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Results for “DERMATOMYOSITIS”

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PubMed2004

Severe, reversible pulmonary hypertension in a patient with monoclonal gammopathy and features of dermatomyositis.

Pulmonary hypertension is an extremely serious and potentially fatal disorder. Although pulmonary hypertension is a potential complication of connective tissue disease, it has been reported rarely in patients with dermatomyositis. Similarly, multiple myeloma is rare in patients with dermatomyositis. We describe a patient with severe pulmonary hypertension who also had features of dermatomyositis and monoclonal gammopathy. To our knowledge, this is the first reported case of a patient in whom all 3 disorders occurred concurrently. Even more striking is the fact that the patient responded to treatment with cyclophosphamide and prostacyclin. He is asymptomatic more than 5 years after treatment was discontinued.

Adult↗
PubMed1990

Muscle regeneration and cell-mediated cytotoxicity in the autologous muscle culture of dermatomyositis.

Muscle culture from a dermatomyositis patient was performed to investigate the muscle regeneration and cell-mediated cytotoxicity. In the primary culture, spindle-shaped mononuclear myoblasts exponentially increased in number. In the secondary culture, the myotubes fused with the myoblasts and/or other myotubes into large thick syncytia. The cell morphology and growth pattern of the cultured muscle cells in dermatomyositis were identical to those of normal healthy controls. The autologous mononuclear cells added in the secondary culture of day 14 adhered to the surface membrane of the myotubes. Three days after this treatment, the myotubes underwent degenerative changes. On the other hand, in two series of sister cultures, each added with the autologous serum and control medium, no remarkable morphological changes were observed. The results of the present study suggest that dermatomyositis could be precipitated by the associated abnormal cell-mediated cytotoxicity, but not by abnormalities in muscle fibers per se.

Aged↗
PubMed1997

[Dermatomyositis and malignancy of the pharynx (report of 3 cases)].

Three patients with dermatomyositis are reported, two of them had suffered from carcinoma of the nasopharynx and the last one suffered tonsillar carcinoma. The diagnosis the therapy, the prognosis and the relationship between dermatomyositis and malignancy were discussed in detail in this paper. The author emphasised that careful examination of the nose and pharynx would be very important for the patient with dermatomyositis.

Carcinoma, Squamous Cell↗
PubMedDate not supplied

Inclusion body myositis long after dermatomyositis: a report of two cases.

Dermatomyositis, polymyositis, and inclusion body myositis are rare illnesses which appear to be distinct in clinical and pathologic features, pathogenesis, natural history, and response to therapy. We report two patients who first developed dermatomyositis, and then, after a disease-free interval of many years, developed inclusion body myositis. This may have useful therapeutic implications for patients with dermatomyositis whose illness bocomes refractory to treatment.

Adult↗
PubMed1999

Bullous dermatomyositis associated with nasopharyngeal carcinoma--a case report.

Cutaneous manifestations of dermatomyositis commonly include Gottron's papules, heliotrope rash, photosensitivity, poikiloderma and nailfold telangiectasia. Vesicles and bulla are rare. We report a patient with dermatomyositis who presented with blisters and oral ulcers. It is important to recognise this bullous variant in order to avoid a delay in diagnosis. Bullous dermatomyositis may also portend a poorer prognosis. Our patient was subsequently diagnosed to have undifferentiated nasopharyngeal carcinoma.

Dermatomyositis↗
PubMed2000

Fatal bladder cancer and dermatomyositis.

Dermatomyositis is an uncommon inflammatory myopathy accompanied by characteristic cutaneous findings. Adult-onset dermatomyositis is often associated with internal malignancy. We report a case of dermatomyositis associated with an aggressive and fatal case of transitional cell carcinoma of the bladder.

Aged↗
PubMed2000

[Dermatomyositis and nasopharyngeal carcinoma: 3 cases].

BACKGROUND: Nasopharyngeal carcinoma is a common cancer in Tunisia with an estimated incidence of 1.8/100,000. The tumor shows a characteristic association with paraneoplastic syndromes. CASE REPORTS: We report three cases of histologically proven dermatomyositis associated with nasopharyngeal carcinoma in patients aged 40, 24 and 65 years. In all cases, the course of the paraneoplastic syndrome ran parallel to the nasopharyngeal carcinoma. DISCUSSION: Dermatomyositis is one of the numerous paraneoplastic syndromes associated with nasopharyngeal carcinoma. Systematic examination of the nasopharynx is required in case of dermatomyositis.

Adult↗
PubMed2000

[Dermatomyositis in childhood].

