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PubMedDate not supplied

Restrictive cardiomyopathy in dermatomyositis.

The association between dermatomyositis and restrictive cardiomyopathy has not been reported before. We present here the clinical, echocardiographic and muscle biopsy data for a patient with dermatomyositis and restrictive cardiomyopathy. In a 78-year-old male with a history of arterial hypertension, recurrent episodes of atrial fibrillation and syncopes, rupture of an infra-renal aortic aneurysm with complications (recurrent QT-prolongation, lumbo-sacral plexopathy, transient ischaemic attack, peripheral embolism), monoclonal gammopathy, subdural haematoma, focal seizures, megaloblastic anaemia, leucopenia, eosinophilia, elevated muscle enzymes and increasing tiredness, dermatomyositis was diagnosed upon clinical presentation, muscle enzyme and muscle biopsy findings. Cardiological examination revealed atrial fibrillation, left anterior hemiblock and restrictive cardiomyopathy. After the exclusion of various differentials for restrictive cardiomyopathy, a causative relationship between restrictive cardiomyopathy and dermatomyositis was assumed. This case suggests the need for suspecting restrictive cardiomyopathy in patients with dermatomyositis. Patients with dermatomyositis should undergo a comprehensive cardiological investigation as soon as the neurological diagnosis is established.

Aged↗
PubMed1996

Case Report: amyopathic dermatomyositis associated with transformed malignant lymphoma.

Amyopathic dermatomyositis is a disease of unknown origin characterized by the specific skin lesions of dermatomyositis but without clinical or laboratory evidence of myopathy. During the past 15 years, a great controversy between the different reports concerning a possible association of dermatomyositis with malignancy has been noted. In this report, the authors describe a patient with amyopathic dermatomyositis who presented first with a benign hyperplasia of the lymph node, which finally transformed into frank malignant lymphoma. In addition to follow-up care, screening tests to search for occult malignancy in patients with amyopathic dermatomyositis (or dermatomyositis) are recommended.

Aged↗
PubMed2003

Update on juvenile dermatomyositis: new advances in understanding its etiopathogenesis.

PURPOSE OF REVIEW: Juvenile dermatomyositis is the most common of the idiopathic inflammatory myopathies in children. It is considered an autoimmune disease of relatively unknown etiology, although environmental exposures and infectious agents are thought to play a role in disease pathogenesis. More recently, data has become available regarding the molecular genetics of children affected with juvenile dermatomyositis and the impact these genes have on disease expression and clinical course. Additionally, features of the immune response, including specific pathways of the humoral and cellular immune systems, have been further described. This article summarizes the most recent advances in understanding the etiopathogenesis of juvenile dermatomyositis. RECENT FINDINGS: This article focuses on advances made in understanding the role that complement, soluble adhesion molecules, thrombospondin-1 levels, and genetics play in the evolution of juvenile dermatomyositis. It also describes microarray technology and gene expression profiling as means of identifying those genes overexpressed in affected children and thus likely involved in disease pathogenesis; microarray technology may also be used to distinguish dermatomyositis from the other inflammatory myopathies, as well as from other myopathies. SUMMARY: In better understanding the pathogenetic mechanisms whereby disease evolves and the means by which genetic profiles influence susceptibility to and expression of disease, immunotherapies to better treat juvenile dermatomyositis may become available in the future.

Child↗
PubMed1993

Analysis of lymphocyte subpopulations in peripheral blood in adult and juvenile cases of dermatomyositis.

Dermatomyositis, recognized as an autoimmune disorder, occurs not only in adults but also in children. In this study, we evaluated lymphocyte subpopulations in the peripheral blood of 24 adult dermatomyositis and 14 juvenile dermatomyositis patients and in 17 healthy adults and 9 healthy children by flow cytometry using monoclonal antibodies. When compared with healthy adults and adult patients with inactive dermatomyositis, the adult patients with active disease had significantly lower percentages of CD3+ and CD8+ cells and a significantly higher percentage of CD20+ cells. In contrast, juvenile dermatomyositis patients had lymphocyte subpopulations not significantly different from those of healthy children; the activity/inactivity of disease did not make any difference. These results support our hypothesis that adult and juvenile dermatomyositis may be diseases of entirely different scope.

