皮肌炎样皮炎还是无肌病性皮肌炎?——关于无肌病性皮肌炎命名的商榷

Pearson首次用“无肌病性皮肌炎(ADM)”来命名有皮肌炎的典型皮损而无肌肉损害的患者。然而大家对这一疾病的命名一直存在争议。复习大量文献后,笔者提出“皮肌炎样皮炎”这样的命名,并认为它是一种复杂的综合群,可以是皮肌炎的早期改变,也可以伴有严重的肺部损害或疾病或向红斑狼疮转归,让人忽略的是这样的皮炎还可以合并恶性肿瘤,是“副肿瘤综合征”的一种表现。对这类患者要加强随访,定期作肿瘤学检查和胸部放射学检查。     

无肌病性皮肌炎16例临床分析

目的：探讨无肌病性皮肌炎（ADM）的命名。方法：回顾性分析符合Euwer提出的ADM诊断标准的16例患者的临床资料。结果：16例患者中2例进展为皮肌炎,1例进展为慢性皮肤型红斑狼疮（CCLE）。间质性肺炎（ILD）发生率为68．8％,恶性肿瘤发生率为25．0％。结论：提出皮肌炎样皮炎的命名,并认为它是一种复杂的综合征,可以是皮肌炎的早期表现,也可以伴有严重的肺部损害或疾病向红斑狼疮转归,皮肌炎样皮炎可并发恶性肿瘤,因此需要行全身系统检查,并加强随访。     

无肌病性皮肌炎--附1例报告

患者女性，５４岁，两上眼睑紫红色水肿性红斑３年，未出现肌肉损害的临床表现，与肌肉损害有关的实验室检查及肌电图、肌活检均无异常发现。ＪＯ－１抗体弱阳性，ＡＮＡ滴度１：１６０，皮损的组织病理变化及直接免疫荧光检查结果符合皮肌炎诊断。     

儿童无肌病性皮肌炎1例

报告1例具有典型的皮肌炎皮疹而无肌肉受累的儿童无肌病性皮肌炎患者。结合文献对本病的临床表现、诊断及治疗进行讨论。本病的诊断需临床、病理与实验室检查相结合。     

儿童皮肌炎28例临床分析

目的：根据28例儿童皮肌炎的临床治疗及转归,探讨儿童皮肌炎的诊断和治疗方法。方法：回顾性分析28例儿童皮肌炎患儿的一般资料、临床表现、实验室检查、治疗及转归。结果：28例儿童皮肌炎患儿均有皮肤损害和肌酶升高,24例患儿有程度不同的肌肉症状。28例患儿均采用糖皮质激素治疗,23例患儿联合应用大剂量静脉注射用人免疫球蛋白治疗。除1例患儿因病情危重转入儿科医院治疗外,其余27例患儿均病情稳定后出院,出院后仍使用糖皮质激素维持治疗。结论：糖皮质激素目前仍是治疗儿童皮肌炎的首选药物,同时联合应用大剂量静脉注射用人免疫球蛋白治疗,可以减少糖皮质激素的用量,能够短期内控制肌肉症状、改善皮损、减少复发。     

98例皮肌炎患者临床分析

<正>皮肌炎(DM)是一种自身免疫性结缔组织病,该病典型症状是肌肉炎症和皮肤损害,可累及多系统病变,如肺部、关节及心脏等,部分患者还可伴发恶性肿瘤。现将我科近年收治的98例皮肌炎患者的临床资料进行回顾性分析,旨在探讨皮肌炎的首发症状、临床表现、实验室检查等特点,分析易误诊的原因和合     

皮肌炎合并间质性肺疾病患者的临床特点

间质性肺疾病(interstitial lungdisease,ILD)是皮肌炎的常见并发症^[1],并发率高达5%-46%^[2].合并ILD的皮肌炎患者预后差,治疗困难,死亡率高.部分患者肺部损害先于皮肤、肌肉症状出现,容易造成误诊,因此应重视皮肌炎的肺部损害.我们分析了27例皮肌炎-ILD患者的临床特点和肺部表现,以提高对ILD的认识.     

