Heliotrope Rash Research Articles

Clinical analysis of 29 patients with clinically amyopathic dermatomyositis

To explore the clinical features and prognosis of clinically amyopathic dermatomyositis (C-ADM). The clinical data, including skin lesion, muscle involvement, and lung disease, of 29 patients with C-ADM, 6 males and 23 females with a male/female ratio of 1:3.83, aged 44 +/- 8, were analyzed. Skin biopsy was taken in 5 patients and lung HRCT was done in all the patients. The mean age at onset was (44 +/- 8) years. All patients presented with characteristic skin lesions such as Gottron's papulae (91.3%); heliotropic periorbital erythremia (75.9%); V-sign rash (37.9%); shawl-sign rash (24.1%); and periungual erythema and telangiectasias (20.7%). Gottron's papule was most commonly seen on the dorsal aspect of elbow (86.2%), proximal interphalangeal joints (58.6%), metacarpophalangeal joints (48.3%), patellae (23.8%), hip (20.7%), shoulder (13.8%), and ankle (9.5%). The cutaneous histopathologic pictures of these patients were all compatible with the skin lesions of dermatomyositis. Interstitial lung disease (ILD) was found by lung HRCT in 19 patients (65.5%). All patients received steroids combined with immunosuppressants, but rapidly progressive ILD happened to some of the patients and finally led to death due to respiratory failure in 5 of them. C-ADM is most commonly seen in the middle-aged women. ILD is the commonest respiratory problem arising in C-ADM patients and can be fatal, therefore should be properly treated.

Severe subcutaneous generalized edema in a patient with dermatomyositis

Subcutaneous generalized edema associated with dermatomyositis (DM)/polymyositis (PM) is extremely rare. Herein we report a case of severe subcutaneous generalized edema complicating DM. A 78-year-old woman was hospitalized in our department because of massive edema in the four limbs. Elevated muscle enzymes, heliotrope rash, results of electromyography, and muscle biopsy confirmed the diagnosis of DM. The absence of other diseases that could cause the symptoms indicated that massive edema was correlated with the pathophysiology of DM. Although myopathy and edema responded well to oral prednisolone, dysphagia persisted. We conclude that subcutaneous generalized edema can occur during the course of DM/PM, and subcutaneous vasculopathy may be involved in the pathogenesis of DM/PM.

A patient with interstitial pneumonia associated with dermatomyositis who relapsed after reducing cyclosporin-A treatment

A 67-year-old female noticed dyspnea on exertion associated with the development of erythema in the eyelids and the bilateral fingers, and was admitted to our hospital on July 21, 2004. Proximal muscle weakness in the limbs, heliotrope rash, and Gottron's sign were observed, but the CK level was normal (194 U/l). All autoantibodies except for rheumatoid factor were negative. Hypoxemia and interstitial pneumonia on chest CT images were observed. Based on these findings, a diagnosis of advanced interstitial pneumonia associated with dermatomyositis was made. Combination immunosuppressive therapy was initiated with corticosteroid pulse therapy and cyclosporin-A (Cy-A), resulting in marked improvement. The Cy-A trough concentration was markedly high (456.4 ng/ml). When cytomegalovirus infection developed, the dose of Cy-A was reduced. Although the blood trough concentration of Cy-A was maintained at an adequately high level, the patient died of recurrence of rapidly progressive interstitial pneumonia. Careful observation is required when the dose of Cy-A is reduced for a patient with interstitial pneumonia associated with dermatomyositis. Furthermore, it is suggested that the trough concentration level of Cy-A is not always a useful parameter.

