A case of dermatomyositis associated with mechanic's hand

A 67-year-old man was referred to the Department of Internal Medicine at Tokyo Medical University with interstitial pneumonia in July 1999. He presented with keratotic plaques on both palsm and on the ventral and lateral sides of his fingers. Erythematous keratosis was observed on the dosal aspect of his fingers and metatarsophalangeal (MP) joints. Edematous erythema was seen on the patient's chest, back, and the extensor surfaces of his arms. Electromyography revealed a myogenic pattern and an increased level of myogenic enzymes was found in the blood. Histological findings of the ventral sides of his fingers showed hyperkeratosis and parakeratosis of the dermal tissue and liquefaction degeneration of the basal layer at the papilla. Based on these findings, the patient was given a diagnosis of dermatomyositis associated with mechanic's hand. A systemic examination confirmed interstitial pneumonia and carcinoma of the duodenal papilla. Mechanic's hand is a type of dermatitis associated with myopathy first reported by Stahl et al. in patients with collagen disease. We report herein the first documented case of mechanic's hand in Asians.     

Clinicopathological analysis of mechanic's hand associated with dermatomyositis

Mechanic's hand is often seen in the fingers of patients with dermatomyositis and is frequently associated with anti‐aminoacyl‐transfer RNA synthetase autoantibodies and interstitial lung disease. We analysed the clinical symptoms of 50 patients with dermatomyositis who had visited our department, 26 of whom also had mechanic's hand. A histological examination was carried out in 16 of the 26 cases, which revealed hyperkeratosis in all cases and colloid bodies in the epidermis in 15 cases. The number of cases of interstitial lung disease in patients with mechanic's hand (22/26, 85%) was significantly higher than that in those without mechanic's hand (12/24, 50%) (P < 0.05). Mechanic's hand is an important skin lesion of dermatomyositis, and increases the likelihood of interstitial lung disease     

Fulminant dermatomyositis after removal of a cancer.

Dermatomyositis developed suddenly in a diabetic patient with CREST syndrome after the removal of a malignant tumor. Scrupulous physical examination excluded further systemic or cutaneous involvement. We raise certain still unsolved aspects regarding the association between dermatomyositis and neoplastic disorders.     

Plasma homocysteine levels are positively associated with interstitial lung disease in dermatomyositis patients with anti‐aminoacyl‐tRNA synthetase antibody

Homocysteine is a sulfhydryl‐containing amino acid that is derived from dietary methionine, and there has been increasing evidence that elevated plasma homocysteine levels are associated with increased risk of central and peripheral vascular disorders, including carotid, coronary and peripheral arterial diseases, and Raynaud’s phenomenon. Recently, associations of plasma homocysteine levels with autoimmune diseases such as systemic lupus erythematodes and systemic sclerosis have been reported. However, no study analyzed the association between plasma homocysteine levels and dermatomyositis (DM). The objective of this study was to examine plasma homocysteine levels and their clinical associations in patients with DM. Plasma homocysteine levels in 28 Japanese patients with DM and 22 healthy controls were examined. We found that the plasma homocysteine levels in DM patients were significantly higher than those in healthy individuals (15.8 ± 1.1 vs 8.5 ± 0.5 µmol/L, P < 0.01). Presence of mechanic’s hand, complication of interstitial lung disease (ILD), high serum Krebs von den Lungen‐6 (KL‐6), surfactant protein‐D and creatine kinase levels, and anti‐aminoacyl‐tRNA synthetase (ARS) antibody (Ab) positivity were significantly more prevalent among DM patients with elevated plasma homocysteine levels. The plasma homocysteine levels in DM patients with mechanic’s hand, ILD and anti‐ARS Ab were significantly higher than those in DM without those features. Furthermore, the plasma homocysteine levels were positively correlated with serum KL‐6 levels. These results suggest that the pathogenesis of elevated plasma homocysteine levels may be associated with ILD in DM patients, especially with anti‐ARS Ab, and further examination is required.     

Cutaneous involvement in Hodgkin's lymphoma: report of two cases

Hodgkin's lymphoma (HL) comprises 20 to 30% of all lymphomas. Skin involvement is almost always secondary to visceral or nodal involvement. Secondary cutaneous HL is rare, occurring in only 0.5 to 3.4% of the cases. Herein we report two cases of skin involvement in Hodgkin's disease. One was a 25-year-old female admitted with a draining sinus in the neck. The other was a 19-year-old female admitted with a neck mass and skin papules on her chest and arm. Skin involvement may antecede or can be seen during the course of HL. Better understanding of such cutaneous involvement, which occasionally may be the initial sign of HL, is needed, and a biopsy of any suspicious skin lesion should be considered.     

