Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011633 (dermatomyositis)
 4,181 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clinical and laboratory findings were correlated from 46 patients with IgG localization in epidermal nuclei in a speckled (particulate) pattern on direct immunofluorescence of normal skin. Cutaneous manifestations included lupus erythematosus (LE), swollen hands or sclerodactyly, alopecia, vasculitis, and dyspigmentation. Systemic manifestations included arthritis or arthralgia, Raynaud's phenomenon, serositis, vascular headaches, mild renal disease, myositis, and sicca syndrome. High titer (mean = 1:142, 800) serum antibody to extractable nuclear antigen (ENA) was found in 81%. Eighty-six percent had antibody to an RNase-sensitive antigenic component of ENA (ribonucleoprotein or RNP); 14% had antibody to an RNase-resistant ENA termed Sm. Deposition of IgG in a speckled pattern in epidermal nuclei is an immunopathologic marker for a subset of connective tissue disease characterized by antibody to ENA. Those with Sm specificity had systemic LE (SLE); Those with RNP specificity had Raynaud's phenomenon usually associated with overlapping features of SLE, scleroderma, and/or dermatomyositis.
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PMID:Speckled (particulate) epidermal nuclear IgG deposition in normal skin. Correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen. 34 15

The study included 19 children with dermatomyositis. All showed frequently atypical cutaneous disords which in most cases were the initial manifestation. In every case there was some clinical evidence of proximal myopathy. Arthralgias were frequently found and sinusal tachycardia was present in 10 cases. The correct diagnosis was made from the beginning only in 6 cases. Systemic lupus erythematosus was the main cause of confusion. The differential diagnosis between these two entities cannot be made only by the presence or not of nephropathy, even if this disease was evident in 42% of patients with dermatomyositis. The most useful laboratory tests for the diagnosis and management were CPK, aldose and serum TGO. Electromyography was abnormal in every case where it was practiced. Muscular biopsy was valuable in 15 of the 19 patients. All were treated with prednisone and in most cases with good results.
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PMID:[Dermatomyositis in pediatrics]. 62 32

Several cases of isolated C2 deficiency in man have been reported in the medical literature. The earliest cases did not seem to be associated with known diseases or syndromes; more recently, reports of C2 deficiency associated with systemic lupus erythematosus; anaphylactoid purpura, recurrent infections, and dermatomyositis have appeared. We report here another case of C2 deficiency. The propositus, a 24-year-old woman, had a lupus erythematosus-like rash and a history of arthralgia, as well as a selective deficiency of C2. Studies of hemolytic C2 of the immediate members of her family indicate an autosomal-recessive mode of inheritance. These findings add to the increasing evidence that a C2 deficiency predisposes some persons to serious vascular diseases.
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PMID:Lupus erythematosus-like syndrome with a familial deficiency of C2. 127 22

A female patient with acanthosis nigricans, insulin resistant diabetes, and generalized lipoatrophy is reported. The patient developed skin pigmentation and acanthosis nigricans around the age of 34. Arthralgia, muscle weakness, and peripheral neuropathy were also present when she first visited us at 36 years of age. Dermatomyositis, systemic sclerosis, and internal malignancy were ruled out, and the diagnosis of acanthosis nigricans and insulin resistant diabetes was made. Her diabetes gradually worsened and, since the age of 39, she has been treated with an oral anti-diabetic drug. Around the age of 47, generalized lipoatrophy became prominent. Insulin receptor studies ruled out insulin resistant diabetes type A and B. At this point, we diagnosed this patient as having lipoatrophic diabetes, which is a syndrome characterized by insulin resistant diabetes, acanthosis nigricans, generalized lipoatrophy, and other metabolic disturbances. The control of her diabetes has been poor, and diabetic neuropathy and lipoatrophy-induced painful skin lesions such as clavus and tylosis have been persistent. The present case indicates the importance of careful skin examinations in the diagnosis of this syndrome.
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PMID:Lipoatrophic diabetes. 160 89

