Dermatomyositis masquerading as pulmonary embolism
© I. Holzapfel Publishers 2009
Published: 7 December 2009
A 61-year-old Caucasian was admitted to Department of Chest Diseases and Tuberculosis, Medical University of Bialystok, Poland for progressive muscle weakness and weight loss. Eighteen months prior to admission, the patient had been diagnosed with pulmonary embolism. At that point he was started on Enoxaparin QD. Past medical history was unremarkable. In the interim, the patient developed fever, myalgia and progressive dyspnea. Physical examination on admission revealed a rash on his upper torso and back, and the extensor surfaces of all four extremities. Laboratory values included CPK 8229, MB fraction 219, LDH 981. Chest X-ray and CT scan revealed bilateral patchy consolidations and ground-glass opacities. EMG was consistent with myositis. The patient was started on solumedrol 40 mg i.v., b.i.d., and then switched to prednisone 40 mg b.i.d. His symptoms and muscle strength improved remarkably. The patient was discharged with prednisone with an outpatient follow up.
1. Progressive symmetrical general muscle weakness
2. Typical for myositis histopathology findings
3. Elevated plasma level of CK and/or aldolase
4. EMG findings typical for primary myopathy
5. Typical skin findings (Gottron's sign, helitrope rash of eyelids, upper torso, arms)
Number of symptoms 5
plus characteristic skin findings
The study was performed according to the standards set by the Helsinki Declaration of 1975, regarding the Human Research and was approved by an institutional Ethics Committee. Informed consent was obtained from the patient described in this article.
This patient was diagnosed with dermatomyositis, fitting 4 out of the five diagnostic criteria, as outlined above: proximal muscle weakness, CPK and LDH elevations, consistent with myositis EMG, and skin manifestations [4, 5]. The latter were as follows: purple confluent discoloration and swelling of the eyelids - heliotrope rush, the V-shape rash with areas of hyperpigmentation of his upper torso and shoulders, back, knees, and erythematous papules over joints - 'Gottron's sign', and hypertrophic changes of palms and fingers - 'Mechanic's hands'. The diagnosis is confirmed if a patient presents with at least 4 diagnostic criteria for PM or 3 for DM [4, 5]. The fifth criterion which is confirmation of inflammatory infiltrations on muscle biopsy was not done in this case. Since our patient exhibited clinical and laboratory findings consistent with inflammatory myopathy with skin manifestations present, our diagnosis was consistent with dermatomyositis.
The cause of the disease remains unknown. Altered immune response towards viral infection is considered to play a major role [1, 2]. Vascular deposits of immune complexes and complement, leading to endothelial cell injury and small vessel obstruction are most important pathogenetic factors underlying the disease . The muscles and endothelial cells are infiltrated with predominance of T CD4+ cells in DM and T CD8+ cells in PM patients . An association of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis has recently been reported [7, 8]. Our patient exhibited all reported skin manifestations of DM which were: a heliotrope rash, which is a purple discoloration and swelling of the eyelids, Gottron's sign demonstrated with erythematous papules over joints, and mechanic's hands, which are: erythematous and hypertrophic skin changes of palms and fingers [1–5]. Hemoptysis, progressive dyspnea, subtle dry cracles and wheezing on auscultation, with bilateral patchy consolidations and ground-glass opacities on chest X-ray and CT scans confirmed lung interstitium disease association in our case. Hemoptysis and pulmonary embolism, lack of significant muscle weakness at the onset of the disease masqueraded underlying DM and delayed proper diagnosis. Lung involvement has been reported in minority of PM and DM cases, influencing the course and worsening prognosis in most of the cases [9, 10]. An association with other connective tissue disorders (overlap syndrome) and malignancy has also been reported [9–13]. The course of the disease is usually steadily progressive, leading to profound symmetrical skeletal muscle weakness of all extremities. Patient management includes careful evaluation for underlying malignancy, physical therapy, antihistamines, sunscreen, and oral corticosteroids . Alternative therapies include corticosteroid-sparing agents, cytotoxic drugs, and more recently monoclonal antibodies with cutaneous lesions treatment [14–18]. Irresponsive disease, delay in diagnosis and the underlying malignancies worsen disease prognosis.
Conflicts of interest
The authors declare that they have no competing interests.
