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A 62-Year-Old Man With Skin Rash and an Abnormal Chest RadiographMan With Skin Rash and Abnormal Chest Radiograph FREE TO VIEW

Tyler J. Albert, MD; Sarah Bastawrous, DO; Gregory J. Raugi, MD, PhD; Jan V. Hirschmann, MD
Author and Funding Information

From the Department of Medicine (Drs Albert, Raugi, and Hirschmann), and Department of Radiology (Dr Bastawrous), VA Puget Sound Medical Center; and Department of Medicine (Drs Albert and Hirschmann), Department of Radiology (Dr Bastawrous), and Division of Dermatology (Dr Raugi), University of Washington School of Medicine, Seattle WA.

CORRESPONDENCE TO: Sarah Bastawrous, DO, VA Puget Sound Medical Center, Department of Radiology, Mail Box 358280, S-114, 1660 S Columbian Way, Seattle WA 98108-1597; e-mail: ssheikh@u.washington.edu


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2015;147(3):e90-e94. doi:10.1378/chest.14-1100
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A 62-year-old man developed a scalp rash 2 months ago, followed by bilateral eyelid swelling. The nonpruritic rash then spread to involve most of his skin. He also had fatigue, muscle weakness, mild muscle soreness with activity, and dysphagia for solid foods for the last 3 weeks. He had no other symptoms. He had a 50 pack-year history of smoking and drank two to three shots of alcohol daily.

Figures in this Article

The patient’s vital signs and findings from chest, cardiac, and abdominal examinations were normal. His skin examination was notable for violaceous erythema of the face, scalp, neck, shoulders, and extremities, with multiple areas of superficial ulceration. Violaceous bilateral periorbital edema was present (Fig 1A). His hands had erythema over the proximal interphalangeal joints, particularly on the middle and ring fingers, and periungual suffusion and erythema, especially of the ring finger (Fig 1B). Periungual telangiectasias were also visible. The musculoskeletal examination revealed mild proximal muscle weakness of both arms and legs bilaterally. The remainder of his examination was unremarkable.

Figure Jump LinkFigure 1 –  A, B, Photographs of the (A) face and (B) hand. (The patient provided written consent for the use of this photograph.)Grahic Jump Location

CBC count, renal function, and electrolyte level were normal. Notable laboratory values were as follows: aspartate aminotransferase, 124 U/L; alanine aminotransferase, 53 U/L; and creatine kinase, 1,952 U/L. His chest radiograph showed a small left upper lobe nodular opacity (Fig 2).

Figure Jump LinkFigure 2 –  Posterior-anterior chest radiograph shows small left upper lung nodular opacity (arrow) and left hilar prominence.Grahic Jump Location
What is the diagnosis?
Diagnosis: Dermatomyositis associated with lung cancer

Dermatomyositis is an inflammatory disease with proximal muscle weakness and diverse dermatologic findings. Gottron papules are violaceous papules over bony prominences, such as the knuckles, elbows, or knees. Light-purple (heliotrope) periorbital swelling is common, and, like Gottron papules, is considered pathognomonic of the disease. A violaceous macular erythema is frequent, especially in sun-exposed areas, including the V distribution over the upper chest. Erythematous macules are often present on the upper-lateral thighs (holster sign) and in the “shawl” distribution over the shoulders, arms, and back. These lesions may be poikilodermatous, indicating a combination of hyperpigmentation, hypopigmentation, dermal atrophy, and telangiectasias. Periungual erythema, telangiectasias, and irregular cuticles occur in the nail fold, which, when examined with magnification, demonstrates capillary changes of thrombosis, hemorrhage, enlarged loops, or loss of vessels. The findings of “mechanic’s hands” include hyperkeratosis with scaling, horizontal fissuring, and hyperpigmentation of the digits and palms, which make the hands look soiled. The scalp is often pruritic, scaly, and red or violaceous.

Although skin findings occasionally occur in the absence of myopathy (amyopathic dermatomyositis), most patients have symmetrical proximal muscle weakness. Common complaints are difficulty climbing stairs, rising from a seated position, or raising the arms for such activities as combing hair. Myalgia or muscle tenderness is unusual. When the striated muscle of the pharynx or upper esophagus is involved, dysphagia and aspiration of oropharyngeal contents may occur, leading to pneumonia.