The peculiarities of beginning of disease, clinical factors of risk in 70 children (aged under 8) with dermatomyositis are discussed. We have followed up 70 children (40 girls and 30 boys) with dermatomyositis at the age of 14 months to the age of 8 years. Most of the children were born in normal time, they have normal life functions during the first year of life. The most frequent intercurrent diseases were respiratory infections, angina; the chicken-pox was frequent too. 12 children had food allergy and 20 children-drug allergy. The most frequent factors preceded dermatomyositis were respiratory diseases and inoculations. Assembling of genealogical anamnesis was made according to "family portrait". 14 families of children with dermatomyositis (369 relations in I-IV degree of relationship) were examined. Genealogical investigations revealed the high frequency of rheumatological pathology in proband's families. Small anomaly of development among probands (M +/- m = 12.5 +/- 0.6) exceeded these quantity comparing to the control group (n = 60). Dermatollphyics (98 indices on each child) contained a number of peculiarities for forming the group of risk. Onset of disease was acute or subacute in 2/3 of children. Primary chronic onset and the progress of the disease took place in 10 cases. An acute onset of dermatomyositis was characterized by fever, myaglia, arthralgia, bright skin symptoms: widespread purple violet face erythema, a "butterfly wing" or "paraorbital glasses", palmal erythema and widespread vascular manifestations on face, chest, back and limbs in the form of net--"livedo reticularis".
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PMID:Peculiarities of dermatomyositis (DM) in early age. 184 3

Fourteen patients with childhood scleromyositis followed from 1 to more than 10 years experienced concomitant sclerodermoid and dermatomyositis features, variably expressed at one time or another during the course of the disease. The most characteristic features were myalgia-myositis, arthralgia-arthritis, puffy, atrophic, sclerotic fingers, and Raynaud's phenomenon. This overlap syndrome was the most frequent sclerodermoid condition in children, differing from both systemic scleroderma and dermatomyositis. The course of the disease was protracted and rather benign, and PM-Scl antibody was an important diagnostic and prognostic marker. We present criteria for diagnosis of scleromyositis and its differentiation from systemic scleroderma, dermatomyositis, and Sharp overlap syndrome.
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PMID:Childhood scleromyositis: an overlap syndrome associated with PM-Scl antibody. 190 68

The recent delineation of a clinical syndrome marked by eosinophilia, myalgia, and scleroderma-like skin changes associated with L-tryptophan use has necessitated the Centers for Disease Control to initiate a health alert. The likely association of L-tryptophan ingestion with a syndrome that mimics eosinophilic fasciitis (Shulman's syndrome) further identifies an environmental agent associated with an inflammatory sclerosing rheumatic disease process. In this report, we present the clinical, morphologic, and enzyme histochemical findings in muscle, skin, and fascia biopsies from 14 cases fulfilling the Center for Disease Control diagnostic criteria for L-tryptophan-associated eosinophilia-myalgia syndrome. The clinical syndrome reveals a high incidence of arthralgia, elbow contracture, and clinical neuropathy. The absence of significant change in creatine kinase or sedimentation rate allows for diagnostic separation from other inflammatory myopathies. Histoenzymatic features in muscle biopsies reveal a preferential epimysial-perimysial noneosinophilic infiltration characterized by acid phosphatase reactive histiocytosis, nonnecrotizing venulitis, perineural inflammation within dermis and perimysium, type II fiber atrophy with superimposed denervation features, and perifascicular alkaline phosphatase reactivity representing early neofibroplasia. The constellation of changes in skin, fascia, and muscle, with the defined clinical syndrome, allows for accurate differentiation from allied syndromes, including eosinophilic polymyositis, scleroderma, idiopathic polymyositis/dermatomyositis, polyarteritis nodosa, and toxic oil syndrome. Accurate differentiation from eosinophilic fasciitis still rests on a history of L-tryptophan ingestion.
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PMID:Neuromuscular manifestations of L-tryptophan-associated eosinophilia-myalgia syndrome: a histomorphologic analysis of 14 patients. 198 74

