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Necrotic lesions of the scrotum usually result from infectious processes, vascular occlusion or trauma. The first case of necrotic non-purulent skin changes associated with dermatomyositis is reported.
Dermatomyositis is a classic example of a disease that has both cutaneous and systemic manifestations. The skin and muscle disease are described as well as the possible systemic manifestations including overlap syndromes, joint symptoms, pulmonary disease, and other internal organ involvement. The association with pregnancy is also discussed.
Juvenile dermatomyositis is a chronic disease of childhood that is manifested by severe symmetrical progressive muscle weakness, a characteristic heliotrope colored skin rash which involves the face, and by elevated serum enzymes related to muscle damage.
Although microvascular injury has been postulated as the pathogenetic basis of skeletal muscle injury in dermatomyositis (DM), its role in the genesis of the skin lesions, which are said to be difficult to distinguish light microscopically from systemic lupus erythematosus (SLE) and subacute lupus erythematosus (SCLE), has not been analyzed. The authors' intention was to assess the role of microvascular injury in the pathogenesis of skin lesions in DM, SLE, and SCLE. Light microscopic features of biopsies of lesional skin from 20 patients with myopathic DM and 11 with amyopathic DM were compared to eight lesional skin biopsies from eight patients with SLE and 12 lesional skin biopsies from 12 patients with SCLE. Vascular density was compared in the three groups using an immunohistochemical preparation with an antibody to factor VIII. In 12 biopsies from the DM group, and in 19 of 20 lupus erythematosus (LE) specimens, frozen tissue was available. An indirect immunofluorescence methodology was used to detect C5b-9 deposition, and direct immunofluorescence studies for other immunoreactants were performed in standard fashion. Compared with LE, lesions of DM showed a greater degree of endothelial injury, vascular ectasia, and vascular fibrin deposition; there were no differences between myopathic versus amyopathic DM. C5b-9 deposition in vessels was significantly greater in DM than in LE. The superficial vascular plexus density was reduced in lesions of DM versus LE control groups with the greatest reduction observed in myopathic DM. Epithelial injury and mucin was greatest in myopathic DM. Microvascular injury is the apparent pathophysiological basis of skin lesions in DM. Careful attention to microvascular pathology enables distinction of DM from SLE and SCLE. Indirect immunofluorescence testing using a monoclonal antibody to C5b-9 is a valuable tool to distinguish DM from LE in biopsies of lesional skin.
OBJECTIVES: Dermatomyositis and polymyositis (DM/PM) are associated with neoplasms. The aim of the present study is to compare our experience in Israel with other published data. METHODS: Thirty-five adult patients with DM/PM, admitted to Sheba Medical Center during the 11-year interval between 1984 and 1994, were studied for the prevalence and features of malignant diseases. Patients with DM/PM alone and with DM/PM and malignancy were identified by using the hospital computer system. The manifestations of DM/PM and features of the malignant diseases were abstracted from the patients' charts. The presence or absence of malignancy and the type of cancer were verified in the National Cancer Registry. RESULTS: There were 15 men and 20 women. The mean age at the onset of the disease was 53 +/- 18 years. A total of 15 had PM and 20 DM. Malignancies occurred in four patients with PM (27%) and in nine with DM (45%) a frequency 12.6 times higher than in the general population. In six patients, the malignancy and the DM/PM were diagnosed simultaneously; in four before and in three after the appearance of the DM/PM. Hematologic, gastrointestinal, breast, ovarian, and lung tumors, malignant melanoma, and metastatic carcinoma of unknown primary were found among our patients. Eight DM/PM patients with malignancy died during the study period of infection, pulmonary embolism, and tumor spread. CONCLUSIONS: Our study found that DM/PM is associated with high rates of malignancy and mortality.
This case is the first reported presentation of obstructive anuria due to extrinsic ureteral compression in a patient with dermatomyositis. Further evaluation revealed disseminated sigmoid colon adenocarcinoma. Conservative treatment of ureteral obstruction by internal double-J stents achieved appropriate palliation.
Coxsackie-B-virus-specific probes prepared by reverse transcription of purified virus genomic RNA and molecular cloning techniques were used in quantitative slot-blot hybridisations to test for the presence of virus RNA in skeletal muscle biopsy samples. The samples tested were from two patients with adult polymyositis, seven with juvenile dermatomyositis, four with Duchenne muscular dystrophy, and six normal controls. Of the nine patients with inflammatory muscle disease, five were positive for Coxsackie-B-virus RNA; no viral sequences were found in the ten Duchenne muscular dystrophy and control biopsy samples.