Adult↗
PubMed2005

Linkage of dermatomyositis in the Shetland Sheepdog to chromosome 35.

Dermatomyositis is an inflammatory disease of the skin and muscle and is most commonly found in the Shetland sheepdog. Both the clinical presentation and the age of onset of dermatomyositis vary widely, and the inability to diagnose dermatomyositis before clinical symptoms ensue has made control of the disease difficult. Identification of a genetic marker that cosegregates with dermatomyositis would facilitate the development of a DNA-based test for the early detection of affected dogs. We report the use of linkage disequilibrium (LD) mapping to identify linkage to phenotypic dermatomyositis in the Shetland sheepdog. One marker, microsatellite marker FH3570 on canine chromosome 35, had evidence of LD (P=0.00002). Further studies are necessary to narrow the region harbouring the dermatomyositis locus, identify candidate genes and determine mode of inheritance.

Age of Onset↗
PubMed2005

Successful cure of dermatomyositis after treatment of nonseminomatous testicular cancer.

Although the association between dermatomyositis and cancer is well recognized, there have been a limited number of reports of dermatomyositis associated with testicular cancer. We report the case of a 31-year-old man who was diagnosed with dermatomyositis. Because the patient's serum levels of alpha-fetoprotein and human chorionic gonadotropin beta subunit were elevated, the patient was referred to our department. Physical examination revealed an induration (2 cm x 2 cm) in the left testis and the patient was diagnosed with stage IIIB left testicular cancer. A left high orchidectomy was then performed, and pathological examination revealed embryonal carcinoma. The patient was started on a systemic chemotherapeutic (etoposide, ifosfamide and cisplatin; VIP) regimen. After the third chemotherapy session, metastases had completely disappeared, the serum levels of alpha-fetoprotein and human chorionic gonadotropin beta subunit had normalized and the dermatomyositis symptoms had improved. Cancer is known to be a trigger of dermatomyositis. This case illustrates the importance of palpation of the testes in young patients with dermatomyositis.

Adult↗
PubMed2006

Identification of autoantibodies elicited in a patient with prostate cancer presenting as dermatomyositis.

OBJECTIVES: Dermatomyositis is an uncommon autoimmune disease distinguished by proximal muscle weakness and a characteristic skin rash. Dermatomyositis has also frequently been associated with malignancy, typically heralding the diagnosis of ovarian, lung, gastric, or colorectal cancer. We report an unusual case of prostate adenocarcinoma preceded by a diagnosis of dermatomyositis. We hypothesized that in this particular patient, proteins produced by the neoplastic prostatic tissue, which might be normally expressed in muscle tissue, were immunologically recognized as autoantigens. METHODS: Serum from this patient was used to screen a cDNA lambda phage expression library from normal prostate tissue for prostate protein-specific IgG. RESULTS: We identified several immunoreactive plaques encoding known autoantigens, and several encoding known muscle-related proteins, including aldolase C, eukaryotic translation elongation factor 1 alpha 1, transgelin, and acetyl-coenzyme A acyltransferase 1. IgG specific for these proteins were not specifically recognized in sera from other patients with prostate cancer compared with male control blood donors, and were not specifically recognized in a small panel of sera from patients with breast or ovarian cancer and dermatomyositis. CONCLUSIONS: Our results demonstrate that this patient with prostate cancer presenting as dermatomyositis had autoantibodies to specific proteins, possibly associated with his autoimmune myopathy. Moreover, given this patient's history and the multiple treatment options for prostate cancer, the identification of dermatomyositis in men should prompt an evaluation to exclude a concurrent diagnosis of prostate cancer.

Adenocarcinoma↗
PubMed2006

Ureteral cancer associated with dermatomyositis.

