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Hydroxyurea is a cytotoxic chemotherapeutic agent used for myelodysplasia. The adverse cutaneous effects due to hydroxyurea include leg ulcers, hyperpigmentation of the skin and nails, a lichen planus-like eruption, lupus erythematosus, and a dermatomyositis-like eruption. We present a case of hydroxyurea-induced dermatomyositis-like eruption and review the features of this entity as previously reported.
Serum levels of KL-6 were examined in 8 cases of juvenile dermatomyositis: 3 with interstitial lung disease (ILD) and 5 without ILD. The KL-6 levels were elevated in the ILD cases and correlated with the degree of computed tomography findings. The measurement of serum KL-6 levels is useful for evaluating juvenile dermatomyositis-associated ILD.
Milk of calcium developed in 2 children with juvenile dermatomyositis. The fluid of the collection contained macrophages, interleukin-6, IL-1, and tumor necrosis factor. The patient who had dystrophic calcinosis had a dramatic improvement with the introduction of alendronate. These findings suggest that calcinosis of juvenile dermatomyositis may be mediated by activated macrophages and that alendronate can be an effective treatment for this condition.
A case of autoimmune disease-associated lymphadenopathy (ADAL) in the lymph nodes linked to well documented dermatomyositis is presented. A 49-year-old Japanese woman was affected by a left axillary and a left inguinal lymphadenopathy at the onset of disease. A biopsy specimen taken from a left inguinal lymph node showed distortion of the nodal architecture and polymorphous lymphoid infiltration containing many plasma cells and plasmacytoid cells with scattered transformed lymphocytes, as well as a large number of capillaries. The polytypic nature of B-lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction. Epstein-Barr virus-associated lymphoplasmacytic infiltrates may occur in patients immunosuppressed by methotrexate administered for the treatment of dermatomyositis. However, Epstein-Barr virus-encoded small RNA-positive cells were not identified by in situ hybridization. Moreover, the patient had no history of methotrexate therapy.
We report a case of acute onset of dermatomyositis with rhabdomyolysis and myoglobinuria, which presented in the 14th week of pregnancy and resulted in spontaneous abortion of the fetus. The diagnostic work up for an underlying disease was negative and the histologic examination confirmed the diagnosis of dermatomyositis, which subsequently improved with corticosteroids.
The authors report a case of dermatomyositis (D.M) in a 57-year-old man, associated with a squamous cell bronchial carcinoma. The complete resolution of the dermatomyositis after radical resection of the bronchial tumor strongly suggested a paraneoplastic phenomenon.
A patient is described who had insulin-dependent diabetes mellitus for 2 years, prior to developing rheumatoid arthritis and then subsequently ankylosing spondylitis and dermatomyositis. Diagnostic criteria for all diseases are fulfilled. HLA typing revealed the presence of HLA A2, A9, B8, B27, DR3 and DR4 antigens. The concomitant coexistence of diabetes mellitus, rheumatoid arthritis, ankylosing spondylitis and dermatomyositis appears to have occurred in an individual genetically susceptible to these diseases.
Retinopathy has been described in dermatomyositis but visual loss is rare. We describe an unusual case of dermatomyositis where retinal complications resulted in permanent and profound central visual loss. Pulsed intravenous and oral methylprednisolone and cyclophosphamide therapy controlled the systemic disease but the macular and optic disc damage was irreversible. Early diagnosis and aggressive immunosuppressive therapy may help to prevent and control severe and potentially blinding intraocular inflammation. We emphasize the need for close co-operation between ophthalmologist and physician in the management of this condition.
We report a case of dermatomyositis associated with molecular evidence of parvovirus B19 DNA in two muscle biopsies collected 5 months apart. IgG- but not IgM-specific antibodies were detected in serum. None of four serum samples was positive for parvovirus B19 DNA. The two biopsies contained B19 VP1 sequences and the second one was also positive for NS1. This is the first report of viral parvovirus B19 DNA in muscle of a patient with dermatomyositis. Latent muscle infection may contribute to the clinical picture.
Central nervous system (CNS) complications are rarely reported in either juvenile or adult onset inflammatory myositides, such as dermatomyositis and polymyositis. We report two children, aged 4 and 10 yr respectively, with a diagnosis of juvenile dermatomyositis, both of whom subsequently developed clinical features of severe CNS involvement, possibly consistent with cerebral vasculopathy. One child died from apparent brainstem involvement; the other developed seizures, pseudoseizures and clinical depression which responded to aggressive immunosuppression. Although the vasculopathy or vasculitis underlying this disorder is known to have a systemic distribution, CNS involvement has rarely been reported and may be under-recognized.
Since idiopathic inflammatory myositis is relatively uncommon, randomized placebo controlled trials are rare. Although corticosteroids have not been tested in randomized controlled trials, general clinical consensus among physicians has accepted it as effective therapy. However, corticosteroid toxicity leads to significant disability in many patients. For patients with refractory dermatomyositis, intravenous immunoglobulin is an effective short-term treatment but its long-term effect remains unknown. Immunosuppressants are commonly used in refractory inflammatory myositis; evidence for their efficacy, with very few exceptions, has been derived from case reports and open studies with small numbers of patients. Even in randomized trials, the lack of validated and generally accepted outcome measures makes it difficult to compare the effect of interventions in different studies. Although the balance of evidence suggests that immunosuppressants are equally effective in dermatomyositis and polymyositis, there are no randomized controlled trials to show if any of these drugs, individually or in combination, is best. For uncommon diseases, such as inflammatory myositis, only multicentre randomized controlled trials involving rheumatologists and neurologists will define the optimal therapy.