Juvenile dermatomyositis in fourteen children who had not responded fully to steroids and other immunosuppressants and who had had chronic active disease for an average of 3 years was successfully treated with cyclosporin. Twelve patients had serious complications of the disease or of previous treatment. The response to cyclosporin included recovery of muscle strength and function and resolution of complications. It was possible to stop steroids or to reduce the steroid dose, which had previously been difficult, in all fourteen patients. In general, a low dose of cyclosporin (2.5-7.5 mg/kg daily) was sufficient and no serious side-effects were seen.
To examine the role of complement in certain autoimmune neuromuscular diseases, we used an in-vitro quantitative complement uptake assay that allows measurement of the capacity of patients' sera to deposit fragments of the third complement component onto sensitized targets. C3 uptake was significantly higher in patients with active dermatomyositis, Guillain-Barré syndrome and myasthenia gravis, compared to inclusion body myositis and controls. The in-vitro C3 uptake assay supports the role of C3b neoantigen and Membranolytic Attack Complex deposition in the target tissues and may be a useful tool to monitor disease activity in patients with complement-mediated neurological disorders.
Dermatomyositis (DM) has been linked to internal malignancy in adult patients. However, the value of an extensive malignancy evaluation in patients with DM is controversial. Fifty-seven patients who had DM with malignancies, in whom data were available regarding the discovery of malignancy, have been analyzed. Fifty-three of these were reported previously. There were sixty-seven malignancies in the fifty-seven patients. The malignancy preceded (26 cases), followed (23 cases), or occurred with the DM (18 cases). A "blind" (nondirected) malignancy search was not of value in any of the cases analyzed. Rather, the tumors were discovered in forty cases by history (preceding tumor, or abnormal symptoms), in fourteen cases by physical examination, or in twelve cases by abnormal laboratory findings (chest x-ray, urinalysis, stoll guaiac, etc). One case was not discovered until autopsy (adenocarcinoma of the broad ligament). Analysis of tumor sites further negates the value of a malignancy work-up, because most (greater than 90%) tumors occur in areas not amenable to a "routine malignancy search." In several instances patients had an extensive search, without having complete physical examinations. Malignancy evaluations should be directed by abnormal history, physical findings, or routine laboratory testing.
Dermatomyositis (DM) is a collagen vascular disease with prominent cutaneous findings. Although the myositis often responds to therapy with corticosteroids and/or immunosuppressives, the cutaneous disease may not respond. Seven patients with cutaneous lesions of DM that had not responded to therapy were treated with hydroxychloroquine in an open study. Three patients had idiopathic DM, one had DM without myositis, one had DM with malignancy, and one had adolescent DM. The response to the addition of hydroxychloroquine was good in all of the patients, and three had total resolution of their skin lesions. In two patients the corticosteroid dosage could be tapered. Therapy with hydroxychloroquine did not appear to have any beneficial effect on the myositis. We conclude that hydroxychloroquine may have a role as an adjuvant to the therapy of patients with cutaneous lesions of DM.
Many consider porcelain white atrophic papules as pathognomonic for malignant atrophic papulosis (MAP), or Degos' disease. During the past three decades, five patients with a collagen vascular disease have been reported to have MAP-like lesions as a manifestation of their underlying illness. We describe a patient with dermatomyositis who had porcelain-white atrophic papules resembling malignant atrophic papulosis.
Annually approximately 1:200,000 young children and adolescents are affected by juvenile dermatomyositis (JDM). Genetic factors are thought to contribute to the etiology. Since the discovery of the human leukocyte antigen class II associated DM molecule much has been learned about its role in the normal processing of HLA-class II molecules with a limited number of polymorphisms being found. Blood samples were collected from 30 patients who were seen in the clinic and 40 healthy volunteers. Exon 3 of the HLA-DM A and B genes were amplified and specific polymorphisms were identified given allele designations. The DMA*0103 allele was found in 43% of patient alleles versus 8% in the control group, this difference reached significance at a p value of 0.0004. The DMB*0102 allele was found in 20% of patients compared with 3% of the controls with a calculated p value of 0.037. Relative risk (RR) ratios with CI were as follows: DMA*0103 vs control RR = 5.7 and DMB*0102 vs control RR = 8. In conclusion, we feel that the polymorphisms represented in the DMA*0103 and the DMB*0102 alleles are increased in frequency in our JDM patients.