Dermatomyositis With Panniculitis

Case 1. A 23-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and limbs, particularly over the dorsum of the hands and fingers; diffuse alopecia; and an inability to climb stairs and get up from a low seat. The clinical examination showed red to violaceous well-demarcated plaques on sun-exposed areas on the dorsum of the fingers and hands, with periungual erythema and telangiectasia; facial erythema; and heliotrope rash. There was also symmetric involvement of proximal muscles of the limbs. Laboratory examination showed hypergammaglobulinemia, elevated serum aspartate aminotransferase, and serum alanine aminotransferase; normal activities of creatinokinase, lactate dehydrogenase, and aldolase; an antinuclear antibody titer of 1:40 with a speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Skin biopsy histopathology showed hyperkeratosis, edema of the upper epidermis, scattered inflammatory infiltrate, and focal accumulation of mucin in the form of acid mucopolysaccharides. Deep asymptomatic nodules on the inner upper limbs appeared later. Histopathology of these lesions showed focal areas of lobular panniculitis in the subcutaneous tissue, with lymphoplasmocytic inflammatory infiltrate without vasculitis (Figure 1 and Figure 2). Case 2. A 29-year-old white housewife presented with an erythematous violaceous rash on her face, neck, chest, and lower extremities. Clinical examination showed red to violaceous well-demarcated aching plaques on the internal surface of the thighs and tips of the fingers; periungual erythema and digital petechiae; Raynaud's phenomenon; and bilateral ulnar and cervical enlarged lymph nodes. Laboratory examination showed elevated serum aspartate aminotransferase, alanine aminotransferase, creatinokinase, lactate dehydrogenase, and aldolase; negative venereal disease research test results; an antinuclear antibody titer of 1:1024 with speckled pattern; negative anti-DNA and anti-Scl70; and normal serum complement levels (C3, C4, and CH50). Urinalysis results were within normal limits. Histopathology of the deep asymptomatic nodule on the inner left thigh showed lobular panniculitis with a scattered inflammatory infiltrate and diffuse fat necrosis, in addition to calcium deposition between the lipocytes and microcysts without vasculitis (Figure 3).

Juvenile Dermatomyositis

Juvenile dermatomyositis (JDM) is an inflammatory multi-system disease of unknown etiology with classic involvement of the skin and striated muscles. Following a prodromal period, patients develop a progressive proximal muscle weakness. Typical skin involvement includes heliotrope rash, facial erythema, Gottron's sign and nailfold capillary abnormalities. For the diagnosis of JDM, modified Bohan and Peter criteria are used including clinical skin and muscle signs plus elevated muscle enzymes and typical findings from electromyography, muscle biopsy and - more recently - also on magnetic resonance imaging. Steroids are administered classically as high-dose oral treatment. Intravenous pulse therapy with intermittent lower dose oral treatment and other immunosuppressive drugs such as methotrexate may reduce steroid side-effects. Prognosis in JDM has improved, and most patients eventually make a full functional recovery. However, a few patients still die from their disease, and in a minority significant sequelae with muscle atrophy or severe calcinosis ensue.

Identification of a novel autoantibody reactive with 155 and 140 kDa nuclear proteins in patients with dermatomyositis: an association with malignancy

Myositis-specific autoantibodies (MSAs) are a useful tool in diagnosis, defining clinical subsets and predicting prognosis of dermatomyositis (DM) and polymyositis (PM). In this study, we identified a novel MSA reactive with 155 and 140 kDa nuclear proteins [anti-155/140 antibody (Ab)] and determined the clinical feature of DM patients positive for this autoantibody (autoAb). Sera from 52 Japanese patients with DM, 9 with PM, 48 with systemic lupus erythematosus (SLE), 126 with systemic sclerosis and 18 with idiopathic interstitial pneumonia were examined by immunoprecipitation assays. Positive sera were further characterized by immunodepletion and immunofluorescence staining. Seven of the 52 (13%) Japanese patients with DM immunoprecipitated 155 and 140 kDa proteins from 35S-labelled K562 leukaemia cell extract. No patients with SLE, systemic sclerosis or idiopathic interstitial pneumonia as well as healthy controls were positive for this autoAb. Patients with anti-155/140 Ab developed heliotrope rash, Gottron's papules or sign and flagellate erythema significantly more frequently than those negative. Notably, internal malignancy was found at significantly higher frequency in those positive than those negative (71 vs 11%; P < 0.005). In contrast, none of these patients positive for this autoAb had interstitial lung disease. This novel MSA is associated with cancer-associated DM and may serve as a diagnostic serological marker for this specific subset.

