Classic Dermatomyositis Research Articles

Topical tacrolimus 0.1% ointment for refractory skin disease in dermatomyositis: a pilot study

BACKGROUND: Dermatomyositis is an inflammatory disease primarily involving the striated muscles and skin. Muscle disease usually responds to aggressive therapy with systemic corticosteroids. However, cutaneous lesions can be very resistant to systemic and topical therapies, even in combination. More treatment options are needed. Tacrolimus is an immunomodulator now available in a topical ointment.OBJECTIVE: To study the treatment of patients with refractory cutaneous lesions of dermatomyositis using topical tacrolimus 0.1% ointment.METHODS: Six patients with recalcitrant cutaneous lesions of dermatomyositis were included in this brief observational study: five adults and one pediatric patient. Five patients had classic dermatomyositis and one had dermatomyositis sine myositis.RESULTS: All had some degree of improvement of their dermatologic disease following 6–8 weeks of treatment with topical tacrolimus 0.1% ointment. Two had dramatic responses (>90% improvement), one had moderate (40–90%) improvement and three had minimal (20–40%) improvement.CONCLUSIONS: The dermatologic manifestations of dermatomyositis can be very difficult to treat. Multiple systemic and topical therapies have been studied. Combinations of treatments are often used, sometimes still not successfully. The results of this brief observational study using topical tacrolimus ointment for the treatment of refractory cutaneous lesions of dermatomyositis are encouraging. Topical tacrolimus was a useful adjunct in the treatment of the dermatologic component of dermatomyositis in adults and children in this pilot study.

Amyopathic dermatomyositis: Retrospective review of 37 cases

Criteria for diagnosis of amyopathic dermatomyositis vary, and the prognosis is not clear. Our purpose was to investigate prognosis regarding progression to myositis and associated malignancy. We reviewed the medical records of patients with dermatomyositis evaluated at our institution from 1976 to 1994. Of 746 patients with dermatomyositis, 37 (5%) with the amyopathic subtype were divided into 3 groups: group 1 (73%), no subjective or objective evidence of myopathy; group 2 (13%), no subjective muscle weakness but abnormalities detected by objective tests; group 3 (13%), subjective muscle weakness but no objective evidence of myopathy. Follow-up was conducted by means of a mailed questionnaire. For 25 patients, follow-up of 1 to 17 years after diagnosis showed muscle weakness in 2 patients in group 1 within 5 years after diagnosis. Five patients (13%) had associated malignancies. Of 7 (19%) patients with disease onset before the age of 18 years, none had progression to myopathy. Although it presents with cutaneous lesions indistinguishable from those of classic dermatomyositis, amyopathic dermatomyositis is a distinct entity. In most patients, amyopathic dermatomyositis does not progress to myopathy. Prognosis appears favorable, but malignancy may develop. (J Am Acad Dermatol 2002;46:560-5.)

Dermatomyositis sine myositis and antisynthetase syndrome

We describe a 66-year-old woman with cutaneous lesions typical of classic dermatomyositis, clinical evidence of antisynthetase syndrome (arthritis, Raynaud’s phenomenon, mechanic’s hands and interstitial lung disease with anti-Jo-1 autoantibody) and lack of muscle disease after a full muscle evaluation that included clinical, enzymatic, electromyographic, magnetic resonance imaging and histological studies. The patient did not develop myositis after 9 years of clinical disease. The association of dermatomyositis sine myositis with antisynthetase antibodies suggests that the characteristic skin lesions are closely linked with dermatomyositis on the basis of the similar clinical and serological features of both dermatomyositis sine myositis and classic dermatomyositis.

Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.

To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis. Retrospective study. University hospitals. Thirteen patients with amyopathic dermatomyositis. The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one. Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.

Dermatomyositis and cutaneous necrosis: report of five cases

We report on five patients with dermatomyositis (DM) and cutaneous necrosis. Patients presented with classic DM skin eruptions, mild myositis, and a high incidence (4/5) of interstitial pneumonia. Cutaneous necrosis developed independently of steroid therapy, with the majority of lesions being cured following several months of sterilization treatment. In addition, one patient with accompanying cancer presented with multiple necrotic lesions. Topical treatment using gentiana violet against local infection was considered to have been essential in accelerating healing.

Cutaneous features of classic dermatomyositis and amyopathic dermatomyositis

This review will focus on the work that has been reported over the past year attempting to better characterize the overall clinical significance of the cutaneous manifestations of dermatomyositis (DM). It will be organized under two headings--amyopathic dermatomyositis (ADM) and classic dermatomyositis (CDM). The distinction between these two clinical phenotypes of DM is the absence of clinically-evident muscle disease in ADM; to date it has not been possible to distinguish the cutaneous manifestations of ADM and CDM. The term hypomyopathic DM is introduced to distinguish those patients with the hallmark skin changes of DM and no clinical evidence of muscle disease who are found to have subclinical evidence of myositis upon electrophysiologic, histopathologic, and/or radiologic evaluation. Recent issues that have been raised concerning the definition, classification, epidemiology, etiopathogenesis, clinical features, treatment, differential diagnosis, and prognosis of ADM and CDM are discussed. In view of the irrefutable evidence documenting the existence of ADM for 20 years or more, the term idiopathic inflammatory dermato-myopathies may be more appropriate for this group of clinical disorders than the currently used designation, idiopathic inflammatory myopathies.

