MMF and EC‐MPS.     

Lichen Planus Pemphigoides Presenting Preferentially Over Preexisting Scars: A Rare Instance of Isotopic Phenomenon

An 18-year-old girl presented with multiple itchy hyperpigmented papules and plaques, along with tense blisters over the lower limbs and buttocks for last 3 months. These papules, plaques, and bullae were mostly localized to preexisting scars. The histopathological findings from papule and bulla were consistent with lichen planus (LP) and bullous pemphigoid, respectively. Direct immunofluorescence (DIF) of perilesional skin around bulla showed linear deposition of IgG and C3. Considering clinical, histopathological and DIF findings, diagnosis of LP pemphigoides (LPP) was made. The preferential localization of LPP lesions over preexisting scars was a very interesting finding in our case an extremely rare instance of the isotopic phenomenon.