Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium-201 scanning

Background It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis. Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes. Methods In this single blind study we used thallium-201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined. Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported. Conclusions Non-invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.     

Dermatomyositis sine myositis in five cases

Background A small subset of patients with dermatomyositis develop the characteristic cutaneous manifestations without muscle involvement, the so-called dermatomyositis sine myositis or amyopathic dermatomyositis. Whether systemic treatment of skin disease can prevent the development of myositis remains controversial. Objective The purpose of this study was to evaluate the development of the disease in terms of the onset of muscle symptoms under systemic treatment with corticosteroids or antimalarials. Methods Five patients with dermatomyositis sine myositis were included in this study. All skin biopsy specimens had features consistent with dermatomyositis. Muscle enzymes, electromyograms and muscle biopsies did not reveal any abnormality. Results Corticosteroids were given in three patients and hydroxychloroquine in two. Both regimens proved to be effective in the treatment of skin symptoms. Four patients, three on corticosteroids and one on hydroxychloroquine, did not develop muscle disease 4–7 years after presentation. One patient on hydroxychloroquine showed laboratory evidence of myositis 6 years after initiation of treatment. Conclusion It seems that muscle disease may appear within long periods of time after the onset of the cutaneous manifestations. The term dermatomyositis sine myositis can be used as a provisional diagnosis. Our observations suggest that systemic treatment of skin disease with corticosteroids is successful and may alter the disease course. Hydroxychloroquine may be helpful in some cases.     

Recurrent Photosensitive Dermatitis Preceding Juvenile Dermatomyositis

The classic features of childhood dermatomyositis include muscle weakness, elevated muscle enzymes, and characteristic abnormal muscle biopsy and electromyography. Paramount to the diagnosis are cutaneous dermatoses that include a heliotrope rash and Gottron's papules. Rarely, a photo-sensitive dermatosis may occur. A recurrent photoexacerbated dermatoses can be an initial sign of occult childhood dermatomyositis.     

Juvenile amyopathic dermatomyositis

We report a 15-year-old girl with a 10-year-old history of typical skin features of dermatomyositis (DM) without evidence of muscle involvement. Amyopathic dermatomyositis(ADM) is defined by the presence of biopsy confirmed classic cutaneous findings of dermatomyositis in the absence of any clinical or laboratory signs of muscle disease for at least 2 years after onset of skin pathology. To exclude muscle involvement muscle enzymes should be normal; moreover additional use of magnetic resonance imaging and muscle ultrasound is currently being proposed. It is as yet undetermined. whether early aggressive immunosuppressive treatment of ADM might prevent the development of myositis at a later date or influence the course of the skin disease. In a paediatric patient with ADM we advocate a more expectant attitude with careful and regular monitoring for possible development of muscle disease.     

Cutaneous changes of dermatomyositis precede muscle weakness

A retrospective review of 50 patients with dermatomyositis was performed to determine the temporal relationship between onset of muscle weakness and skin involvement. We found that cutaneous changes sometimes preceded muscle weakness more than a year before the onset of muscle weakness. These findings suggest that the characteristic dermatomyositis eruption without muscle weakness should not preclude a diagnosis of dermatomyositis, and these cases should be carefully followed.     

Dermatomyositis in association with transitional cell carcinoma of the bladder

We report a case of dermatomyositis occurring in association with transitional cell carcinoma of the bladder. The case illustrates the importance of a thorough search for neoplasms in elderly patients with dermatomyositis and is a reminder that bladder cancer may be a rare cause of dermatomyositis. The case also shows that successful treatment of an underlying tumour may lead to resolution of paraneoplastic dermatomyositis, and relapse of cutaneous and muscle symptoms and signs may indicate recurrence of tumour.     

Dermatomyositis and mucinosis

BACKGROUND: Mucin deposition is a common feature in autoimmune collagen diseases including dermatomyositis. Nevertheless, clinical manifestations of mucinosis are uncommon in patients with dermatomyositis. Two cases of mucinosis associated with dermatomyositis are reported. PATIENTS: A 53-year-old woman presented with symmetrical plaques on the upper limbs formed by the coalescence of small, violaceous papules. In addition, she showed the typical cutaneous and muscle features of dermatomyositis. A 44-year-old woman with dermatomyositis of 5 years' evolution developed linear, flesh-colored papules across the flexural creases of her palms and fingers. RESULTS: Skin biopsy of the upper limb lesions in the first patient showed epidermal changes compatible with dermatomyositis and dermal mucin deposition. Histopathologic examination of the palmar lesions of the second patient showed less intense epidermal changes of dermatomyositis and dermal mucin deposition. CONCLUSIONS: Mucin deposition in patients with dermatomyositis may have an unusual clinical presentation, and it should be considered in the differential diagnosis of atypical cutaneous lesions in these patients.     

Bullous pemphigoid associated with dermatomyositis and colon carcinoma

It has been suggested that both bullous pemphigoid (BP) and dermatomyositis are associated with internal malignancies. However, there are no reports of the coexistence of these three conditions in one patient. We report the first case, to our knowledge. of coexistent BP, dermatomyositis and colon carcinoma. An 81-year-old Japanese woman was diagnosed with dermatomyositis based on muscular weakness and tenderness of the extremities, increased levels of circulating muscle enzymes and histological inflammatory changes in skeletal muscle and skin. Carcinoma of the sigmoid colon was detected during screening for internal malignancy; she was therefore surgically treated. Ten days after the operation, several large bullous eruptions appeared on her extremities. From the histopathological, immunofluorescence microscopy and BP180 ELISA study findings, we diagnosed the bullous eruptions as BP. Coexistence of BP with dermatomyositis or colon carcinoma is rare. Although the reason why our case exhibited these three disorders is not understood, it is suggested that these conditions may be associated with epitope spreading.     