Cutaneous manifestations of dermatomyositis (DM) commonly include Gottron's papules, cuticular erythema and telangiectasia, periorbital edema with a "heliotrope" rash, a papulosquamous eruption of the hairline, face, and trunk, as well as poikiloderma. Very few references can be found concerning vesicular and bullous lesions, however. We present two patients with dermatomyositis who manifested vesicular and bullous lesions who were initially misdiagnosed. Although rare, it is important to recognize this form of DM to avoid misdiagnosis. Furthermore, in some cases, vesiculo-bullous DM may portend a poor prognosis.
OBJECT: Gastrointestinal involvement in adult dermatomyositis (DM) and polymyositis (PM) is usually mild, resulting from myoenteric dismotility. Severe inflammation of the alimentary tract in cases of adult DM and PM is rare. The purpose of this study was to examine the prevalence and clinical characteristics of inflammatory gastrointestinal involvement in patients with DM. METHODS: The charts of all cases with polymyositis or dermatomyositis, registered in our rheumatology clinic between 1984 and 2004, were reviewed retrospectively for documentation of severe gastrointestinal involvement. The clinical course and the histopathologic findings in all the patients were noted, and the prevalence of this disorder was computed. RESULTS: Among 48 patients with DM or PM, 3 patients with DM and severe gastrointestinal tract manifestations were identified (6% of the study population). Edematous hyperemic bowel wall, with multiple erosions and ulcerous lesions were the prominent endoscopic findings, whereas diffuse mucosal inflammation and multiple vascular ectasias without vasculitis dominated the histologic picture. The resulting clinical course was notable for recurrent abdominal pain and bloody diarrhea, ending catastrophically in two patients with fatal gastrointestinal perforations, despite aggressive immunosuppressive therapy. CONCLUSIONS: Severe inflammatory gastrointestinal tract disease should be recognized as a grave, albeit rare, manifestation of adult DM that portends a poor prognosis and carries a high rate of fatal complications. The role of vasculopathy in the pathogenesis of this syndrome remains to be determined.
Dermatomyositis is associated with malignancy in approximately 20-25% of cases. The most common associated cancers are ovarian, lung, pancreatic, stomach, colon and non-Hodgkin's lymphoma. Nasopharyngeal cancer is not common in the Caucasian population; however, there is a much higher incidence in Asian patients. Radiotherapy is the mainstay of treatment for early nasopharyngeal cancer, but combination chemoradiotherapy is becoming more common for patients with advanced disease since the Intergroup trial 0099 demonstrated improved progression-free survival and overall survival for chemoradiotherapy. Increasingly, the cytotoxic agent amifostine is being used prior to radiotherapy in an attempt to decrease associated morbidities. Amifostine has been found to significantly decrease acute and chronic xerostomia but not mucositis. It appears to be selectively protective to salivary glands and kidneys without being tumor protective. The most common side effects associated with amifostine are nausea, vomiting, hypotension, hypocalcemia and allergic reactions. We describe the case of a man with dermatomyositis and stage IV nasopharyngeal cancer treated with chemoradiotherapy and s.c. amifostine. The patient suffered a life-threatening anaphylactoid reaction to amifostine.
PURPOSE OF REVIEW: Dermatomyositis (DM) is a rare multisystem autoimmune disorder of adults and children that primarily affects skin and skeletal muscle. Classification systems of dermatomyositis, polymyositis, and the other idiopathic inflammatory myopathies focus primarily on features of muscle involvement. However, cutaneous disease does not always parallel muscle disease in its onset, activity, or response to therapy. This review will describe the distinct cutaneous clinical and histopathologic presentation of DM and the relation between these cutaneous findings, pathogenesis of DM, and serological subsets of DM. RECENT FINDINGS: This review discusses recent findings that have begun to elucidate the pathogenesis of DM, including polymorphism of tumor necrosis factor-alpha 308A allele and maternal fetal microchimerism. The recent description of other systemic diseases and drugs causing DM-like eruptions and the recognition that DM can resemble other common dermatoses highlights the need for a cutaneous biopsy to diagnose and distinguish the cutaneous features of DM. Once diagnosed, a number of noninvasive imaging modalities and new cutaneous assessment instruments can be used to follow and evaluate patients with DM. SUMMARY: Recognition of cutaneous and histopathologic findings in DM is essential for prompt and accurate diagnosis and treatment of DM.
Two children with juvenile dermatomyositis and extensive, debilitating soft tissue calcifications are described. Whole-body bone scans with Tc-99m MDP were performed in both cases before and during specific treatment for the calcinosis. Baseline studies showed marked tracer localization in the soft tissues and provided an objective baseline assessment of the extent of the soft tissue calcifications. Follow-up studies showed gradual clearance of the extraskeletal uptake and were useful in monitoring the therapeutic response. These cases show that skeletal scintigraphy can function as a useful auxiliary tool to evaluate calcinosis in children with juvenile dermatomyositis.
This paper presents the findings on two patients with a final diagnosis of dermatomyositis in whom 111In-antimyosin imaging revealed diffuse muscle necrosis of the limbs. The potential application of this scintigraphic procedure for diagnosis and follow-up of patients with dermatomyositis-polymyositis is addressed.
In this case of dermatomyositis, the patient's clinical course was complicated by the development of acute granulocytic leukemia. Various malignancies complicating dermatomyositis have been reported in the literature. Rarely have these been of the hematopoietic system.