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Few cases of dermatomyositis affecting pregnancy have been reported. In this case the fetal outcome was good, but postpartum, pregnancy-induced hypertension was associated with acute exacerbation of the disease and led to maternal death. Both the fetal and maternal outcome differed from those in previously reported cases.
Skin biopsies from patients with scleroderma and juvenile dermatomyositis (DM) share many histologic features. Characteristics common to both diseases are particularly evident in the dermal microvasculature and include endothelial swelling and concentric thickening of the vascular basement membrane. Biopsies performed on 3 patients with the severe vasculitic form of juvenile DM showed these changes as well as dropout of vessels and linear deposition of collagen. The latter findings, seen late in the course of the disease, are indistinguishable from those of advanced scleroderma. A hypothesis is presented which attempts to relate these histological findings to a common underlying pathophysiologic mechanism.
Patients with childhood dermatomyositis are generally categorized into one of 3 groups: limited or monocyclic, polycyclic, or continuous. Disease resolution in those with monocyclic disease after a period of 1 to 2 years is expected and those patients are not considered at life-long risk for connective tissue disease. We describe a patient, with an uncomplicated initial course, who had disease recurrence after 8 years of disease inactivity and almost 4 years after discontinuation of all medication.
Two girls, one with progressive systemic sclerosis and a second with dermatomyositis, developed calcinosis in the skin of the prepatellar area. Calcinosis was accompanied by local inflammation and skin ulceration. Oral colchicine therapy in a dosage of 1 mg per day was followed within two months by significant regression of local inflammation and healing of the skin ulcers.
The clinical and serologic features of 36 patients with polymyositis (PM) or dermatomyositis (DM) were observed over a 5-year period. The mean age of the patients at the time of diagnosis was 48.5 years, and 61% were female. According to widely accepted diagnostic criteria 50% had PM (group I), 14% DM (group II), 11% PM or DM associated with malignant disease (group III) and 25% PM or DM associated with a connective tissue disorder (group V). None of the patients had childhood PM or DM associated with vasculitis (group IV). All the patients had muscle weakness, and 94% of the patients tested had an elevated serum level of creatine kinase. The average delay from the onset of symptoms to diagnosis was 14 months overall but only 2.3 months for the DM patients. Of the 30 patients whose serum was tested, 73% had antinuclear antibodies, with antibodies to nuclear ribonucleoprotein being most common in group V patients and antibodies directed against the Jo-1 antigen being restricted to patients with PM alone (group I).
Seventy-one patients with polymyositis-dermatomyositis (PM/DM) admitted to the Wellesley Hospital Rheumatic Disease Unit (RDU) in Toronto between 1965 and 1980 were followed up to 1981. The frequencies of malignant neoplasms occurring prior to or concurrent with initial RDU admission were compared, using case-control methods, with age- and sex-matched control groups with a diagnosis of any non-PM/DM rheumatic disease (rheumatic disease controls) or osteoarthritis, fibrositis, or fracture (noninflammatory musculoskeletal controls). In a cohort analysis, the incidence of malignant neoplasm subsequent to initial RDU admission in patients with PM/DM was compared with the expected incidence in the Canadian population. Fifteen of 71 patients with PM/DM had an antecedent or concurrent cancer compared with four of 71 rheumatic disease controls and one of 71 noninflammatory musculoskeletal controls. Cohort analysis showed no increase in the number of subsequent malignant neoplasms in patients with PM/DM compared with the age- and sex-matched Canadian population.
Laboratory studies were performed on 3 Collies with familial canine dermatomyositis, 6 progeny from a breeding of 2 of the Collies (incross litter), and 4 progeny from the breeding of an affected Collie male and a normal Labrador Retriever (outcross litter). Hematologic abnormalities were leukogram changes consistent with inflammation, and, in 2 severely affected incross dogs, anemia of inflammatory disease. Serum muscle enzyme activities were not markedly increased. High concentrations of immune complexes and proportionally increased total immunoglobulin G were present in the sera of moderately and severely affected incross dogs. The same dogs had weakly positive direct Coombs' tests, and 1 affected outcross dog had a strongly positive direct Coombs' test. Rheumatoid factor was present in a severely affected incross dog. A few dogs had polyclonal globulin increases that were attributed to inflammation. Low antibody titers to canine calicivirus were present in 3 dogs and a low titer to canine coronavirus was present in 1 dog.
Dermatomyositis and polymyositis are rare disorders of connective tissue that manifest themselves primarily as muscular weakness. Fifty-five percent of 45 patients with this diagnosis at Thomas Jefferson University Hospital had symptoms referable to the head and neck. While dysphagia and the cutaneous manifestation predominate, such symptoms as facial swelling and oral mucosal disorders may require the treating physician to seek the advice of an otolaryngologist before the patient suffers any muscular changes. A thorough knowledge of this disease entity is essential to specialists in head and neck diseases.
