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Amyopathic dermatomyositis is a variant of dermatomyositis that is characterized by the typical skin rash but without the muscle abnormalities. It has been proposed that the amyopathic and myopathic forms of dermatomyositis exist on a continuum, a concept that is supported by family and genetic studies and the observation that a small proportion of amyopathic patients transform to a frankly myopathic state. The amyopathic state is defined by a lack of muscle weakness and through diagnostic tests, including serum muscle enzymes, electromyogram studies, and muscle biopsies, that are usually normal or show only minimal abnormalities. Despite the lack of weakness, many patients complain of debilitating fatigue. More sensitive measures of muscle function, such as P-31 magnetic resonance spectroscopy, suggest that muscle metabolism is abnormal in amyopathic patients. The amyopathic form is more commonly seen in adults than in children, although juvenile cases are reported. Some early series suggested no association with underlying malignancies, but recent reports indicate that malignancies occur. Determining whether a patient has amyopathic rather than myopathic disease may have prognostic implications.
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PMID:Amyopathic dermatomyositis. 1147 54

Dermatomyositis represents a progressive autoimmune disease of the connective tissue with SLE-like dermatitis, with inflammatory myopathy and muscular weakness. It is an infrequent and serious disease affecting not only the skin and striated muscle. Cases without skin affection are classified as polymyositis. Beside autoimmune mechanisms, aetiology includes also genetic, environmental and paraneoplastic factors. Clinical skin symptoms as well as histopathological findings include Gottron's papulae, heliotropic periorbital erythrema, which, together with palpebral oedema, brings about the weepy look of patients. None of these signs is pathognomic and beside the cutaneous symptoms the diagnosis requires 3 out of 4 other diagnostic criteria for the muscle symptoms. Cutaneous symptoms can precede myositis with various time intervals; such situation is sometimes classified as Amyopathic dermatomyositis (ADM), dermatitis sine myositis or skin dermatomyositis. A case of an ADM patient and the analysis of problematic including the strategy of therapy are presented.
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PMID:[Amyopathic dermatomyositis]. 1468 22

Amyopathic dermatomyositis (ADM) is characterized by the typical cutaneous features of dermatomyositis and minor involvement of the skeletal muscles. A 50-year-old woman had fever, reddening and pain in the distal part of all four limbs, and cutaneous findings such as Gottron's papules and periorbital heliotrope. She showed no muscle weakness or atrophy, and her serum creatine kinase was within the normal range. Electromyography showed no myopathic pattern. Magnetic resonance imaging (MRI) recorded abnormal hyperintensity in the fascia and muscle of the tibialis anterior. A biopsy from the tibialis anterior muscle showed fasciitis and mild myopathic changes with focal perivascular infiltration. This patient also presented with interstitial pneumonitis, although evaluation for malignancy was negative. With steroid therapy, her symptoms and MRI abnormality disappeared within 2 months. This case is therefore considered to be a variant of ADM, presenting as dermato-fasciitis.
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PMID:A case of dermato-fasciitis: amyopathic dermatomyositis associated with fasciitis. 1504 32

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the lack of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We report a 49-year-old patient who presented with nonspecific interstitial pneumonia (NSIP) associated with ADM. The patient failed to respond to prednisolone and immunosuppressive therapy and died. Although idiopathic NSIP is known to have a better prognosis, NSIP in ADM could be a fatal disease. Therefore, we should appropriately treat interstitial pneumonia in ADM even if it is NSIP.
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PMID:Nonspecific interstitial pneumonia with poor prognosis associated with amyopathic dermatomyositis. 1549 21

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.
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PMID:[Amyopathic dermatomyositis associated with a recurrence of breast cancer]. 1647 91

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by interstitial pneumonia that shows a rapid progressive course associated with a poor prognosis. We describe a 70-year-old man who presented rapidly progressive interstitial pneumonia associated with clinically ADM (C-ADM); he was successfully treated with polymyxin B-immobilized fiber column (PMX) hemoperfusion.
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PMID:Rapidly progressive interstitial pneumonia associated with clinically amyopathic dermatomyositis successfully treated with polymyxin B-immobilized fiber column hemoperfusion. 1842 Nov 99