Dermatomyositis is a rare inflammatory myopathy that has characteristic cutaneous lesions. Although many malignancies are associated with dermatomyositis, urogenital malignancies have rarely been reported to be associated with dermatomyositis. We report here on the first case of ureteral cancer associated with dermatomyositis. A 42-year-old man presented to us with a skin rash. A clinical diagnosis of dermatomyositis was made due to the skin lesions, muscle weakness, arthralgia, the increased erythrocyte sedimentation rate and the increased creatine kinase level. The patient revealed microscopic hematuria and abnormal urine cytology during the investigation for the underlying malignancy. Retrograde pyelography demonstrated a suspicious lesion in the right mid-ureter, and the ureteroscopic biopsy revealed the urothelial carcinoma. Although an operation was recommended, the patient died of pneumonia associated with his interstitial lung disease, which is one of the poor prognostic indicators of dermatomyositis.

Adult↗
PubMed2005

Altered cytokine expression of peripheral blood lymphocytes in polymyositis and dermatomyositis.

OBJECTIVE: To investigate the intracellular and soluble cytokine levels and T cell subsets in peripheral blood of patients with active and inactive polymyositis and dermatomyositis. METHODS: The frequencies of T and B lymphocytes, T helper (Th), and T cytotoxic (Tc) cells and of interferon gamma (IFNgamma), interleukin (IL)4, and IL10 expression of CD4+ or CD8+ cells were determined by flow cytometry. The concentrations of soluble cytokines were measured with commercial enzyme linked immunosorbent assays. RESULTS: In active dermatomyositis there was a decreased percentage of T (CD3+) lymphocytes and Tc (CD8+) lymphocytes, decreased IFNgamma expression of CD4+ and CD8+ cells, but an increase in B and IL4 producing CD4+ lymphocyte frequencies. These prominent changes disappeared in the inactive stage of the disease. In polymyositis no significant change in these lymphocyte subsets or in intracellular cytokine expression could be detected in either the active or the inactive form. The frequency of IL4+/IFNgamma+ Th cells was calculated and a significantly increased Th2/Th1 frequency was found in active dermatomyositis, and a decreased frequency in inactive dermatomyositis, compared with the control population. CONCLUSIONS: There appears to be a difference between polymyositis and dermatomyositis in the level of peripheral blood lymphocytes and their intracellular cytokine content. These findings provide further evidence for a difference in the pathogenesis of polymyositis and dermatomyositis.

Adolescent↗
PubMed2006

Dermatomyositis associated with celiac disease: response to a gluten-free diet.

The association between dermatomyositis and celiac disease in children has been well documented. In the adult population, however, the association has not been clearly established. A rare case of concomitant dermatomyositis and celiac disease in a 40-year-old woman is presented. After having been diagnosed with dermatomyositis and iron deficiency anemia, this patient was referred to the gastroenterology clinic to exclude a gastrointestinal malignancy. Blood tests revealed various vitamin deficiencies consistent with malabsorption. The results of gastroscopy with duodenal biopsy were consistent with celiac disease. After she was put on a strict gluten-free diet, both nutritional deficiencies and the dermatomyositis resolved. The patient's human leukocyte antigen haplotype study was positive for DR3 and DQ2, which have been shown to be associated with both juvenile dermatomyositis and celiac disease. It is suggested that patients with newly diagnosed dermatomyositis be investigated for concomitant celiac disease even in the absence of gastrointestinal symptoms.

Adult↗
PubMed2005

Diagnostic significance of membrane attack complex and vitronectin in childhood dermatomyositis.

In the current study, whether particular histologic distributions of membrane attack complex and vitronectin are correlated to clinical characteristics of patients with childhood dermatomyositis was investigated. The specimens from 11 patients with childhood dermatomyositis were examined immunocytochemically and compared with those taken from 6 adult dermatomyositis and 5 childhood polymyositis patients. Four out of five membrane attack complex-positive specimens were derived from patients who were within 1 to 3 months from the onset of childhood dermatomyositis and were not treated with prednisone. There were two patients at early stages of childhood dermatomyositis without pathologic characteristics of the disease. However, marked deposits of membrane attack complex on capillaries were seen in one of them. The immunoreactivity of vitronectin was detected at locations similar to those of membrane attack complex. Our results suggest that the detection of membrane attack complex and vitronectin in muscle capillaries will help us diagnose childhood dermatomyositis at an early stage.

Adolescent↗
PubMed1993

Association between bovine collagen dermal implants and a dermatomyositis or a polymyositis-like syndrome.