Gottron’s papules-like eruption developing under hydroxyurea therapy

A 57-year-old white female developed a Gottron's papules (GP)-like eruption, without any of the other clinical or laboratory signs of dermatomyositis (DM). She was under hydroxyurea treatment for chronic myeloid leukemia at the time. Skin biopsy was compatible with seborrheic keratosis. Conditions presenting with GP but unrelated to DM are reviewed, with emphasis on hydroxyurea-induced skin lesions.

Treatment of Recalcitrant Dermatomyositis with Efalizumab

Dermatomyositis (DM) is an auto-immune disease that primarily affects muscles and skin. Muscle weakness appears in the shoulders and hips in particular. Skin symp-toms include a diffuse violaceous erythema and oedema, predominantly of the upper face including the orbita, and violaceous-red papules of the finger (Gottron papules). The pathogenesis of DM is unclear, but recent studies suggest a central role of auto-aggressive lymphocytes and type I interferons (1–4), as has been proposed earlier for cutaneous lupus erythematosus (5–7). Corticosteroids are the established first-line treatment for DM. However, their use is limited because of side-effects. Unfortunately, recalcitrant courses exist that respond poorly to corticosteroids and to other immuno-modulatory and immunosuppressive drugs (8). CASe RepORTAn 82-year-old woman presented with peri-orbital viola-ceous-red oedema and erythema extending to the chest with proximal muscle weakness (Fig. 1a). She suffered from dysphagia and 6 kg weight loss during the last 4 months. Histology of lesional skin biopsy, taken from the chest, revealed an interface dermatitis associated with sparse lymphocytic infiltration in a perivascular distribution and mucin depositions. electromyographic investigations confirmed a pathological reaction pattern in the biceps brachii muscle, indicating myositis. Muscle biopsy was denied. She had slightly elevated antinuclear antibodies (1:80, granular pattern), but blood count, liver enzymes, and serum creatinine kinase were within normal limits. No malignancy could be found.Initial treatment with 60 mg prednisolone per day did not improve the clinical picture. Additional intravenous administration of 15 mg methotrexate per week for 8 weeks also had no effect.This prompted us to initiate a subcutaneous treatment with efalizumab (Raptiva

Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies

The idiopathic inflammatory myopathies are a heterogeneous group of diseases that can involve various systems. Antibodies directed against aminoacyl-tRNA synthetases, such as anti-Jo-1 antibodies, are strongly associated with a syndrome which consists of myositis, interstitial lung disease (ILD), arthritis and Raynaud's phenomenon.Forty-one patients with various forms of idiopathic inflammatory myopathies were assessed: 14 patients with anti-Jo-1 antibodies and 27 patients without anti-Jo-1 antibodies as a control group. We retrospectively analysed clinical symptoms, treatment and outcome in both groups.Patients with anti-Jo-1 antibodies more often had ILD (64.2 vs. 11.1%), arthritis (64.2 vs. 18.1%) and Raynaud's phenomenon (38 vs. 0%). Patients without the anti-Jo-1 antibody presented worse muscle strength and more frequently myalgia (37 vs. 21%), cutaneous rash (18.5 vs. 7%), heliotrope rash (29% vs. 7%) and periungueal changes (22 vs. 0%) than the anti-Jo-1-positive patients. Outcome was good in both groups. Improvement was achieved in the 14 (100%) Jo-1 positive patients, and in 25 (92.5%) controls. Two (7.5%) patients from control group achieved remission.

National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary—Clinical characteristics and disease course of 44 patients with juvenile dermatomyositis

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs.Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM).A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan–Meier method.Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.

Amyopathic dermatomyositis

Amyopathic dermatomyositis (ADM) is a rare condition characterized by skin lesions suggestive of dermatomyositis (DM) without detectable muscle abnormalities after at least 2 years of follow-up. Pulmonary fibrosis is uncommon in patients with ADM. Case report. – A 64-year-old woman presented with a 2 years and 6 months history of nondestructive polyarthritis. She had skin changes suggestive of DM, including a pink rash over the face, neck, and forearms; Gottron's papules over the metacarpophalangeal joints; and heliotrope edema of the eyelids. She reported no muscle symptoms. Findings were normal from muscle enzyme assays, electromyography, and muscle biopsies. A diagnosis of ADM was given. Early lung fibrosis was found. Investigations for a tumor were negative. Discussion. – ADM is a rare condition that may be an abortive form of DM with a favorable outcome and a lower risk of malignancy compared to classic DM. However, the development of pulmonary fibrosis may cloud the prognosis.