关于无肌病性皮肌炎命名的商榷

拜读了曹华等“皮肌炎样皮炎还是无肌病性皮肌炎？——关于无肌病性皮肌炎命名的商榷”一文，又读到编者按“欢迎广大读者对此展开讨论”，故谈几个观点与作者及读者商榷。     

儿童皮肌炎27例临床特点分析

目的探讨儿童皮肌炎的临床特点。方法对27例儿童皮肌炎的临床表现及实验室检查结果进行回顾性分析。结果本组JDM中,男女比例为1:1.7,首发症状以皮疹为主,儿童患者的面部水肿性紫红斑阳性率显著高于成人患者,发热、关节痛、心脏受累、吞咽困难的阳性率显著低于成人患者,未见间质性肺炎及恶性肿瘤。结论儿童皮肌炎以女性多见,皮肤损害为首发症状,系统损害轻,且不伴发恶性肿瘤,预后较好。     

无肌炎性皮肌炎1例

皮肌炎是临床常见的结缔组织病，主要以皮炎和肌肉炎症与变性为特征。临床上可分为6型，而无肌炎性皮肌炎（amyoputhie dermatomyositis，ADM）是一种仅有皮肤特征性损害而至少在1年以上无肌肉病变的特殊类型的皮肌炎，临床上较为罕见，易于误诊。我科于2003年10月诊治1例，现报道如下。     

临床无肌病性皮肌炎预后相关特征研究进展

临床无肌病性皮肌炎是皮肌炎的特殊亚型,表现为典型皮肌炎的皮肤病变,但缺乏特征性肌病。与经典皮肌炎相比,临床无肌病性皮肌炎在肌炎特异性自身抗体谱和间质性肺病、恶性肿瘤方面具有其独特预后特征,本文对其进行综述。     

临床无肌病性皮肌炎的诊断及治疗

临床无肌病性皮肌炎被认为是皮肌炎的一种少见类型,是指具有皮肌炎的典型皮损,临床上不表现为肌无力,肌酶谱正常或仅轻度异常,分为无肌病性皮肌炎和低肌病性皮肌炎。部分患者与严重的肺部病变及恶性肿瘤相关,而部分严重患者对糖皮质激素治疗不敏感,故早期发现和正确认识该病并给予合理的干预,对改善患者预后非常重要。     

Amyopathic dermatomyositis: retrospective review of 37 cases

Criteria for diagnosis of amyopathic dermatomyositis vary, and the prognosis is not clear. Our purpose was to investigate prognosis regarding progression to myositis and associated malignancy. We reviewed the medical records of patients with dermatomyositis evaluated at our institution from 1976 to 1994. Of 746 patients with dermatomyositis, 37 (5%) with the amyopathic subtype were divided into 3 groups: group 1 (73%), no subjective or objective evidence of myopathy; group 2 (13%), no subjective muscle weakness but abnormalities detected by objective tests; group 3 (13%), subjective muscle weakness but no objective evidence of myopathy. Follow-up was conducted by means of a mailed questionnaire. For 25 patients, follow-up of 1 to 17 years after diagnosis showed muscle weakness in 2 patients in group 1 within 5 years after diagnosis. Five patients (13%) had associated malignancies. Of 7 (19%) patients with disease onset before the age of 18 years, none had progression to myopathy. Although it presents with cutaneous lesions indistinguishable from those of classic dermatomyositis, amyopathic dermatomyositis is a distinct entity. In most patients, amyopathic dermatomyositis does not progress to myopathy. Prognosis appears favorable, but malignancy may develop.     

Amyopathic Dermatomyositis: A Concise Review of Clinical Manifestations and Associated Malignancies

Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron’s sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. A literature review of amyopathic dermatomyositis was performed using the PubMed medical database. The key features of amyopathic dermatomyositis, including autoantibodies, clinical presentation and dermatologic manifestations, epidemiology, history, associated malignancies, management, and pathogenesis, are summarized in this review. Cancer (solid tumor) (73/79, 89 %) and hematologic malignancies (9/79, 11 %) were reported in 79 patients, with three patients having more than one malignancy. In addition, there were six patients with amyopathic dermatomyositis who had tumor of unknown primary, and eight patients with cancer-associated amyopathic dermatomyositis for whom no additional details were provided. From the group of 73 tumors for whom primary origin and sex were available, malignancy of the genitourinary organs (24/73, 33 %), aerorespiratory organs (15/73, 21 %), and breast (14/73, 19 %) were the most commonly observed solid organ tumors. Tumors of the genitourinary organs (15/48, 31 %) and breast (14/48, 29 %) were the most frequent neoplasms in women, accounting for 29 of 48 (60 %) cancers, with the most common sites being breast (14/48, 29 %), ovary (8/48, 17 %), and cervix or uterus (5/48, 10 %). In men, tumors of the aerorespiratory (9/25, 36 %) and genitourinary (9/25, 36 %) tracts were the most common neoplasms, accounting for 72 % (18/25) of cancers; the most common sites of primary malignancy were nasopharyngeal (6/25, 24 %), bladder (4/25, 16 %), and either colorectal, lung or prostate cancer (three cancers each, 12 %). In summary, the search for an undiagnosed associated malignancy in patients with amyopathic dermatomyositis should focus towards the organs most frequently affected. Similar to classic dermatomyositis, ovarian and nasopharyngeal cancers are also common in amyopathic dermatomyositis. However, in contrast to lung cancer, which is the most frequent malignancy associated with classic dermatomyositis, breast cancer was the most common type of malignancy reported in patients with amyopathic dermatomyosotis.     