- Krathen MS, Fiorentino D, Werth VP: Dermatomyositis. Curr Dir Autoimmun 2008, 10: 313–32.PubMed CentralPubMedView ArticleGoogle Scholar
- Targoff IN: Dermatomyositis and polymyositis. Curr Probl Dermatol 1991, 3: 131–180. 10.1016/1040-0486(91)90009-SView ArticleGoogle Scholar
- Marie I, Hatron PY, Levesque H, et al.: Influence of age on characteristics of polymyositis and dermatomyositis in adults. Medicine (Baltimore) 1999,78(3):139–47. 10.1097/00005792-199905000-00001View ArticleGoogle Scholar
- Bohan A, Peter JB: Polymyositis and dermatomyositis (first of two parts). N Engl J Med 1975,292(7):344–7. 10.1056/NEJM197502132920706PubMedView ArticleGoogle Scholar
- Bohan A, Peter JB: Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975,292(8):403–7. 10.1056/NEJM197502202920807PubMedView ArticleGoogle Scholar
- Dalakas MC: Molecular immunology and genetics of inflammatory muscle diseases. Arch Neurol 1998,55(12):1509–12. 10.1001/archneur.55.12.1509PubMedView ArticleGoogle Scholar
- Werth VP, Callen JP, Ang G, Sullivan KE: Associations of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis: implications for a unique pathogenesis. J Invest Dermatol 2002,119(3):617–20. 10.1046/j.1523-1747.2002.01869.xPubMedView ArticleGoogle Scholar
- Efthimiou P: Tumor necrosis factor-alpha in inflammatory myopathies: pathophysiology and therapeutic implications. Semin Arthritis Rheum 2006,36(3):168–72. 10.1016/j.semarthrit.2006.07.003PubMedView ArticleGoogle Scholar
- Fujisawa T, Suda T, Nakamura Y, Enomoto N, Ide K, Toyoshima M, Uchiyama H, Tamura R, Ida M, Yagi T, Yasuda K, Genma H, Hayakawa H, Chida K, Nakamura H: Differences in clinical features and prognosis of interstitial lung diseases between polymyositis and dermatomyositis. J Rheumatol 2005,32(1):58–64.PubMedGoogle Scholar
- Yokoyama T, Sakamoto T, Shida N, Kinoshita M, Kunou Y, Karukaya R, Takamatsu M, Aizawa H: Fatal rapidly progressive interstitial pneumonitis associated with amyopathic dermatomyositis and CD8 T lymphocytes. J Intensive Care Med 2005,20(3):160–3. 10.1177/0885066605275391PubMedView ArticleGoogle Scholar
- Callen JP: The value of malignancy evaluation in patients with dermatomyositis. J Am Acad Dermatol 1982,6(2):253–9. 10.1016/S0190-9622(82)70018-0PubMedView ArticleGoogle Scholar
- Chow WH, Gridley G, Mellemkjaer L, McLaughlin JK, Olsen JH, Fraumeni JF Jr: Cancer risk following polymyositis and dermatomyositis: a nationwide cohort study in Denmark. Cancer Causes Control 1995,6(1):9–13. 10.1007/BF00051675PubMedView ArticleGoogle Scholar
- Whitmore SE, Rosenshein NB, Provost TT: Ovarian cancer in patients with dermatomyositis. Medicine (Baltimore) 1994,73(3):153–60.View ArticleGoogle Scholar
- Iorizzo LJ, Jorizzo JL: The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol 2008,59(1):99–112. 10.1016/j.jaad.2008.02.043PubMedView ArticleGoogle Scholar
- Majithia V, Harisdangkul V: Mycophenolate mofetil (Cell-Cept): an alternative therapy for autoimmune inflammatory myopathy. Rheumatology (Oxford) 2005,44(3):386–9. 10.1093/rheumatology/keh499View ArticleGoogle Scholar
- Nadiminti U, Arbiser JL: Rapamycin (sirolimus) as a steroid-sparing agent in dermatomyositis. J Am Acad Dermatol 2005,52(2 Suppl 1):17–9.PubMedView ArticleGoogle Scholar
- Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, Dinsmore ST, McCrosky S: A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 30 1993,329(27):1993–2000.View ArticleGoogle Scholar
- Levine TD: Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum 2005, 52: 601–7. 10.1002/art.20849PubMedView ArticleGoogle Scholar