Some patients have arthralgias or a symmetrical polyarthritis affecting primarily the small joints of the hands, wrists, and ankles. Occasionally, Raynaud phenomenon occurs. Cardiac involvement is uncommon but may cause abnormal conduction, congestive heart failure, or arrhythmias. Respiratory muscle weakness may lead to hypoventilation. Interstitial lung disease occurs in about 5% to 50% of patients, most commonly as nonspecific interstitial pneumonia but also occasionally from usual interstitial pneumonia or organizing pneumonia. Pulmonary disease may precede or follow the diagnosis of dermatomyositis.

The laboratory findings typically include evidence of muscle injury, with elevation of creatine kinase, lactic dehydrogenase, aldolase, aspartate aminotransferase, and alanine aminotransferase. A bewildering array of antibodies may be present; patients with anti-Jo-1 are more likely to have interstitial lung disease.

Electromyography shows increased spontaneous activity with fibrillations, complex repetitive discharges, and positive sharp waves. The motor unit potentials are typically short, small, and polyphasic. These findings are not specific for dermatomyositis.

Muscle biopsy reveals chronic, primarily lymphocytic, inflammation that is predominantly perivascular or in the interfascicular septae and around, rather than within, the fascicles. Endothelial hyperplasia and fibrin thrombi are present in the intramuscular vasculature. The muscle fibers undergo phagocytosis and necrosis, commonly from microinfarcts involving a part of the muscle fascicle, eventuating in perifascicular atrophy.

The skin histopathology, which is not pathognomonic of dermatomyositis, includes vacuolar degeneration of the basal layer, basement membrane thickening, hyperkeratosis, epidermal atrophy, and a sparse inflammatory dermal infiltrate. These findings are very similar to those of systemic lupus erythematosus.

The diagnosis of dermatomyositis is usually made by the combination of dermatologic findings, evidence of muscle weakness, and increased serum levels of muscle enzymes. When uncertainty exists, muscle biopsy, electromyography, or skin biopsies may be helpful. MRI of the muscles may show evidence of myositis and demonstrate which muscle would be most appropriate to biopsy.

Treatment typically begins with systemic corticosteroids, and most patients experience improvement in strength by 2 to 3 months. For refractory cases or for steroid sparing, methotrexate, azathioprine, or IV immunoglobulin can be added. Other options include mycophenolate, rituximab, cyclophosphamide, cyclosporine, tacrolimus, chlorambucil, and biologic agents such as etanercept or adalimumab.

The frequency of malignancy is increased in adults with dermatomyositis as compared with the general population. The statistics vary, but at least 20% to 25% of patients in most series are found to have a malignancy within 5 years of the diagnosis of dermatomyositis, with many occurring during the first year. The kinds of cancers are diverse and depend on the populations studied. In European and American series, the most common malignancies are lung (most frequently small cell carcinoma), breast, ovarian, gastric, and colorectal, but prostate, head and neck, and non-Hodgkin’s lymphomas, among others, have been reported. In some Asian countries, such as Singapore, China, and Taiwan, the most common is nasopharyngeal carcinoma. Successful treatment of the malignancy usually leads to improvement of the dermatomyositis, but sometimes the courses of the two diseases are independent. Because of the increased risk of malignancy, adult patients with dermatomyositis should be investigated for an underlying cancer.

Clinical Course

Photographs of the patient’s skin findings were sent under secure electronic medical record to a teledermatologist, who confirmed the presence of a heliotrope facial rash and Gottron papules on the hands, diagnostic of dermatomyositis. A chest CT scan revealed left hilar and mediastinal lymph node enlargement and a left upper lobe nodule. Bronchoscopy disclosed a hypervascular lesion at the left upper lobe carina and an irregular and narrowed left upper lobe bronchus (Fig 3). Biopsies from these areas revealed small cell cancer. MRI of the lower extremities demonstrated abnormal increased fluid signal intensity throughout most of the thigh and pelvic muscles, which enhanced after administration of gadolinium, consistent with myositis (Fig 4). Treatment with prednisone led to marked improvement of the cutaneous findings and mild increase in strength. The patient is currently receiving chemotherapy.