糖皮质激素联合静脉用免疫球蛋白治疗老年皮肌炎2例

采用中等剂量糖皮质激素联合大剂量静脉注射用免疫球蛋白(IVIg)冲击治疗2例有糖皮质激素应用相对禁忌、且心肌酶谱非常高的老年皮肌炎患者。1例患者IVIg剂量为300mg/(kg·d),共5d,1例患者IVIg剂量为400mg/(kg·d),共4d。2例患者经治疗肌肉症状迅速缓解,心肌酶谱值在短期内降至正常,治疗中无明显不良反应。提示中等剂量糖皮质激素联合大剂量IVIg冲击治疗老年皮肌炎具有良好的疗效。     

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.     

Cutaneous Immunofluorescence in Dermatomyositis

ABSTRACT: Seven patients with dermatomyositis who displayed severe skin and muscle disease, and in whom coexistent systemic lupus erythematosus (LE) was excluded, were evaluated by direct immunofluorescent biopsies of skin lesions. Specimens from six showed deposits of immunoglobulins and complement in small to moderate amounts at the dermal-epidermal junction zone. These deposits were usually in the form of focal granular accumulations which lacked continuity and were not well developed as are those seen in LE, or fluorescent subepidermal hyaline bodies. One patient, however, had a more well developed hand of immunoglobulins at the dermal-epidermal junction. All normal skin specimens in these patients were negative by immunofluorescence.
These findings were helpful in clarifying differences in cutaneous immunofluorescence between dermatomyositis and LE, indicating that dermatomyositis specimens can usually be differentiated from those of LE patients. but demonstrating the possibility that confusion might rarely occur in interpreting a lesional immunofluorescent biopsy in dermatomyositis.     

Panniculitis in a patient with dermatomyositis

Panniculitis is a rarely reported clinical finding in dermatomyositis. It may precede the other manifestations associated with dermatomyositis by as much as 14 months. In all cases, myositis and panniculitis improve simultaneously during treatment. The present report describes the case of a 30-year-old female patient with clinical and histopathological findings consistent with panniculitis two months after the onset of the muscle and cutaneous symptoms that permitted diagnosis of dermatomyositis. The skin lesions regressed following steroid treatment.     

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.     

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.     

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。     

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.     

Dermatomiositis paraneoplásica: estudio de 12 casos

Introduction and objectivesAdult dermatomyositis presents as a paraneoplastic syndrome in up to 25% of cases, but no clinical, histologic, or laboratory markers completely specific for paraneoplastic disease in dermatomyositis have been identified to date. Furthermore, studies on adult dermatomyositis do not usually report the frequency of cutaneous features of dermatomyositis in patients with associated cancer. Our aim was to review the characteristics of paraneoplastic dermatomyositis in patients seen at our hospital.Material and methodsWe studied 12 cases of paraneoplastic dermatomyositis and recorded patient age and sex, associated cancer, time between onset of dermatomyositis and cancer, emergent cutaneous manifestations, muscle involvement, dysphagia, lung disease, and levels of creatine phosphokinase and circulating autoantibodies.ResultsThe mean age of the patients was 61 years and the 2 most common malignancies were ovarian cancer and bladder cancer. The mean time between the diagnosis of cancer and dermatomyositis was 7 months and in most cases, the cancer was diagnosed first. Seven patients had amyopathic dermatomyositis. The most common cutaneous signs were a violaceous photodistributed rash sparing the interscapular area and a heliotrope rash, followed by Gottron papules and cuticle involvement. Superficial cutaneous necrosis was observed in 3 cases. Myositis-specific autoantibodies were not detected in any of the 6 patients who underwent this test.ConclusionsParaneoplastic dermatomyositis is often amyopathic. There are no specific cutaneous markers for malignancy in dermatomyositis. Myositis-specific antibodies are not associated with paraneoplastic dermatomyositis.     

Dermatomyositis-like eruption after long-term hydroxyurea therapy for polycythemia vera

Hydroxyurea (HU) is an antitumor agent used to treat chronic myeloproliferative disorders. HU is usually well tolerated, but several cutaneous changes have been reported during long-term HU therapy. Although the occurrence of dermatomyositis-like changes during long-term HU therapy are well known, they are rarely described in patents with polycythemia vera. We report a 69 year-old-male with polycythemia vera who developed a dermatomyositis-like eruption on his face and dorsum of the hands following long-term HU therapy. Despite the cutaneous features, there were no clinical signs of muscular involvement, and muscle specific enzymes were within normal ranges. After interruption of HU administration, the skin lesions disappeared within two months. The improvement following withdrawal of HU implicated this drug as a possible etiological factor in the development of cutaneous features of dermatomyositis in our patient.     

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。     

Dermatomyositis with a pityriasis rubra pilaris-like eruption: an uncommon cutaneous manifestation in dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.