Amyopathic dermatomyositis (ADM) is characterized by the presence of dermatomyositis (DM) for 6 months or more in individuals who have normal muscle enzymes and no clinically significant muscle weakness. The aim of the study was to investigate the initial laboratory data, clinical manifestations, complications, and clinical outcomes of patients with the diagnosis of ADM. We reported 16 cases with the cutaneous findings of dermatomyositis without clinical or laboratory evidence of muscle disease for at least 2 years after onset of the skin manifestations in the Department of Dermatology and Rheumatology at Shanghai Ruijin Hospital between 1998 and 2004. All patients had Gottron's papules, periungual erythema/telangiectasia, and violaceous discoloration of the face, neck, upper chest, and back at some time during the course of their disease. Follow-up of 1 to 10 years after diagnosis found muscle weakness in three patients (18.75%) within 5 years of diagnosis. One patient (6.15%) was rediagnosed as chronic cutaneous lupus erythematosus (CCLE). Four patients (25%) had associated malignancies. Twelve patients (75%) had radiographic evidence indicative of interstitial fibrosis irrespective of respiratory symptoms. Patients with ADM appear to be at risk for developing the same potentially fatal disease complications as those patients with DM (e.g., interstitial lung disease and internal malignancy). These cases further emphasize that the cutaneous manifestations of dermatomyositis are pathognomonic for DM and we propose the term dermatomyositis-like skin disease as a better designation than amyopathic dermatomyositis to describe this distinctive subset of cutaneous symptoms. Dermatomyositis-like skin disease is a complex syndrome, which includes the characteristic cutaneous eruption of dermatomyositis without clinical evidence of muscle disease. Our findings suggest that patients diagnosed with this syndrome are at risk for fatal interstitial lung disease, malignancy, and/or delayed onset of DM or CCLE. Cautious systematic clinical trials should be considered for this group of patients.
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PMID:Amyopathic dermatomyositis or dermatomyositis-like skin disease: retrospective review of 16 cases with amyopathic dermatomyositis. 1965 85

Amyopathic dermatomyositis (ADM) is a clinical subtype of dermatomyositis, characterized by the absence of motor weakness and the presence of normal muscle enzyme levels. ADM is sometimes accompanied by neoplasm or interstitial pneumonia that shows a rapid progressive course both of them are associated with a poor prognosis. A 56-year-old woman with no medical history was referred to the department of medicine because of arthralgia with a remarkable weight loss. She also complained of rapidly progressive dyspnea, cough and photosensitivity. Physical examination on admission showed scaly erythema on the dorsum of the hands (Gottron sign) and periorbital edema with a purplish appearance (heliotropic rash), arthritis, but no muscle weakness. Auscultation of the chest identified audible fine crackles on the lower aspects of both lungs. Results of laboratory findings on admission revealed a lymphopenia. The serum creatine kinase and serum lactate dehydrogenase concentration were normal. IRM muscle and electromyography were normal. Antinuclear antibody was positive 1:80 and anti-Jo-1 antibody and other autoantibodies to specific antigens were all negative. High resolution computed tomographic chest scans also revealed diffuse ground-glass opacities in both lungs with basilar predominance. Arterial blood gas analysis revealed hypoxia and hypocapnia. LBA was not performed because of the deterioration of respiratory symptoms. There was no neoplasm associated. The diagnosis of ADM complicated with ADM rapidly progressive interstitial pneumonia was made. Despite of IV methylprednisolone pulse therapy (1g*day-1 for 3 days) and cyclophosphamide, she died by respiratory failure.
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PMID:[Interstitial pneumonia complicating amyopathic dermatomyositis: a case report]. 1999 56

A 37-year-old Caucasian woman was evaluated for a photosensitive dermatitis. A positive anti-nuclear antibody with a titer of 1 : 1280 and a speckled pattern was noted and she was diagnosed with subacute cutaneous lupus erythematosus (SCLE). Although the initial dermatologic diagnosis was SCLE, a skin biopsy suggested the additional possibility of dermatomyositis because of increased dermal mucin. We began following her at the request of the dermatology department, and a diagnosis of amyopathic dermatomyositis was made based on the lack of objective muscle weakness, normal muscle enzymes, negative double-stranded DNA, SSA/SSB, and RNP/Smith antibody panel, and especially on the cutaneous examination findings. A malignancy evaluation included a normal CT scan of her chest, abdomen, and pelvis, esophagogastroduodenoscopy, colonoscopy, mammography, pelvic ultrasound, Papanicolaou smear, and endometrial biopsy. She developed vaginal bleeding 1 year after the onset of her skin manifestations. Repeat gynecologic evaluation, including cervical biopsy, revealed a large B-cell cervical lymphoma. Amyopathic dermatomyositis and lymphoma of the cervix are both rare conditions. Our case emphasizes the importance of considering underlying malignancy at the time of diagnosis and while the patient is followed clinically. Additionally, the clinician must remain vigilant in evaluating any new clinical changes in follow-up care. To our knowledge, this is the first documented case of lymphoma of the cervix in the setting of amyopathic dermatomyositis.
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PMID:A rare lymphoma in a patient with amyopathic dermatomyositis. 2014 Dec 37

Inflammatory myopathies are a heterogeneous group of diseases with unknown etiology characterized by inflammation of the skeletal muscles and proximal muscle weakness. Dermatomyositis (DM) is an idiopathic inflammatory myopathy with characteristic cutaneous findings such as heliotrope rash, Gottron's sign, Gottron's papules, shawl sign and machinist hand. Amyopathic dermatomyositis (ADM) is a rare but well-recognized clinical subtype of DM, constituting aproximately 10-20% of patients with this disease. It generally manifests only pathognomonic skin findings without clinical and laboratory evidence of muscle involvement. In this report, we present a rare case of ADM associated with interstitial pulmonary disease.
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PMID:A case of Amyopathic Dermatomyositis associated with Interstitial Pulmonary Disease. 2679 13


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