PURPOSE: Polymyositis (PM) and dermatomyositis (DM) are inflammatory idiopathic myopathies of dysimmune origin. Studies have shown two different pathogenic mechanisms: a primitive vascular mechanism mediated mainly by a humoral mechanism responsible for a muscular ischemia in juvenile DM; and a primitive injury of muscle cells mediated by a cytotoxic cellular mechanism, directed against the myofibrillae, in PM. CURRENT KNOWLEDGE AND KEY POINTS: Oral corticosteroids are the treatment of first choice in patients with PM/DM. This therapy has transformed the prognosis of these diseases. The efficacy of polyvalent human intravenous immunoglobulin (IVIG) was first described in an open study with severe refractory PM and DM, and confirmed by a controlled study in DM. FUTURE PROSPECTS AND PROJECTS: We present our experience about the interest of intravenous immunoglobulin (IVIG) in refractory PM and DM and discuss the different mechanisms of action of this new immunotherapy.
PURPOSE: Interstitial lung disease is one of the most common respiratory manifestations in polymyositis and dermatomyositis. It still remains a severe complication of the disease, leading to death related to ventilatory insufficiency in 30-66% of patients. CURRENT KNOWLEDGE AND KEY POINTS: Time onset of interstitial lung disease is variable, although interstitial lung disease onset precedes initial manifestations of polymyositis/dermatomyositis in roughly half of the patients. Moreover, clinical presentation of interstitial lung disease can be dichotomized, according to patients' pulmonary manifestations, into: 1) both acute and aggressive lung disease similar to Hamman-Rich syndrome; 2) slowly progressive lung disease; and 3) an asymptomatic pattern. The methods of choice adopted for early diagnosis of interstitial lung disease are high-resolution computed tomography scan and pulmonary function tests, which should be performed during both initial evaluation of polymyositis/dermatomyositis and follow-up. Because anti-JO1 antibody is considered to be a marker of interstitial lung disease in polymyositis/dermatomyositis, close pulmonary follow-up of anti-JO1-positive patients with polymyositis is therefore required for early detection of subclinical impairment. Furthermore, histological lung findings provide prognostic data; patients with bronchiolitis obliterans organizing pneumonia (BOOP) indeed appear to have a more favorable outcome than those with usual interstitial pneumonia or diffuse alveolar damage. Finally, as a guide to both the severity and progress of interstitial lung disease, the significance of other investigations, notably bronchoalveolar lavage, remains controversial. FUTURE PROSPECTS AND PROJECTS: Specific therapy of interstitial lung disease has not yet been clearly established in polymyositis/dermatomyositis patients. Corticosteroid therapy is considered the first line of therapy for polymyositis/dermatomyositis patients with interstitial lung disease. The association of cyclophosphamide and corticosteroids may be the most effective in patients with steroid-resistant interstitial lung disease. Early diagnosis and management of this disease is therefore of the utmost importance.
We report a case of peliosis hepatis in a 47-year old male patient with dermatomyositis treated with azathioprine and corticosteroids. Three months after the combined treatment was initiated, the patient developed right thoracic herpes zoster, agranulocytosis and liver enlargement with signs of portal hypertension. Needle biopsy of the liver revealed peliosis. Azathioprine was withdrawn. The clinical and laboratory abnormalities disappeared progressively. The main causes of peliosis hepatis are considered. Up to now, azathioprine had been held responsible for peliosis hepatis only in renal transplant recipients.
We report a case of a 35 year-old-man with dermatomyositis associated with tracheopathia osteoplastica. The swallowing perturbation secondary to myositis and airway involvement by tracheopathia induced fatal outcome. Tracheopathia osteoplastica is a rare disease and occurs exclusively in men over the age of 50. The association of two rarest disease is not a fortuitous event. The common pathogenic factor may be, in this case, the occupational exposure to silicon.
To our knowledge, the occurrence of extensive pharyngeal necrosis in patients with dermatomyositis has not yet been reported. We report such a case characterized by a vast ulceration of the posterior laryngeal wall, extending from the velum palati to the larynx and responsible for both dysphagia and dysphonia. Treatment with an immunosuppressant had no effect on the lesions.