Rheumatologic manifestations of malignancy.

The association of rheumatic syndromes and malignancy is highlighted in this review. The prevalence of malignancy in a series of patients with unclassified rheumatic syndromes is reported. The spectrum of arthropathies associated with malignancy includes bilateral knee effusions, sacroiliitis, and adult-onset Still's disease. There are further reports on the well-recognized association between dermatomyositis and malignancy. The importance of screening for malignancy in patients with classic dermatomyositis as well as dermatomyositis sine myositis is highlighted. The association of mixed cryoglobulinemia with hepatitis C virus infection, hepatocellular carcinoma, and non-Hodgkin's lymphoma is discussed. Finally, the association of miscellaneous rheumatic features such as autoantibodies, vasculitis, carpal tunnel syndrome, and multicentric reticulohistiocytosis with malignancy is described.

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium‐201 scanning

AbstractBackground It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis.Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes.Methods In this single blind study we used thallium‐201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined.Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported.Conclusions Non‐invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.

Dermatomyositis with normal muscle enzymes: a single blind study of the diagnostic value of thallium-201 scanning

Background It is well documented that the classic cutaneous lesions of dermatomyositis can precede the onset of muscle involvement or may occur in the absence of any detectable muscle disease by current diagnostic criteria. Thallium scanning has been reported to be a useful test in the evaluation of muscle disease in patients with dermatomyositis.Objective Our purpose was to examine the utility of thallium scanning in patients with dermatomyositis with normal muscle enzymes.Methods In this single blind study we used thallium-201 scanning to evaluate 5 patients who presented with classical clinicopathologic dermatomyositis but with normal levels of serum muscle enzymes. A positive control patient with active myositis and a negative control patient were also examined.Results All patients had muscle groups with decreased signal intensity on thallium scanning. Even the negative control patient demonstrated marked asymmetry of several different muscle groups. These results are very different from those previously reported.Conclusions Non-invasive radiologic examinations clearly have a role in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. We have previously reported that magnetic resonance imaging and ultrasound are useful adjunctive tests. However, thallium scanning does not seem to be useful in the evaluation of patients with dermatomyositis with normal muscle enzymes. Thallium scanning appears to be a very sensitive test. However, the abnormal findings on every patient, including the negative control patient, suggest a very low specificity.

Dermatomyositis without muscle weakness. Long-term follow-up of 12 patients without systemic corticosteroids

We retrospectively studied 12 patients with dermatomyositis (DM) with at least one laboratory criterion of muscle involvement but without muscle weakness. We reported their outcome without systemic corticosteroid treatment and searched for distinctive baseline data and associated neoplasia among a group of 29 patients with classic DM. After a mean follow-up of 5.3 years, complete or partial skin improvement occurred in nine patients with DM without muscle weakness, and muscle enzyme levels were normalized in all. When compared with the classic DM group, no statistical differences were found for associated neoplasia or for any baseline data studied except, as expected, for muscle parameters. The lack of distinctive baseline data between DM without muscle weakness and classic DM suggests that there is a continuum from amyopathic to classic DM. However, DM without muscle weakness is a clinical entity with respect to favorable long-term prognosis. Unless frank muscle weakness develops, treatment with systemic corticosteroids can be avoided.

Amyopathic dermatomyositis (dermatomyositis sine myositis) and cutaneous dermatomyositis with mild muscle disease

We report on one patient suffering from amyopathic dermatomyositis, and two patients with transient or mild muscle involvement, respectively. The rare subset of amyopathic dermatomyositis is characterized by benign clinical course and prognosis. Leading cutaneous symptoms are heliotropic rash, periorbital violaceous-red erythema and edema, erythematous lichenoid plaques at the dorsum of the hands and fingers, hyperkeratotic, hyperesthetic eponychium, nailfold capillary abnormalities and the skin histology, while itching and photosensitiviry are optional features. On the other hand, clinical or laboratory evidence of muscle involvement is absent for at least 2 years. The two patients with transient or mild muscle involvement point to transitional forms between amyopathic and classical dermatomyositis. As differential diagnosis lupus erythematosus, (photo)contact eczema, lichen planus, psoriasis, seborrhoic dermatitis and atopic dermatitis, as well as polymorphic light eruption and erythroderma, should be considered. Association with internal malignancy is rare, but is to be excluded. Waiting, hydroxychlorochine and systemic glucocorticoids are the steps of treatment, depending on the clinical course.