OBJECTIVE: To determine whether an excess incidence of dermatomyositis or polymyositis or both exist in patients treated with injectable bovine collagen implants and to characterize the clinical picture. DESIGN: Historical cohort study (July 1980 through June 1988). PATIENTS: Patients were identified from personal experience or adverse reaction reports received by the manufacturer. SETTING: An 8-year period in the United States during which approximately 345,000 patients received implants. RESULTS: Eight patients with dermatomyositis and an additional patient with polymyositis were identified from approximately 345,000 patients receiving injectable bovine collagen implants from July 1980 through June 1988. The nine patients with dermatomyositis or polymyositis were diagnosed an average of 6.4 months (range, 0.7 to 24.9 months) after collagen implant or skin test exposure or both. Eight of the nine patients had a delayed-type hypersensitivity response at the test or treatment sites or both, and five of six patients tested were found to have increased serum antibodies to collagen. Compared with the general population, the incidence of dermatomyositis or polymyositis among collagen-treated patients was statistically increased (standardized incidence ratio, 5.05; 95% CI, 2.31 to 9.59; P < 0.0001). A similar analysis of the eight dermatomyositis case patients produced a standardized incidence ratio of 18.8 (CI, 8.1 to 37.0; P < 0.0001). Using a Monte Carlo simulation, an interval of 6.4 months or less from exposure to onset of disease was found to be an extremely rare event, occurring less than 72 times per one million simulation trials (CI, 57 to 91). CONCLUSIONS: Because these data suggest that an immunologic response to bovine type I or type III collagen or both caused this dermatomyositis or polymyositis-like syndrome, the risks versus benefits for the cosmetic use of collagen implants should be reassessed.

Adult↗
PubMed1975

Dermatomyositis in six patients without initial muscle involvement.

Six patients initially had the characteristic skin eruption of dermatomyositis but no other evidence of the disease. They all later developed dermatomyositis. The clinical, laboratory, and pathological data suggest that whereas polymyositis may occur without skin involvement, the cutaneous features od dermatomyositis may be seen in the absence of detectable muscle disease. This variant of dermatomyositis occurs in children and adults but has not been found to be associated with internal malignant disease. The persistence of skin lesions despite systemic corticosteroid therapy is a poor prognostic sign. There were two patients with pulmonary fibrosis and dermatomyositis in this group. The presence of pulmonary fibrosis can contribute to a delay in diagnosis of dermatomyositis. Patients with pulmonary fibrosis had a poorer prognosis, despite moderate dosages of corticosteroids.

Adolescent↗
PubMed1984

Familial canine dermatomyositis. Initial characterization of the cutaneous and muscular lesions.

Familial canine dermatomyositis is a recently identified disease of collie dogs that resembles human juvenile dermatomyositis. The lesions in the skin and muscles obtained by biopsy from two litters of dogs were characterized for the purpose of determining the similarity of the lesions to those of human dermatomyositis. The cutaneous lesions began between 7 and 11 weeks of age and were present on the face, lips, ears, and skin over bony prominences of the limbs, feet, sternum, and tip of the tail. Histologically the cutaneous lesions frequently consisted of vesicles, pustules, and ulcers on the lips, face, and ears. Neutrophils, lymphocytes, mast cells, and macrophages were present throughout the dermis. Neutrophils and lymphocytes were also present in and around vessels. Between 13 and 19 weeks of age generalized muscle atrophy was noted. The muscle lesions consisted of interstitial lymphocyte, plasma cell, macrophage, and neutrophil accumulation; myofiber degeneration, regeneration, and atrophy; and fibrosis. Perivascular neutrophils, lymphocytes, and plasma cells were also seen. Histologically, the lesions resembled those present in human juvenile dermatomyositis; and these observations, coupled with clinical, immunologic, and clinical pathologic observations presented elsewhere, suggest that familial canine dermatomyositis is an appropriate and potentially useful model for human juvenile dermatomyositis.

Animals↗
PubMed1984

Gynecologic carcinoma associated with dermatomyositis-polymyositis.