Unexpected recalcitrant course of drug‐induced erythema multiforme‐like eruption and interstitial pneumonia sequentially occurring after nivolumab therapy

Vemurafenib improves survival of melanoma patients. However, cutaneous side‐effects commonly occur in them. Nivolumab and ipilimumab are monoclonal antibodies against programmed death 1 and cytotoxic T‐lymphocyte‐associated antigen 4, both of which regulate excessive T‐cell activation. Although these agents induce antitumor immunity against melanoma, the modified immune condition may result in an unexpected adverse reaction which has not been observed previously. Herein, we report a case who manifested severe erythema multiforme‐like eruption with mucosal involvement associated with vemurafenib following nivolumab. The patient also subsequently suffered from ipilimumab‐induced interstitial pneumonia with refractory course. Such a case has never been reported. This case suggested that dermatologists should pay special attention to unexpected adverse events of these drugs, and carefully observe cutaneous and respiratory status of patients during the treatment of melanoma.     

Clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia: The relation between the disease activity and the serum interleukin‐6 level

A 67‐year‐old woman exhibited chilblain‐like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions. Fine crackles were audible on the dorsal side of the bilateral lower lung regions. We diagnosed clinically amyopathic dermatomyositis accompanied by rapidly progressive interstitial pneumonia. While we initiated immunosuppressive therapy, the respiratory failure showed no responses. We performed endotoxin adsorption therapy using polymyxin B‐immobilized fiber column direct hemoperfusion (PMX‐DHP). After the first PMX‐DHP session, the respiratory condition was slightly improved but it soon deteriorated. At the second PMX‐DHP session, subcutaneous and mediastinal emphysema was noted. The patient died, showing no improvement in the respiratory condition. We measured the levels of serum interleukin (IL)‐6, tumor necrosis factor (TNF)‐α and IL‐10 before and after PMX‐DHP and compared them with previously reported levels. The levels of TNF‐α and IL‐10 showed no or slight changes, but those of IL‐6 at the beginning of the treatment were higher compared with previous reports. After the first PMX‐DHP, the IL‐6 level decreased with slight improvement in respiratory condition. However, after the second PMX‐DHP, it increased markedly with exacerbation of the respiratory failure. The serum IL‐6 level is considered to be effective for the evaluation of the disease activity and prognosis under the treatment by PMX‐DHP.     

合并肺间质病变或恶性肿瘤皮肌炎患者的外周血差异基因表达分析

【摘要】 目的 研究合并肺间质病变或恶性肿瘤的皮肌炎/临床无肌病性皮肌炎（DM/CADM）患者的差异表达基因及相关信号通路。方法 2017年1月至2018年1月于上海交通大学医学院附属瑞金医院皮肤科确诊的DM/CADM患者27例,按照合并症状分为3组,即合并肺间质病变组10例,合并恶性肿瘤组8例,无肺间质病变和恶性肿瘤组9例。同时收集7例健康对照。采用高通量RNA测序技术筛选上述4组受试者外周血中差异表达基因,进行基因本体论（GO）分析以及京都基因与基因组百科全书（KEGG）通路富集分析。结果 与健康对照相比,DM/CADM患者4 820条基因表达上调,137条基因表达下调;GO分析获得显著富集条目49个,其中37个（75.5%）与生物过程相关;KEGG分析差异基因富集于感染、肿瘤以及免疫相关通路。与无肺间质病变和恶性肿瘤组相比,合并肺间质病变组272条基因表达上调,158条基因下调;GO分析获得显著富集条目157个,其中114个（72.6%）与生物过程相关;KEGG分析差异基因富集于细菌感染和自身免疫/炎症通路。合并恶性肿瘤组398条基因表达上调,68条基因表达下调;GO分析获得显著富集条目117个,其中94个（80.3%）与生物过程相关;KEGG分析差异基因富集于糖基化、代谢以及肿瘤相关信号通路。结论 DM/CADM患者与健康对照组间、合并肺间质病变或恶性肿瘤的DM/CADM组与无合并症患者组间转录组基因及通路存在差异,细菌感染、细胞因子/趋化因子通路在合并ILD的DM/CADM患者中显著富集,而糖基化、蛋白代谢以及抗原提呈和自然杀伤细胞的细胞毒作用在合并恶性肿瘤的DM/CADM患者中显著富集。     

Three cases of pigmented cosmetic dermatitis‐like eruptions associated with primary Sjögren's syndrome or anti‐SSA antibody