AIM: The aim of this study is to review the presenting signs and symptoms, laboratory findings and therapeutic regimens of juvenile dermatomyositis in a tertiary hospital. METHODS: We reviewed retrospectively the available medical records of patients who met the clinicopathologic criteria of Bohan and Peter for definite juvenile dermatomyositis. They were followed between 1986 and july 1999 at the pediatric rheumatology section at our institution. RESULTS: The patient population included 3 male and 6 female patients. The mean age at diagnosis was 7 years. Clinical features demanding medical attention at the hospital were: muscle weakness and pain, with associated general symptoms in 4 cases; isolated muscle weakness in 2 cases; muscle weakness associated to general symptoms in 1 case; and monoarthritis in another case. One patient presented initially only with cutaneous rash. Three patients developed calcinosis. Serum CPK and LDH levels were elevated in 8 patients, aldolase in 7 and aminotransferases in 6. Autoantibodies were undetectable in all the patients. Electomyography demonstrated myopatic or mixed pattern in the 5 patients it was practiced. Muscle biopsy showed features of inflammatory myopathy in all the cases. Seven patients were treated exclusively with steroids and 2 patients with steroids, methotrexate and intravenous gammaglobuline. Actually, 6 patients are asymptomatic, 2 have mild muscle weakness, and 1 has died. CONCLUSIONS: The results of our review agree with other series reported. Juvenile dermatomyositis suspicious should be made upon muscle weakness and general symptoms. Treatment with steroids should be started promptly.

Adolescent↗
PubMedDate not supplied

Dermatomyositis with membranous nephropathy.

Dermatomyositis is the connective tissue disease with the least renal involvement. Although some renal findings like proteinuria, hematuria, pyuria, progressive renal insufficiency, and glomerular and tubular calcium deposits with arteriolar fibrosis have been described, glomerulonephritides have rarely been associated with dermatomyositis, especially in childhood cases. We describe a 10-year old boy with the clinical picture of dermatomyositis who underwent renal biopsy due to microscopic hematuria demonstrating membranous glomerulonephritis with Clq deposition. Children with "full-house" membranous glomerulonephritis with deposition of Clq and the other immunoglobulins have been reported to present in the future with the clinical findings of systemic lupus erythematosus. However, laboratory evaluation of our patient for systemic lupus erythematosus was negative at the present time. Thus, we think this case should be followed up closely with special attention to the possible clinical and laboratory findings of systemic lupus erythematosus.

Child↗
PubMedDate not supplied

Hypercapnic coma due to diaphragmatic involvement in a patient with dermatomyositis.

We report an acute respiratory distress by diaphragmatic involvement due to dermatomyositis. A fifty year-old patient with typical dermatomyositis presented an acute respiratory insufficiency with hypercapnic coma due to diaphragmatic muscle involvement. Respiratory state required mechanical ventilation initially and improved secondarily gradually upon corticosteroids and intravenous immunoglobulins. Only few cases of acute respiratory distress in dermatomyositis due to respiratory muscle involvement are reported in literature.

Coma↗
PubMed2001

Juvenile dermatomyositis complicated with vasculitis and duodenal perforation.

Duodenal perforation has been reported in patients taking steroids and non-steroidal anti-inflammatory drugs (NSAIDs). However, its association with juvenile dermatomyositis is extremely rare. A 4-year-old boy with dermatomyositis presented with intractable abdominal pain which was aggravated after steroid and NSAID therapies. A widespread retroperitoneal abscess was noted on abdominal computerized tomography. An emergency operation showed an ulcer and perforation at the junction of the third and fourth portions of the duodenum. Debridement of the necrotic tissue and repair of the perforation were performed. The postoperative course was complicated by an anastomotic leak, which was corrected by reanastomosis. In addition to intestinal vasculitis, duodenal vasculitis complicated with ulcers and perforation should be included in the differential diagnosis of a child with juvenile dermatomyositis presenting with abdominal complaints.

Abdominal Abscess↗
PubMed1975

Azathioprine hypersensitivity mimicking an acute exacerbation of dermatomyositis.

We describe an unusual hypersensitivity reaction to azathioprine occurring in a patient with dermatomyositis, in which the acute manifestations suggested an exacerbation of the patient's underlying disorder. However, laboratory studies were not consistent with an acute exacerbation of dermatomyositis, and the subsequent clinical course confirmed the impression that the patient's symptoms were due to hypersensitivity to azathioprine. Physicians should be aware that a hypersensitivity reaction to azathioprine may mimic a flare of dermatomyositis.