Dermatomyositis-polymyositis was associated with gynecologic carcinoma in ten patients treated at the Mayo Clinic between 1952 and 1982. Only two patients did not manifest the typical skin changes of acute dermatomyositis. In all ten cases, the onset of dermatomyositis-polymyositis preceded evidence of carcinoma. The intervals between initial symptoms of dermatomyositis-polymyositis and subsequent diagnosis of carcinoma ranged from three months to six years. Usually, the carcinoma became evident within two years. The carcinomas were ovarian in five cases, cervical in three, endometrial in one, and vaginal in one. Ages of the ten patients ranged from 40 to 66 years (mean, 53 years). In most cases, symptoms of dermatomyositis-polymyositis regressed markedly after diagnosis and treatment of the underlying carcinoma. In selected patients for whom more detailed follow-up information was available, the manifestations of dermatomyositis-polymyositis progressed and regressed in synchrony with tumor spread and effective treatment, respectively.

Adenocarcinoma↗
PubMed1994

Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed.

OBJECTIVE: To characterize scalp involvement in patients with dermatomyositis. DESIGN: Case series. PATIENTS: All patients with dermatomyositis seen in our office between 1988 and mid 1993. Patient inclusion in this study required fulfillment of three or more of Bohan and Peter's criteria for dermatomyositis. RESULTS: Of 17 patients with the diagnosis of dermatomyositis, scalp involvement was present in 14. Five of the 14 patients with scalp involvement were diagnosed as having scalp psoriasis or seborrheic dermatitis before progression of their disease or tissue examination revealed the diagnosis of dermatomyositis. In all patients, the scalp involvement was manifested as atrophic, erythematous, scaly plaques. In addition, alopecia was noted in six of the 14 patients. Treatment of cutaneous involvement included sun avoidance, topical corticosteroids and/or antimalarials, and/or methotrexate. CONCLUSIONS: Recognition of this process is important because scalp involvement is often overlooked, may be misdiagnosed initially, and can be the presenting complaint in some patients with dermatomyositis.

Adolescent↗
PubMed1996

[Dermatomyositis complicated by sarcoidosis].

We encountered a patient with dermatomyositis complicated by sarcoidosis. A 57-year-old woman was admitted to our hospital because of fever dry cough, and myalgias. There were reticular shadows on her chest X-ray film. Although the typical skin rash and myositis suggested the diagnosis of dermatomyositis biopsy specimens from a salivary gland, muscle, and lung revealed noncaseating granulomas as well. Uveitis was also noted. These findings suggested the coexistence of sarcoidosis with dermatomyositis. Examination of the lung-biopsy specimens showed interstitial pneumonia compatible with dermatomyositis, except for the granuloma. The typical rash of dermatomyositis and pathological findings of the lung specimen were inconsistent with sarcoidosis. Therefore we concluded that this patient had both dermatomyositis and sarcoidosis. This case sheds new light on the importance of pathological examinations.

Dermatomyositis↗
PubMed1996

[Prognostic factors and predictive signs of malignancy in adult dermatomyositis].

INTRODUCTION: Prognosis in dermatomyositis is severe, partly due to the development of cancer. The aim of this study was to identify factors predicting cancer development and assess factors predicting reduced survival rate. PATIENTS AND METHODS: A retrospective analysis of 32 cases of dermatomyositis diagnosed on the basis of the Bohan and Peter criteria was performed. Diagnosis was certain in 7 cases, probable in 13 and possible in 5. There were thus 7 cases of pure cutaneous dermatomyositis. Clinical and laboratory data were compared between patients with and without cancer and between deceased and surviving patients. RESULTS: Overall mortality was 37.5% at 4 years, confirming the gravity of dermatomyositis. Malignancy developed in 9 patients (28.1%) leading to death in all cases, within 18 months in 8. Amyopathic dermatomyositis was observed in 2 of these patients. Necrotic skin ulcerations (p < 0.01) and pruritus (p < 0.05) were significant predictive factors for the development of cancer. Poor prognosis factors were malignancy (p < 0.001), necrotic skin ulcerations (p < 0.01), and pruritus (p < 0.05). CONCLUSION: Prognosis is poor in certain sub-groups of patients with dermatomyositis. Such patients can be identified on the basis of skin lesions, notable necrotic ulcerations.

Adult↗