Juvenile amyopathic dermatomyositis

A 3-year-old girl presented with a 6-month history of multiple, light-pink, flat-topped papules over the dorsal aspects of the metacarpophalangeal and interphalangeal joints of the hands and feet. Nailfold telangiectases, ragged cuticles, and a heliotrope color of the upper eyelids were also evident, but there was no clinical evidence of muscle weakness and levels of muscle enzymes were normal. A biopsy specimen from one of the papules showed a vacuolar interface dermatitis consistent with a diagnosis of dermatomyositis. This report draws attention to juvenile amyopathic dermatomyositis, which is an uncommon subtype of dermatomyositis with an excellent prognosis.     

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.     

无肌炎性皮肌炎诊断与治疗

无肌炎性皮肌炎是皮肌炎的一种新类型，它有特征性的皮肌炎皮肤改变而无肌炎，持续24个月，它与皮肌炎一样有恶性肿瘤的高伴发率，属于伴肿瘤性皮肤病．无肌炎性皮肌炎有独立的诊断标准。     

Juvenile dermatomyositis

Juvenile dermatomyositis (JDMS) is a chronic inflammatory condition characterized by muscle weakness and a distinctive rash caused by underlying vasculopathy. Long-term complications include subcutaneous and muscular calcification, contractures and in some cases the gradual development of a second connective tissue disease. Early aggressive treatment with systemic immunosuppressants and other agents such as intravenous immunoglobulin (IVIG) reduces mortality and morbidity.     

Wong型无肌病性皮肌炎一例

患者,女,72岁。头面颈部红斑、丘疹伴瘙痒16年,日晒后加重。实验室检查示：抗TIF1-γ抗体阳性。额部皮肤组织病理表现为表皮角化过度,局灶性角化不全和界面皮炎改变。结合患者典型皮肌炎皮肤表现和额部毛发红糠疹样皮疹,诊断为Wong型无肌病性皮肌炎。给予阿维A、海棠合剂、1%吡美莫司乳膏等治疗,病情逐渐好转。     

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。     

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。     

A systematic review of juvenile-onset clinically amyopathic dermatomyositis

BACKGROUND: Dermatomyositis (DM) presenting during childhood or adolescence classically encompasses hallmark cutaneous changes, proximal muscle weakness, and laboratory evidence of myositis. When cutaneous manifestations of DM are present without muscle weakness for > 6 months, the term 'clinically amyopathic DM' applies (syn. DM sine myositis). OBJECTIVES: To review the clinical and epidemiological features of published cases of juvenile-onset clinically amyopathic DM, with comparison with adult-onset clinically amyopathic DM and juvenile-onset classical DM. METHODS: Systematic review of the published literature. RESULTS: We identified 68 cases of juvenile-onset clinically amyopathic DM published during 1963-2006. The disease in 18 of 68 (26%) patients subsequently evolved to classical DM. Overall, the mean age at diagnosis was 10.8 years (range 2-17) with nearly equal male/female ratio and mean follow-up of 3.9 years. Among cases with diagnostic testing, 10 of 19 had a positive antinuclear antibody titre, two of nine had elevated erythrocyte sedimentation rate and two of 51 had elevated creatine kinase (CK). Of patients with normal CK, three of 22 had abnormal electromyography, one of 19 had abnormal muscle biopsy, and one of nine had abnormal magnetic resonance imaging. Calcinosis was reported in three of 68. No cases of severe vasculopathy (resulting in ulceration), interstitial lung disease or internal malignancy were reported. CONCLUSIONS: This review suggests a good prognosis for children with clinically amyopathic DM. A minority of patients with negative muscle enzymes had positive ancillary testing for myositis, and these patients rarely developed muscle weakness. Predictive factors for progression to classical DM were not identified. Symptomatic treatment of cutaneous involvement and close clinical monitoring may be an alternative to aggressive immunosuppression.     