Pigmented cosmetic dermatitis‐like (Riehl's melanosis‐like) pigmentation was reported in three of 27 patients with primary Sjögren's syndrome. But case reports of such eruptions are rare. We describe three cases of such eruptions associated with primary Sjögren's syndrome or anti‐SSA antibody and possible associations with specific types of human leukocyte antigen (HLA) and infiltrating lymphocytes. These middle‐aged Japanese women had reticular facial pigmentation and histopathological examination revealed interface dermatitis, melanophages, and dense lymphocytic infiltration around hair follicles and sweat ducts. HLA typing revealed common antigenic equivalents or genetic typing of HLA‐A2, DR52, DPA1(02:02) and DPB1(05:01). Immunohistochemical staining revealed major subsets of T cells to be CD8 and CD45RO. Some Foxp3‐ and few IL17‐positive cells were found in strong contrast to the major CD4 subset of infiltrated T cells in annular erythema associated with Sjögren's syndrome. Apparently, our patients' pigmentation represented a specific etiology associated with primary Sjögren's syndrome or anti‐SSA antibody.     

Benign hypergammaglobulinemia purpura of Waldenström associated with lymphoid interstitial pneumonitis

A 31-year-old female is described who developed benign hypergammaglobulinaemic purpura and lymphoid interstitial pneumonitis concomitantly. High titre anti-nuclear antibodies were also noted. Several years previously, the patient had developed myasthenia gravis and multiple sclerosis. The present case is an example of multiple medical disorders characterized by immune dysregulation and represents the first reported association of hypergammaglobulinaemic purpura with lymphoid interstitial pneumonitis.     

老年人社区获得性肺炎的风险因素：111例分析

目的探索影响老年人社区获得性肺炎（CAP）预后的风险因素。方法使用Logistic回归模型分析111例CAP患者,男68例,女43例,（78．1±7．9）（65～98）岁,病死率的风险因素。结果病死率为25．2％单因素分析发现卧床、合并基础疾病的数量、合并神经系统疾病、意识改变、血白蛋白〈3．0g／dl、血D-二聚体升高、入住ICU、休克、肾功能衰竭、机械通气、APACHEⅡ评分和PSI评分较高均为影响病死率的风险因素,而卧床、休克、APACHEⅡ评分较高为其独立风险因素。结论老年人CAP的病死率较高,影响老年人CAP病死率的风险因素甚多。     

A case of classic Kaposi's sarcoma with multiple organ involvement

Kaposi's sarcoma is a systemic, multifocal, angiomatous tumor which can be classified into four clinical variants. Among them, classic Kaposi's sarcoma is rare in Oriental people, and the involvement of internal organs in this variant is reported to be very rare. We present a case of classic Kaposi's sarcoma in a 67-year-old Korean male, who had multiple organ involvement in the skin, lymph node, and stomach. Lesions of Kaposi's sarcoma cleared after treatment with etoposide, but the skin lesions recurred after a three-month symptom-free period. He was treated with doxorubicin, bleomycin, and vincristine, and all the skin lesions were cleared.     

Clinical value of 18F‐fluorodeoxyglucose positron emission tomography/computed tomography for interstitial lung disease and myositis in patients with dermatomyositis

¹⁸F‐Fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) is usually used to screen malignancy in patients with dermatomyositis (DM). Additionally, it is well known that FDG‐PET/CT provides valuable information for evaluating the activity of several inflammatory diseases, such as sarcoidosis, atherosclerosis, inflammatory bowel disease and rheumatoid arthritis. Therefore, the objective of this study was to evaluate the clinical usefulness of FDG‐PET/CT for the detection of inflammatory lesions and disease activity of both myopathy and interstitial lung disease (ILD) in DM patients. We measured the maximum standardized uptake value (SUVmax) in the muscles and lungs in 22 DM patients, and compared with magnetic resonance imaging (MRI) and high‐resolution computed tomography (HRCT) findings in the same muscle and lung regions as well as with clinical findings. We found that the location of increased FDG uptake was nearly consistent with the region of ILD and myositis detected by HRCT or MRI, respectively. There was a significant positive correlation between lung HRCT score and SUVmax in each lung. Serum Krebs von den Lungen‐6 levels also revealed significant positive correlation with total SUVmax of right and left lungs. Regarding FDG‐PET/CT and myopathy, total SUVmax in the muscles was significantly correlated with serum cytokeratin levels. Our results suggest that FDG uptake (SUVmax) might be useful for not only the detection of malignant tumors, but also the evaluation of the location and activity of ILD and myositis in DM patients.     

A case of Werner's syndrome associated with osteosarcoma.

We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.