Acute Disease↗
PubMed2002

[Rhinopharyngeal carcinoma and dermatomyositis: description of a clinical case].

Nasopharyngeal carcinoma has long been reported as the predominant type of cancer associated with dermatomyositis in many several Asian countries, including Hong Kong, Singapore, and Southern-Cina. Dermatomyositis is one of the idiopathic inflammatory myopathies showing characteristic cutaneous manifestations. Reviews from the western literature have demonstrated that certain cancers, such as ovarian and breast carcinoma in women and lung and prostate carcinoma in men, are highly associated with DM relative to the general population. We report the case of a Caucasian Italian patient with nasopharyngeal carcinoma and dermatomyositis. Considering the rarity of nasopharyngeal carcinoma among whites, both the detection and the report of each new case are noteworthy in defining the geographic and ethnic distribution of this tumor.

Autoimmune Diseases↗
PubMed2002

[Pulmonary sarcoidosis in a case of dermatomyositis under long-term steroid therapy].

Pulmonary sarcoidosis in association with dermatomyositis has rarely been reported. A 52-year-old woman was admitted for evaluation of a previously unrecognized bilateral hilar lymphadenopathy on a chest radiogram in August 1995. She had been receiving oral corticosteroids for dermatomyositis, which was confirmed by the typical skin rash and the myogenic changes on an electromyogram in 1977. She had no respiratory symptoms at the time of admission. Chest computed tomography showed hilar and mediastinal lymphadenopathy with reticular shadows in the peripheral lung parenchyma. A 67gallium-citrate scintigram apparently revealed accumulation in bilateral hilums. The tuberculin skin reaction was negative. Flowcytometry of bronchoalveolar lavage fluid indicated a CD4/CD8 ratio of 5.37. Thoracoscopic biopsy specimens sampled from the mediastinal lymph nodes and the lung demonstrated non-caseating granulomas. A definitive diagnosis of pulmonary sarcoidosis was made from these findings. The chest radiographic findings were spontaneously resolved after three years with no increase in the dosage of oral corticosteroids. It is well known that an abnormal immune response playa an important role in development of dermatomyositis and sarcoidosis. This case suggested close associations in both diseases.

Anti-Inflammatory Agents↗
PubMedDate not supplied

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.

Aged↗
PubMed2002

[Perforating skin ulcers occurring in an adult with dermatomyositis].

BACKGROUND: Skin ulcerations are rarely reported in dermatomyositis of the adult. We report on a case of perforating ulcers resistant to classical treatments in a woman with dermatomyositis. CASE REPORT: A 34 year-old woman, treated for typical dermatomyositis, developed a few weeks after starting her treatment, multiple perforating skin ulcers on her shoulders, elbows and wrists though general signs had completely disappeared. In spite of the increase in oral steroids, the patient presented again with worsened lesions and a new perforating ulcer on the hand which lead to the breaking of a tendon. She underwent hand surgery and was also treated with a bolus of steroids IV (1 g daily during 3 days), followed by oral steroids, IM methotrexate and antimalarial drugs. The lesions healed completely after one month, with no atrophy. DISCUSSION: Skin ulcers are rarely described in dermatomyosites of the adult and are associated with underlying vasculitis and bad prognosis. Our case is remarkable by the absence of vasculitis, a very slow cure requiring aggressive treatment and excellent prognosis after three years of follow-up.

Adult↗
PubMed2002

Steroid refractory interstitial pneumonitis in a patient with juvenile dermatomyositis.

Interstitial pneumonitis is a severe complication of juvenile dermatomyositis. We report a 4-year-old girl with juvenile dermatomyositis. Coughing, shortness of breath, and general malaise developed during steroid treatment. The histology of her lung biopsy is compatible with interstitial pneumonitis. Aggressive treatment including intravenous methylprednisolone pulse therapy, intravenous immunoglobulin, and oral cyclosporin all failed. Creatinine phosphokinase level was within the normal range during the disease course. The clinical features are discussed and the importance of a differential diagnosis of interstitial pneumonitis in patients with juvenile dermatomyositis is emphasized.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Unexpected IgA nephropathy during the treatment of a young woman with idiopathic dermatomyositis: case report and review of the literature.

This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates that the most common pattern seen in idiopathic polymyositis is mesangial proliferative glomerulonephritis. However, both membranous and mesangial proliferative glomerulonephritis are often seen in idiopathic dermatomyositis. It is still not clear, however, whether the humorally- mediated immune process in dermatomyositis and the cell-mediated immune process in polymyositis can explain the different patterns of occurrence of glomerular lesions in these two closely related disease entities.

Adult↗