[开放获取] 临床无肌病性皮肌炎诊断标准的演进

【摘要】 目前皮肌炎诊断使用最广泛的标准是Bohan和Peter标准,该标准要求患者除典型皮疹外,还须满足至少2条肌炎表现才能诊断皮肌炎。临床无肌病性皮肌炎（CADM）因无肌炎表现而被排除在外,这使CADM的早期研究十分匮乏,不被临床医生所重视。另外,诊断CADM依赖于对皮疹的认识和皮损组织病理表现,因CADM早期皮疹表现不典型,往往被误诊为玫瑰痤疮、脂溢性皮炎、红斑狼疮等其他疾病,从而影响早期诊断和治疗以及对疾病预后的判断。本文梳理CADM的诊断标准,以利于其早期识别和诊断,也为开展相关临床研究提供依据。     

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。     

抗信号识别颗粒抗体阳性的皮肌炎/临床无肌病性皮肌炎患者临床特点分析

【摘要】 目的 分析血清抗信号识别颗粒（SRP）抗体阳性的皮肌炎/临床无肌病性皮肌炎（DM/CADM）患者的临床特点。方法 收集2015年6月至2017年7月上海交通大学医学院附属瑞金医院皮肤科病房收治的90例DM/CADM患者的临床资料,用免疫印迹法检测患者血清抗SRP抗体。采用t检验和χ2检验等方法进行统计学分析。结果 90例DM/CADM患者中11例（12.2%）血清抗SRP抗体阳性,其中6例DM,5例CADM。82例成人DM/CADM患者中,抗SRP抗体阳性合并恶性肿瘤的发生率显著高于抗SRP抗体阴性患者［7/9比31.5%（23/73）,χ2 = 7.394,P = 0.006］。11例抗SRP抗体阳性患者均有DM典型皮疹,DM皮损面积和严重指数为18.1 ± 2.9。抗SRP抗体阳性患者翼状征的发生率显著高于抗体阴性患者［7/11比29.9%（20/67）,Fisher精确检验,P = 0.028］。抗SRP抗体阳性患者抗核抗体阳性率显著高于抗体阴性患者［4/8比16.7%（13/78）,χ2 = 6.053,P = 0.014］。10例抗SRP抗体阳性患者（6例DM和4例CADM）行双大腿肌肉磁共振检查,其中8例肌群内信号异常,肌群肿胀2例,皮下水肿2例,肌筋膜肿胀1例,仅2例无异常。抗SRP抗体阳性患者均无肺间质病变和心肌受累。结论 抗SRP抗体阳性DM/CADM患者躯干翼状征发生率高,合并恶性肿瘤的风险显著增加。DM/CADM患者在病程早期检测抗SRP抗体水平有助于预测恶性肿瘤的发生。     

Epidermal nuclear CIq deposits in a patient with amyopathic dermatomyositis

We report a case of amyopathic dermatomyositis, in which C1q deposits on the epidermal nucleus were immunohistologically found as well as Ig deposits at the dermoepidermal junction (DEJ). Direct immunofluorescence (IF) examination of infiltrated erythematous lesions revealed C1q deposits on the epidermal nucleus and fibrinogen at the DEJ, and examination of hyperkeratotic erythematous lesions showed linear deposits of Ig G and Ig A at the DEJ but not in the nuclei of epidermal cells. The authors discuss the direct in vivo interaction between nucleus and immunoreactants in dermatomyositis.     

Fatal interstitial pulmonary fibrosis in anti-Jo-1-negative amyopathic dermatomyositis

Amyopathic dermatomyositis (DM) describes a subpopulation with the cutaneous eruption of DM, but without muscle involvement. Interstitial pulmonary fibrosis is a recognized complication of DM, often correlated with antisynthetase enzymes, such as anti-Jo-1. We describe a case of fatal IPF in a patient with anti-Jo-1 antibody-negative amyopathic DM.     

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.     

Skin metastasis of breast cancer clinically undistinguished from amyopathic dermatomyositis

We report a 65-year-old woman who consulted us on May 25, 1998, showing pruritic, partially flagellate erythema on the back and upper extremities, livedo lesions with erythema on the loins, and erythematous papules on the dorsal finger joints for 2 months. Histopathological findings of erythema on the back showed mononuclear cell infiltration around capillaries and marked edema in the dermis. Laboratory data were within normal range except for positive anti-nuclear antibody. She had undergone total left mastectomy on June 2, 1997 for breast cancer. Supraclavicular lymph node metastasis was found at the beginning of May, 1998. A diagnosis of amyopathic dermatomyositis associated with breast cancer was made. Erythema with itching gradually subsided from the end of August, 1998. Treatment with radiation and chemotherapy reduced lymph node swelling, but complete remission was not obtained. Erythema similar to the previous lesion but without itching re-appeared on the back from January, 2000. Histological findings of erythema showed many carcinoma cells similar to the primary lesion of left breast cancer in the whole dermis. A diagnosis of skin metastasis of breast cancer was made. These findings suggest that skin metastasis should be taken into account for patients with erythema on the trunk similar to dermatomyositis.