International consensus guidelines for trials of therapies in the idiopathic inflammatory myopathies

Polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM) are acquired inflammatory muscle disorders of unknown etiology that are currently classified under the rubric of idiopathic inflammatory myopathy (IIM). All forms of IIM are characterized clinically by muscle weakness and pathologically by chronic muscle inflammation, although systemic manifestations involving many organ systems are common. Clinical subgroups have been recognized for many years and include adult-onset and juvenile-onset forms of PM and DM, myositis associated with another connective tissue disorder or malignancy, and the more recently recognized entity termed inclusion body myositis (1,2). The presence of characteristic cutaneous features such as Gottron’s papules and sign or the classic heliotrope rash distinguish DM from PM, and skin lesions may dominate the clinical presentation in some instances. Although the IIMs are rare diseases with an estimated incidence of 10 cases per million per year (3), they are associated with substantial morbidity and mortality. Currently available treatment of IIM is unsatisfactory. Corticosteroids are the only agents approved by the US Food and Drug Administration (FDA) for myositis. However, anecdotal reports and reports of case series do support the use of other immunosuppressive agents such as methotrexate and azathioprine in the treatment of myositis. Because of the rarity and heterogeneity of IIM, there is a paucity of controlled prospective clinical trials, and most published studies are from single referral centers reporting retrospectively on small numbers of patients followed up for relatively brief periods of time. Furthermore, accurate assessment of the effects of therapeutic interventions in IIM has been hampered by unreliable and insensitive outcome measures, as well as the challenge of distinguishing active and reversible disease from irreversible damage to muscle and other organs. Although previously published trials have utilized predefined outcome measures, no uniform criteria have been used to guide the conduct of these trials. As a result, different inclusion and exclusion criteria, trial duration, frequency of assessments, concomitant therapies, safety monitoring procedures, outcome measures, definitions of responses and disease flares, and stratifications of patients at outcome analysis have been used in almost all trials. Thus, it has been difficult to compare results from any two myositis trials even when the same agent is being studied, resulting in the current uncertainty regarding the safety and efficacy of most agents.

MRI in biopsy-negative dermatomyositis

A 66-year-old man presented with proximal myopathy and a heliotrope skin rash, suggestive of dermatomyositis. Serum creatine kinase was over 40,000 IU/L. Muscle biopsy of the left vastus lateralis failed to show evidence of necrosis or inflammation. MRI demonstrated edema of …

Lamisil Induced Dermatomyositis

Background: Dermatomyositis, a connective tissue disease syndrome where antibodies to the endothelium of the microvasculature of the skin, muscle and lung are implicated in lesional propagation, is characterized by photodistributed erythema, heliotrope rash, Gottron’s papules, muscle weakness and interstitial pulmonary fibrosis. Endotheliotropic viruses and underlying neoplasia are among the inciting triggers. Uncommonly drugs, namely the lipid lowering agents, have been implicated in dermatomyositis. Case Report: The patient, a 57‐year‐old male, developed a photodistributed rash and muscle weakness following treatment with the antifungal medication Lamisil. A skin biopsy was performed, showing an atrophying interface dermatitis with pandermal mucinosis and striking vasculopathic changes including endothelial cell necrosis with denudement and basement membrane zone reduplication. Direct immunofluorescent testing showed prominent staining of C5b‐9 along the dermal‐ epidermal junction and within the vasculature. Conclusion: We have shown a temporal association between use of Lamisil and the development of dermatomyositis. Terbinafine, the active agent in Lamisil, promotes apoptosis of human endothelial cells in culture. Enhanced endothelial cell apoptosis results in the displacement of various cellular antigens creating a state of neo‐antigenicity; its attendant sequelae has held to be one of antiendothelial cell antibody formation, a defining pathogenetic event in the evolution of dermatomyositis.