Amyopathic dermatomyositis associated with a recurrence of breast cancer

The term "amyopathic dermatomyositis", or dermatomyositis "sine myositis" is used to describe those patients who present with the skin manifestations typical of dermatomyositis, but with no evidence of inflammatory myopathy. Amyopathic dermatomyositis may be associated with an underlying neoplasm, the same as with classic dermatomyositis. We present the case of a 59-year-old female patient, with cutaneous findings typical of dermatomyositis, with no proximal muscle weakness and with normal serum muscle enzymes, which stayed in a normal range throughout the later follow-up period, although the electromyogram performed six months later showed alterations with a myopathic pattern. These skin symptoms raised the suspicion of an occult neoplasm, and a recurrence of the patient's breast cancer, apparently inactive for many years, was finally found. The association of amyopathic dermatomyositis with a recurrence of breast cancer is exceptional.     

Prevalence and reactivity of anti-melanoma differentiation-associated gene 5 (anti-MDA-5) autoantibody in Brazilian patients with dermatomyositis

Background:There have been no studies to date on the frequency and reactivity of aanti-melanoma differentiation-associated gene 5 (anti-MDA-5) in samples from the Brazilian population with dermatomyositis.Objectives.To analyze this autoantibody in the Brazilian population.Methods:This was a single-center cross-sectional study in which 131 consecutive adult patients (109 dermatomyositis and 22 clinically amyopathic dermatomyositis) with active disease were evaluated from 2000 to 2016. Analysis of the anti-MDA-5 autoantibody was performed by ELISA.Results:The presence of this autoantibody was observed in 14.7% and 22.7% of patients with dermatomyositis and clinically amyopathic dermatomyositis, respectively. In the case of dermatomyositis, the autoantibody was associated less frequently with Raynaud’s phenomenon and periungual hyperemia (P<0.05). In clinically amyopathic dermatomyositis, the presence of this autoantibody was not associated statistically with any demographic, clinical, laboratory, or imaging characteristics.Study limitations:The cross-sectional study design did not allow establishing a temporal correlation between anti-MDA-5 autoantibody and various study variables. In addition, pulmonary function tests were not performed in the patients.Conclusions.The frequency of anti-MDA-5 autoantibody was comparable to that of other populations with dermatomyositis, but with a different reactivity than described in the literature. In addition, there was a phenotypic variability between our patients with clinically amyopathic dermatomyositis and those described in the literature. Further studies are needed to confirm the current study’s findings and elucidate this autoantibody’s reactivity in Brazilians with idiopathic inflammatory myopathies.     

Regression of primary melanoma with metastases associated with amyopathic dermatomyositis

BACKGROUND: Dermatomyositis is a rare and serious inflammatory connective tissue disease characterized by a typical cutaneous rash and myopathy. Amyopathic dermatomyositis is a particular form of dermatomyositis involving only cutaneous signs and without myopathy present for over 2 years. PATIENTS AND METHODS: A 48 year-old woman presented with a 3-year history of cutaneous rash without myopathy characteristic of amyopathic dermatomyositis. Clinical examination revealed extensive axillary adenopathy, histological examination of which suggested secondary melanoma. The patient reported a black nevus in the axillary area that had disappeared 1 year earlier. Curettage of the lymph node was negative and the patient was treated with interferon (3M 3 times a week). Regression of the cutaneous signs was noted. DISCUSSION: The data, there have been no other reports of paraneoplastic amyopathic dermatomyositis associated with regression of primary melanoma. The literature contains few reports of dermatomyositis associated with melanoma. Amyopathic dermatomyositis may be associated with malignancy.     

Panniculitis associated with amyopathic dermatomyositis

Panniculitis is a rare clinical finding in dermatomyositis, with less than 30 cases reported and there is only one case associated with the amyopathic subtype described in the literature. The present report describes a 49-year-old female patient that one year after being diagnosed with amyopathic dermatomyositis, presented indurated, painful, erythematous to violaceous nodules located on the upper limbs, thighs and gluteal region. Skin biopsy revealed lobular panniculitis with a lymphocytic infiltrate. The patient was treated with prednisone and methotrexate, but remained unresponsive to treatment.     

Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.

OBJECTIVE: To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen patients with amyopathic dermatomyositis. RESULTS: The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. CONCLUSIONS: Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.