Amyopathic dermatomyositis (dermatomyositis sine myositis) and cutaneous dermatomyositis with mild muscle disease

AbstractWe report on one patient suffering from amyopathic dermatomyositis, and two patients with transient or mild muscle involvement, respectively. The rare subset of amyopathic dermatomyositis is characterized by benign clinical course and prognosis. Leading cutaneous symptoms are heliotropic rash, periorbital violaceous‐red erythema and edema, erythematous lichenoid plaques at the dorsum of the hands and fingers, hyperkeratotic, hyperesthetic eponychium, nailfold capillary abnormalities and the skin histology, while itching and photosensitivity are optional features. On the other hand, clinical or laboratory evidence of muscle involvement is absent for at least 2 years. The two patients with transient or mild muscle involvement point to transitional forms between amyopathic and classical dermatomyositis. As differential diagnosis lupus erythematosus, (photo)contact eczema, lichen planus, psoriasis, seborrhoic dermatitis and atopic dermatitis, as well as polymorphic light eruption and erythroderma, should be considered. Association with internal malignancy is rare, but is to be excluded. Waiting, hydroxychlorochine and systemic glucocorticoids are the steps of treatment, depending on the clinical course.

Methotrexate for Dermatomyositis

Dermatomyositis has traditionally been treated with high doses of systemic corticosteroids. In recent years, methotrexate has been used as a steroid-sparing agent. These authors describe the efficacy and adverse effects of methotrexate in 10 adults treated over an 18-year period. Seven patients had classic dermatomyositis and 3 had dermatomyositis sine myositis. Nine patients were treated weekly with low doses of oral methotrexate and one with intravenous methotrexate. All …

Use of magnetic resonance imaging and P-31 magnetic resonance spectroscopy to detect and quantify muscle dysfunction in the amyopathic and myopathic variants of dermatomyositis.

To investigate the use of magnetic resonance imaging (MRI) and P-31 magnetic resonance spectroscopy (MRS) in characterizing the metabolic and functional status of muscles in patients with amyopathic dermatomyositis (DM) and to compare the findings with those in patients with classic myopathic DM. Nine patients with amyopathic DM, 11 patients with myopathic DM, and 11 normal individuals were studied. MRI images of thigh muscles were obtained, and T1 and T2 relaxation times were calculated. Biochemical status was quantitated with P-31 MRS, by determining concentrations of phosphate metabolites during rest and exercise. Patients with amyopathic DM showed no muscle inflammation, and MRS data obtained during rest were normal. During exercise at 25% and 50% maximum voluntary contractile force, the MRS data revealed significant differences between amyopathic DM patients and control subjects indicating inefficient metabolism. In contrast, muscles of patients with myopathic DM showed inflammation and metabolic abnormalities even during rest. Metabolic deficiencies in patients with amyopathic DM were unmasked by exercise, suggesting that the 2 DM syndromes may share muscle abnormalities. MRI/MRS may be useful in diagnosis and optimization of treatment.

Dermatomyositis with normal muscle enzyme concentrations. A single-blind study of the diagnostic value of magnetic resonance imaging and ultrasound

It is well documented that the cutaneous lesions of dermatomyositis may precede clinical myositis or may occur in the absence of any muscle disease detectable by current diagnostic criteria. In this single-blind study, we used magnetic resonance imaging (MRI) and ultrasound to evaluate five patients who presented with classical clinicopathologic dermatomyositis, but with normal levels of serum muscle enzymes. This patients group has not been previously studied with these techniques. Patients who served as positive and negative control subjects were also examined. Ultrasonography revealed hyperechogenicity, and MRI revealed high signals on T2-weighted images in several muscle groups of the patient with active myositis (positive control). Increased echogenicity was also noted in the deltoid region of one patient who had previously had a normal muscle biopsy finding. In the same patient, MRI revealed inflammatory changes in the lumbar paraspinal muscles. Another patient, with all previous study results being normal, had MRI evidence of T2 high signals in the gluteus minimus. Noninvasive examinations such as MRI and ultrasound are beneficial as adjunctive means of examination in the evaluation of patients with dermatomyositis sine myositis or dermatomyositis. Future studies may suggest additional uses for these tests, including serial evaluation of patients, noninvasive confirmation of diagnosis in pediatric patients, or in directing muscle biopsy, thus increasing sensitivity. Ultrasound appears to be the more cost-effective and simple test; but MRI, although more expensive, may be more sensitive and specific.