A case of Interstitial Pneumonia associated with Dermatomyositis effectively treated with Cyclosporin-A and Cyclophosphamide pulse

A 46-year-old male demonstrated edematous fingers on both hands in November 2003, and interstitial pneumonia was noted on chest X-ray during a medical check-up in December 2003. Since muscular weakness and fever developed thereafter, and interstitial pneumonia was aggravated on chest X-ray and CT, the patient was admitted to our hospital on March 25, 2004. Heliotrope-like erythema, and Gottron's sign were noted. Laboratory findings showed the following ; LDH 876 U/l ; CK 224 U/l ; CRP 5.68 mg/dl ; and KL-6 3270 U/ml. Autoantibodies such as anti-Jo-1 antibody were all negative. Chest X-ray and CT showed ground-glass opacity in the bilateral lower dorsal regions of the lung, and reduced volume of the inferior lobe. He was diagnosed as having dermatomyositis (DM) associated with progressive interstitial pneumonia. Although a combination of steroid pulse therapy and Cyclosporin-A were administered, the pulmonary lesions became aggravated. Additional intravenous Cyclophosphamide (IVCY) was initiated on the 6th hospital day, and interstitial pneumonia was markedly improved. Cases of progressive interstitial pneumonia associated with DM that are negative for anti-Jo-1 antibody and show a low ratio of CK/LDH are resistant to various treatments. Our case suggested that combination therapy with steroid, Cyclosporin-A, and IVCY is useful for the treatment of progressive interstitial pneumonia with DM.

Vesiculo‐Bullous Dermatomyositis Associated with Malignant Lymphoma

Dermatomyositis is an inflammatory condition of the skin and muscles, and an underlying malignancy is noted in 10% or more of cases. Clinical features of dermatomyositis include increasing general fatigue and proximal (thighs and shoulders) muscle weakness accompanied by erythematous lesions of the skin. There have been several distinct types of dermatomyositis described. Here we describe a case of vesiculo‐bullous dermatomyositis, which is a rare variant of dermatomyositis. A 49‐year‐old woman was admitted to our hospital with a painful erythematous vesicular eruption of the face, trunk and extremities. In addition, edema of the face and fever were observed. Clinically, dermatomyositis was considered because of typical skin rashes (Gottron’s papules, periorbital heliotrope rash and poikiloderma) and serum creatine phosphokinase level of 1,031 IU/L. A skin biopsy was performed. Microscopically, subepidermal vesiculation with marked edema was present. Lymphoplasmacytic infiltration was also observed in the upper dermis. So far only a few case reports of vesiculo‐bullous dermatomyositis have been reported in the literature. It should be kept in mind that dermatomyositis may present subepidermal vesiculation in order to avoid a misdiagnosis and unnecessary delayed treatment. Furthermore, an internal malignancy should be considered in such a variant of dermatomyositis.

Dermatomyositis and duodenal carcinoid

A 70-year-old female presented with erythematous macular rash over arms, shoulders, trunk and face for 4 months, proximal muscle weakness and dysphagia to liquids for 2 months and ulcers over knuckles and back of thighs for 1 month duration. She had history of recurrent oral ulcers and photosensitivity for 1 year. Examination showed pallor, pedal oedema, blood pressure 160/98 mmHg, heliotrope rash, Gottron’s rash, nail fold infarcts, erythema over the shoulders, arms, neck and back, ulcers over the back of thighs, oral ulcers, left axillary lymphadenopathy, grade II muscle power (inability to lift against gravity) at the shoulder and pelvic girdle muscles and poor gag reflex. Neck holding was poor. The rest of the examination was unremarkable. Investigations revealed hemoglobin 8.4 g/dl, erythrocyte sedimentation rate (ESR) 68 mm, normal platelets, total and differential leucocyte counts, renal functions, skiagram chest and urine examination. Her serum glutamate oxaloacetate transaminase (SGOT), aspartate transaminase (SGPT) and creatine phosphokinase (CPK) (MM), serum protein, albumin, bilirubin, alkaline phosphatase were 131 IU/ml (normal 15–40), 66 IU/ml (normal 13–35) and 174 U/ml (normal 24– 170), 4.7 g/dl, 1.9 g/dl, 0.8 mg/dl and 375 IU/ml (normal 65–306), respectively. Test for antinuclear antibody was negative. Electromyogram showed increased insertional activity, spontaneous high frequency discharges, and polyphasic potentials with low amplitude and short duration in quadriceps and deltoid muscles. Ultrasound of the abdomen showed fatty hepatomegaly. Upper gastrointestinal endoscopy (UGIE) was normal. A diagnosis of dermatomyositis (DM) was made and the patient was started on prednisolone 1 mg/kg per day along with hydroxychloroquine. Over the next 6 months her muscle power normalized, oral ulcers and skin rash disappeared completely and laboratory tests for muscle enzymes (SGOT, SGPT, CPK MM) normalized. Sixteen months after initial presentation she presented to the emergency room in hypotension with recurrent bilious vomiting of 7 days duration. Her muscle power was normal and there were no skin rash and oral ulcers. An UGIE study revealed obstruction in the third part of the duodenum. Contrast-enhanced computerised tomogram revealed a mass in the region of the third part of the duodenum with multiple space-occupying lesions (SOLs) in liver (Fig. 1) and lungs. Fine needle aspiration of the hepatic SOLs revealed malignant tumor of neuroendocrine origin. Excretion of urinary 5-hydroxyindoleacetic acid level was elevated (70 mg/dl). Tests for muscle enzymes (SGOT, SGPT, CPKMM), renal functions and liver functions were normal. During the gastrojejunostomy procedure a mesenteric lymph node was excised which confirmed carcinoid tumor on histopathology. On the 5th postoperative day the patient succumbed to massive upper gastrointestinal bleeding.