A 49-year-old woman was admitted in February 1987, with a six-month history of joint pain, maculopapular and erythematous rash, proximal muscle weakness and a two-month history of skin ulceration and dyspnea on exertion. Physical examination showed Gottron's papules on her fingers and a faint heliotrope rash. Biopsy of erythematous skin lesions on the shoulder and the back of the hand revealed perivascular inflammatory cell infiltration and tiny ulcerative lesions of the cutaneous tissue. Biopsy of the right quadriceps muscle showed type II fiber atrophy and slight perivascular lymphocytic infiltrate, whereas serum CPK level was within normal range. Chest X-ray film showed granular infiltrates in both lower lung fields. Based on the current findings the case was diagnosed as dermatomyositis associated with interstitial pneumonia. The administration of prednisolone, 30 mg/day resulted in the improvement of the skin lesions and muscle weakness, while the intensity of lung infiltrates was little affected. Three months after steroid therapy, the patient was readmitted because of increasing dyspnea on exertion and multiple skin ulcers. Chest X-ray revealed a small amount of gas in the mediastinum, in addition to slight deterioration of interstitial lung disease. In spite of various treatments for pneumomediastinum, including bed rest, administration of analgesics and oxygen inhalation, it developed rapidly, complicated severe subcutaneous emphysema and right-sided pneumothorax. Although high-dose prednisolone therapy and mediastinal drainage were performed, the pneumomediastinum was not resolved and she died from respiratory failure. At autopsy, predominant histological features of the lungs were acute interstitial pneumonia with hyaline membrane and edematous granulation formation in the alveoli.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of dermatomyositis which rapidly developed to respiratory failure in the presence of pneumomediastinum]. 268 9

An effort was made to identify all patients with polymyositis/dermatomyositis (PM/DM) admitted to hospitals in Israel from 1956-1976. The diagnosis of PM/DM was retrospectively reviewed in 92 (46 definite, 26 probable, and 20 possible) cases. The most common complaints and physical findings in the course of the disease were muscle weakness (86 patients), rash (53 patients), arthritis or arthralgia (39 patients), and dysphagia (35 patients). Elevated serum aldolase levels were found in 64% of the patients for whom data were available; 92% had abnormal electromyogram results, and 60.9% had muscle histopathology consistent with PM/DM. Malignancy was diagnosed in 13 patients. Malignancy, ischemic heart disease, and pulmonary complications were the most common causes of death. The actuarial survival curve was heterogeneous, with an accelerated mortality during the first year after diagnosis and a slower mortality during the following 7 years. Independent unfavorable prognostic signs were: failure to induce remission, leukocytosis, fever, older age, a shorter disease history, and dysphagia.
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PMID:Prognostic factors in polymyositis/dermatomyositis. A computer-assisted analysis of ninety-two cases. 397 73

A 54-yr-old man was admitted to Hokkaido University Hospital, complaining of fever, multiple arthralgia, edematous erythema and face and muscular weakness of extremities during the last 2 months. He was diagnosed as dermatomyositis by acceleration of ESR, elevation of GOT, GPT, CPK, aldolase, moderate increases of collagen fibers in biopsy specimen of skin and his clinical signs. Although stools were positive for occult blood, the routine radiographic examination failed to detect the bleeding site in the upper GI. tract. However, in the double contrast picture of the stomach, a very fine abnormal linear shadow was observed at the upper corpus of the lesser curvature. This linear shadow was a margin of the tumor, retrospectively. About 4 months later, abnormal pain occurred and a mass was palpable in the left lumbar region, suggesting a pancreatic tumor. He was operated on excising the tumor, but was performed only exploratory laparotomy because of the presence of intra-abdominal metastases. Death occurred 40 days after the operation and necropsy was done. The gross anatomical findings of the abdomen showed a stomach tumor as large as an infant's head and its metastases to pancreas, lymph nodes, and greater and lesser omentum. Esophageal mucosa including esophagocardiac junction was intact. Histological examination of the intragastric tumor revealed a typical squamous cell carcinoma with keratinization. According to the absence of the components of adenocarcinoma and squamous metaplastic gastric mucosa of non-cancerous areas in the stomach, it seemed likely to be a heterotopic squamous cell carcinoma. It was unknown about the precedence between the stomach cancer and dermatomyositis. There have been 11 cases of primary pure squamous cell carcinoma in the world literature since 1968, but this is the first case report of coexistence of these two diseases.
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PMID:[A case report of a primary pure squamous cell carcinoma of the stomach associated with dermatomyositis (author's transl)]. 726 22


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