Figure Jump LinkFigure 3 –  Bronchoscopic examination shows a hypervascular lesion at the left upper lobe carina with an irregular and narrowed left upper lobe bronchus.Grahic Jump Location
Figure Jump LinkFigure 4 –  Coronal short τ inversion recovery MRI image through the thighs demonstrates abnormal increased signal intensity throughout the majority of the thigh and pelvic musculature (black arrows) compatible with myositis. Star designates normal muscle appearance. F = femur.Grahic Jump Location

  • 1. The diagnosis of dermatomyositis is usually based on a combination of dermatologic findings, evidence of muscle weakness, and elevation in muscle enzymes. The dermatologic findings that are considered diagnostic are a periorbital heliotrope and Gottron papules.

  • 2. Dermatomyositis in adults is associated with an increased risk of cancer within 5 years of diagnosis. Successful treatment of the malignancy usually improves the dermatomyositis, but the courses of the two diseases are sometimes independent.

  • 3. In European and American populations, the most commonly associated malignancies are ovarian, lung, breast, and gastrointestinal. The most frequent type of pulmonary malignancy is small cell carcinoma. In many Asian countries, the predominant cancer is nasopharyngeal carcinoma.

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions: CHEST worked with the authors to ensure that the Journal policies on patient consent to report information were met.

Fujita J, Tokuda M, Bandoh S, et al. Primary lung cancer associated with polymyositis/dermatomyositis, with a review of the literature. Rheumatol Int. 2001;20(2):81-84. [CrossRef] [PubMed]
 
el-Azhary RA, Pakzad SY. Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol. 2002;46(4):560-565. [CrossRef] [PubMed]
 
Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006;24(5):363-373. [CrossRef] [PubMed]
 
Antiochos BB, Brown LA, Li Z, Tosteson TD, Wortmann RL, Rigby WF. Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA. J Rheumatol. 2009;36(12):2704-2710. [CrossRef] [PubMed]
 
Kalluri M, Oddis CV. Pulmonary manifestations of the idiopathic inflammatory myopathies. Clin Chest Med. 2010;31(3):501-512. [CrossRef] [PubMed]
 
Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227-236. [CrossRef] [PubMed]
 

Figures

Figure Jump LinkFigure 1 –  A, B, Photographs of the (A) face and (B) hand. (The patient provided written consent for the use of this photograph.)Grahic Jump Location
Figure Jump LinkFigure 2 –  Posterior-anterior chest radiograph shows small left upper lung nodular opacity (arrow) and left hilar prominence.Grahic Jump Location
Figure Jump LinkFigure 3 –  Bronchoscopic examination shows a hypervascular lesion at the left upper lobe carina with an irregular and narrowed left upper lobe bronchus.Grahic Jump Location
Figure Jump LinkFigure 4 –  Coronal short τ inversion recovery MRI image through the thighs demonstrates abnormal increased signal intensity throughout the majority of the thigh and pelvic musculature (black arrows) compatible with myositis. Star designates normal muscle appearance. F = femur.Grahic Jump Location

Tables

Suggested Readings

Fujita J, Tokuda M, Bandoh S, et al. Primary lung cancer associated with polymyositis/dermatomyositis, with a review of the literature. Rheumatol Int. 2001;20(2):81-84. [CrossRef] [PubMed]
 
el-Azhary RA, Pakzad SY. Amyopathic dermatomyositis: retrospective review of 37 cases. J Am Acad Dermatol. 2002;46(4):560-565. [CrossRef] [PubMed]
 
Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006;24(5):363-373. [CrossRef] [PubMed]
 
Antiochos BB, Brown LA, Li Z, Tosteson TD, Wortmann RL, Rigby WF. Malignancy is associated with dermatomyositis but not polymyositis in Northern New England, USA. J Rheumatol. 2009;36(12):2704-2710. [CrossRef] [PubMed]
 
Kalluri M, Oddis CV. Pulmonary manifestations of the idiopathic inflammatory myopathies. Clin Chest Med. 2010;31(3):501-512. [CrossRef] [PubMed]
 
Dimachkie MM, Barohn RJ. Idiopathic inflammatory myopathies. Semin Neurol. 2012;32(3):227-236. [CrossRef] [PubMed]
 
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