A Case of Dermatomyositis Associated with Different Types of Cancers at Intervals of Six Years

The patient is a 56-year-old Japanese woman who suffered from breast cancer and ovarian cancer at intervals of 6 years, and was also complicated by two episodes of dermatomyositis, each of which occurred simultaneously with each of two cancers. When she was 51 years old, she developed dermatomyositis for the first time 6 months after the resection of breast cancer, whose histological type was tubular adenocarcinoma. The dermatomyositis remitted without oral corticosteroids in 2 months, and the remission had continued for 6 years. However, at the age of 56, dermatomyositis abruptly recurred with a pruritic generalized rash, Gottron's papules and elevated serum CK levels. Examination for malignancy revealed an ovarian tumor, which was diagnosed as serous papillaly adenocarcinoma, and the surgery was performed. After the resection of the ovarian cancer, skin rash was improved dramatically and CK levels were normalized again without oral corticosteroids. Since there were no evidences of recurrence of the breast cancer, it was considered that each episode of dermatomyositis was associated with each of the cancers, respectively. We report this rare and interesting case to consider the etiology of cancer-associated myositis as a paraneoplastic syndrome, since the two cancers have different histological types.

Fasciitis in Amyopathic Dermatomyositis

Dermatomyositis is a disease frequently treated by rheumatologists and dermatologists due to prominent systemic features of inflammatory myositis, less common arthritis, and rare systemic vasculitis, in addition to the characteristic cutaneous manifestations of Gottron's papules over extensor surfaces, and a heliotrope rash over the eyelids. Patients with amyopathic dermatomyositis, a subset of dermatomyositis, display skin disease but no apparent muscle disease. This report describes an adult patient with the typical dermatomyositis rash with no weakness, normal muscle enzymes, and the unique finding of fasciitis without myositis on muscle biopsy, which correlated with a Magnetic Resonance Imaging (MRI) finding of a peripheral halo of intense signal around muscles on T2-weighted and fat suppression sequences. Although MRI finding of presumed fasciitis has been reported in juvenile-onset dermatomyositis, this is the first report of MRI evidence of fasciitis with pathological correlation in adult-onset dermatomyositis. We suggest that if MRI scans are ordered, as part of the work-up of dermatomyositis, a peripheral halo of increased signal should specifically be looked for, which could be interpreted as fasciitis based on this case report.

Accessory findings on F-18 FDG positron emission tomography in bronchogenic carcinoma.

A 76-year-old man with proved small-cell and non-small-cell lung cancer and symptoms of a paraneoplastic syndrome, including a heliotrope rash; scaly erythematous rash over the face, neck, and upper torso; proximal muscle weakness; and creatine kinase elevation, underwent F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) as part of a work-up to detect (mediastinal) lymph nodes or distant metastases.