Because a seven year old boy with severe dermatomyositis failed to respond to a six week treatment course with prednisone (60 mg/m2) and azathioprine (2 mg/kg/d), five plasma separations were performed. Already after the second plasma separation an impressive reduction of skin ulcerations and an increase of muscle strength were noticed. So far there has been no relapse in spite of rapid reduction in the prednisone dosage.
Measurements of maximum respiratory pressures and routine pulmonary function tests were performed in 8 patients with polymyositis (PM) and 2 patients with dermatomyositis (DM). Serial measurements of routine pulmonary function tests in 8 patients remained unchanged. Maximum respiratory pressures were decreased initially in 7 patients with proximal muscle weakness and clinically active muscle disease and improved with corticosteroid therapy in the 5 patients who were followed serially. In 3 patients with clinically stable disease the maximum respiratory muscle power was normal. Serial measurements of maximum respiratory pressures have been of value in monitoring patients with PM and DM.
We report 2 cases of trichinosis in which the clinical features resembled dermatomyositis. In both cases, however, the diagnosis was established histologically by demonstrating trichina larvae in the patients' muscle biopsies. Indirect immunofluorescence and counterimmunoelectrophoresis techniques were used to confirm the diagnosis serologically.
Thirteen cases of dermatomyositis seen in children are described. Records of hospitals serving 250,000 children were reviewed for the 10-year period during which these children were seen and no other cases were found. Eleven of the 13 are living, including seven believed to be in complete remission; the remainder have ongoing disease of varying severity. Literature concerning this unusual childhood disease is reviewed and there is general agreement as to the characteristic nature of the clinical picture. Special features, including rash, muscle findings, deformity, calcinosis and gastrointestinal bleeding, are discussed. Emphasis is placed on the value of steroid therapy, though some serious complications are acknowledged. Methotrexate, found promising by others, was without benefit in two cases.
Acanthosis nigricans of the groin, axillae, and neck developed in a patient on long-term steroid therapy for dermatomyositis after eight years. The dermatosis resolved spontaneously ten years after the steroid use had been discontinued. Review of three personal cases and the literature suggests that acanthosis nigricans may have a hormonal basis.
The ultrastructure of skeletal muscles was studied on 6 bioptates collected from children with the idiopathic form of dermatomyositis. The main type of the affection of muscle fibers was myolysis the intensity of which correlated with the degree of activity of the disease. In focal myolysis, signs of intracellular regeneration were found in fibers. Diffuse myolysis was characterized by retained hypertrophic nuclei and basal membranes of sarcolemma against the background of complete degeneration of myofibrillae and a sharp decrease in the fiber diameter. Considerable hyperplastic changes in myosatellitocytes were found, but with no regenerating fibers, however. The permanent features included hypertrophic and dystrophic changes in the vascular endothelium and pericytes. In the maximally active disease, destruction of capillaries and a decrease in their numbers were found. Microtubular structures and nuclear bodies were found in the endothelium, pericytes, and less frequently in other cells.
A 15-year-old girl suffering from acute dermatomyositis was treated with cyclosporin A after conventional therapy had failed. During this treatment there was a marked decrease of the myolysis and improvement of the paresis.
Typing for HLA-A and -B antigens was performed on 87 children with definite juvenile dermatomyositis (JDMS). A significantly increased frequency of HLA-B8 (estimated relative risk = 2.8, Pc less than 0.01) was observed among White patients, but not among Blacks or Latin Americans with JDMS. No abnormality of HLA haplotype segregation was observed among 38 healthy siblings of the JDMS probands.
Thirteen children with systemic lupus erythematosus (SLE) and 5 with juvenile dermatomyositis (JDM) were studied for natural cytotoxicity (NK) and antibody-dependent cellular cytotoxicity (ADCC). In vitro prednisolone at pharmacological levels failed to alter NK or ADCC in adult controls, nor did it alter ADCC in any children studied. ADCC was normal, but NK was decreased in SLE (p less than .005) and JDM (p less than .05) compared to controls. Since NK but not ADCC is decreased in these patients, different lymphocyte populations may mediate these 2 cytotoxic functions.
A case of dermatomyositis (DM) with only one abnormal muscle function, bilateral diaphragmatic paresis, is reported. The coexistence of interstitial lung disease allowed us to sample the diaphragm during an open lung biopsy. Characteristic features of myositis were found. Lung volumes, compliance, maximal inspiratory pressure, and transdiaphragmatic pressure gradient (delta Pdi) were lower than normal. Lung recoil pressure was normal. Reversible small airways obstruction was also found. Treatment with prednisone produced dramatic improvement, with all functional values becoming normal except for compliance, which remained slightly decreased. A thorough evaluation of inspiratory muscle strength is advised in all patients with DM in order to identify varying degrees of altered diaphragmatic function.