Mixed Connective Tissue Disease in Childhood

Rheumatic diseases with overlapping clinical features have been known since at least 1900 under such descriptive designations as "sclerodermatomyositis," "lupoderma," "rupus," and others. These hybrid descriptors recognize the occasional mixing of features of the classic rheumatic disorders, rheumatoid arthritis, scleroderma, dermatomyositis, and systemic lupus erythematous (SLE), in the same patient. However, because of the absence of precise pathogenetic and etiologic information, even the traditional rheumatic diseases have been largely classified on the basis of aggregations of clinical, histologic, and immunologic findings. In 1972, Sharp and colleagues first described 26 adults with a new syndrome, which they defined as mixed connective tissue disease (MCTD). A major contribution of Sharp and his colleagues was to name and attempt to classify an overlap syndrome in explicit clinical terms and to associate it with specific autoantibodies directed against "extractable nuclear antigen" (ENA). One year later, the first child with MCTD was reported, and in 1977 a series of 14 children with MCTD was first investigated. Where does mixed connective tissue disease, in both children and adults, stand now after more than a decade of study? Directly, the concept of MCTD has focused our attention upon the description of overlapping features of the rheumatic diseases, and it has forced us to confront whether "lumping" or "splitting" descriptors serves the patient from a therapeutic or prognostic point of view.

Mixed connective tissue disease in childhood.

Rheumatic diseases with overlapping clinical features have been known since at least 1900 under such descriptive designations as sclerodermatomyositis, lupoderma, rupus, and others. These hybrid descriptors recognize the occasional mixing of features of the classic rheumatic disorders, rheumatoid arthritis, scleroderma, dermatomyositis, and systemic lupus erythematous (SLE), in the same patient. However, because of the absence of precise pathogenetic and etiologic information, even the traditional rheumatic diseases have been largely classified on the basis of aggregations of clinical, histologic, and immunologic findings. In 1972, Sharp and colleagues first described 26 adults with a new syndrome, which they defined as mixed connective tissue disease (MCTD). A major contribution of Sharp and his colleagues was to name and attempt to classify an overlap syndrome in explicit clinical terms and to associate it with specific autoantibodies directed against extractable nuclear antigen (ENA). One year later, the first child with MCTD was reported, and in 1977 a series of 14 children with MCTD was first investigated. Where does mixed connective tissue disease, in both children and adults, stand now after more than a decade of study? Directly, the concept of MCTD has focused our attention upon the description of overlapping features of the rheumatic diseases, and it has forced us to confront whether lumping or splitting descriptors serves the patient from a therapeutic or prognostic point of view.

Simple method for measurement of lower extremity muscle strength

A simple, rapid, reproducible method for quantification of lower extremity muscle strength was standardized. The time needed to stand 10 times from a standard chair was recorded in 139 healthy subjects, aged 20 to 85 years (77 men, 62 women). Reproducibility was 6.8 percent (±3.4 percent). Neither height nor weight was related to time in either sex. Weight was related to time (p <0.05) after adjusting for age, but this effect was slight compared with the effect of age alone. A highly significant (p <0.0001) relationship between time and age was found in both sexes. Younger men performed better than younger women, although this difference was lost in the older age groups. The results of this simple test correlated well with published data on the strength of knee flexor and extensor muscles in groups of men and women of various ages. This method was used to evaluate serially six consecutive patients with classic polymyosltis or dermatomyositis. Improvement after treatment with prednisone used alone or in combination with azathioprine or methotrexate was found in all cases.

Juvenile dermatomyositis: A clinical and immunologic study

Twenty-one children were diagnosed as having juvenile dermatomyositis on the basis of the strict criteria of Bohan and Peter. In addition to the typical skin and muscle changes, abnormalities of esophageal motility (eight of 19), pulmonary function (14 of 17), ECG (10 of 20), and gastrointestinal absorption of D-xylose (two of eight) with active disease were observed. Clinical signs of other collagen vascular disease appeared in five children. Serologic evaluation demonstrated that ANA and rheumatoid factor were transiently positive in six; one child developed a persistently positive rheumatoid factor after four years of disease inactivity. Antibody to ENA was negative in all, but antibody to PM-1 antigen was present in four of 18. Six had a low C3 or C4; evidence of immune complexes was demonstrated by Clq or Raji binding in eight with active disease. One child was IgA deficient. The HLA-B8 antigen was present in 72% of the Caucasian children as compared with the expected incidence of 21%. Therefore, classical dermatomyositis in children has more systemic involvement then previously appreciated, may be related to the presence of circulating immune complexes, and appears to be under immunogenetic control.

Fatal pulmonary involvement in dermatomyositis.

A 12-year-old boy with classic dermatomyositis developed a rapidly progressive pulmonary process that led to his death a month later. Physiologically, there was interference with the oxygenation of the blood even when 100% oxygen was delivered to the lungs under pressure. Microscopic examination of the lungs disclosed diffuse interstitial pulmonary fibrosis. We conclude that these pulmonary changes were a component of the basic disease.