https://orcid.org/0000-0001-8241-2938
Abstract
Dermatomyositis (DM) is an autoimmune disorder characterized by proximal muscle weakness and distinct cutaneous features. Unfortunately, infection is a frequent and potentially life-threatening complication in patients with DM. Here, we present a case of pyomyositis in a patient with DM resulting from localized cellulitis. The patient also presented with subcutaneous calcification nodules and dermatomyositis-associated lipodermatosclerosis nodules. To our knowledge, there have been no reports of pyomyositis in patients with DM to date. Furthermore, we reviewed the infectious complications related to DM and polymyositis (PM). We found that idiopathic inflammatory myopathy (IIM) patients exhibit a considerable infection-related mortality rate, ranging from 4.3% to 7.2%. In IIM, infections were identified as the primary cause of mortality in a substantial proportion of cases, accounting for 22.0–83.3% of deaths. These findings have implications for the importance of identifying and managing infections in IIM patients and suggest the need for further research into infection-related complications in these patients.
Introduction
Dermatomyositis is an autoimmune disease characterized by weakness in the proximal muscles and distinct skin manifestations. Pyomyositis is a subacute bacterial infection that primarily affects skeletal muscles and can arise from contiguous spread or hematogenous seeding. To date, there have been no documented cases of pyomyositis in individuals with DM. However, our case report suggests that pyomyositis may occur due to the presence of localized cellulitis in the muscles of patients with dermatomyositis. Early identification of pyomyositis is crucial for preventing muscle function loss or even death in patients with dermatomyositis. DM are associated with reduced long-term survival, particularly in cases of anti-MDA5+DM, with infections being a primary cause.Citation1 However, there is a lack of comprehensive reviews of IIM-related infections across different anatomical locations and microbial agents, and reports are scattered and sporadic. Thus, we reviewed the infectious complications related to dermatomyositis/polymyositis.
Case Report
A 49-year-old male with a history of dermatomyositis and long-term use of glucocorticoids presented to our hospital with a painful indurated swelling in the anteromedial aspect of his thigh and a low-grade fever (maximum temperature of 37.9 degrees) persisting for 12 days (). He did not achieve remission with 11 days of ceftazidime in another hospital. Physical examination revealed the presence of multiple subcutaneous nodules, scar tissue with hyperpigmentation, and local swelling and induration with redness and mild fluctuation in anteromedial thigh ( and ). Empirical treatment with cefoperazone-sulbactam was initiated, but the patient subsequently presented with crampy local pain and a “woody” texture of the anteromedial thigh muscle, making it difficult to straighten his left leg.
Figure 1 The time course of treatment and states of illness.
Figure 2 (A and D): The cicatrix, fibrocollagenous nodules and multiple calcifications in abdomen and thighs. (B and E): The characteristic features of dermatomyositis: Heliotrope sign, V sign and Gottron sign. (C) (HE×40) and (F) (HE×100): Biopsy of the subcutaneous firm nodules in the trunk revealed localized collagen hyperplasia in the dermis, accompanied by significant mucin deposition, as well as adipose septal fibrosis and hyalinization in the subcutaneous adipose tissue, featuring lipomatous cysts and membranous changes, with a minor infiltration of lymphocytes in the dermis and subcutaneous adipose tissue. (G and H): T1-weighted MR images revealed high signal intensity in sartorius muscle (white arrows) and intermuscular space. (I): The chest CT scan identified subcutaneous calcified nodules (white arrow).
Laboratory studies showed lymphocytopenia (white blood cell count 11.69*109/L, with 88.8% neutrophils) and evidence of inflammation/infection (procalcitonin 0.08ng/mL, C-reactive protein 7.58mg/L, interleukin-6 7.10pg/mL). An enhanced CT scan demonstrated skin and subcutaneous tissue swelling with irregular enhancement, soft tissue gas, focal fluid accumulation, and multiple calcifications in the left thigh. T1-weighted MR images demonstrated high signal intensity within the sartorius muscle, and culture of the lesion revealed Staphylococcus aureus. The ultimate diagnosis is stage I pyomyositis, resulting from abscess extension of the cellulitis. The patient’s condition markedly improved following ultrasonographic-guided percutaneous drainage and a two-week course of vancomycin. He subsequently received a two-week course of amoxicillin ().
Of note, the patient presented with two distinct types of subcutaneous nodules: fibrocollagenous nodules, confirmed by pathology, and multiple calcifications, demonstrated on enhanced CT imaging (). The diagnosis of deep morphea was ruled out. The existence of fibrocollagenous nodules in dermatomyositis patients is insufficiently reported and could represent the fibrotic/late phase of dermatomyositis panniculitis. This could be related to the presence of membranous cystic changes and mild lymphocytic inflammation in the adipose lobules of skin biopsy ().
Discussion
This report describes a case of pyomyositis in a patient with dermatomyositis, which may be related to the long-term use of glucocorticoids. For a period of 5 years, the patient’s condition of dermatomyositis was effectively managed with a combination of methylprednisolone and methotrexate. Methylprednisolone was initially prescribed at a daily dosage of 30mg and gradually tapered over time. In the last year leading up to admission, the dosage was reduced to 5mg once daily, and six months before admission, it was further decreased to 2.5mg once daily. The occurrence of pyomyositis can be observed in an immunosuppressed patient. This case is expected to be distinguished from necrotizing fasciitis and clostridial myonecrosis. Timely recognition and treatment of pyomyositis are crucial to prevent severe complications and mortality in patients with dermatomyositis. However, early identification can be challenging. Healthcare providers should be vigilant in monitoring and promptly identifying signs and symptoms of pyomyositis in this population.
In addition, we reviewed the clinical studies related to infection in patients with DM/PM. We conducted a comprehensive literature search on PubMed and Web of Science. For the literature screening process, two reviewers independently conducted the screening, and any disagreements were resolved through consensus. illustrates the literature screening flowchart employed in this study. Tuberculosis and COVID-19 were excluded from our study due to prior systematic reviews. Overlap syndrome is also ruled out. As of March 20th, 2023, this study has included a total of 40 studies and 10,694 patients. 33 (82.5%) of the studies are retrospective. The studies were predominantly retrospective in design and exhibited considerable variability in follow-up duration, with mean times ranging from 1 to 13 years. Although the heterogeneity in infection-related mortality was relatively low, the disparate timeframes precluded quantitative synthesis of the data. This literature review strengthens the evidence base and enhances understanding of the link between inflammatory myositis and infections.
Figure 3 The literature screening flowchart.
Our review revealed a wide range of overall mortality proportions (4–45%) among patients with idiopathic inflammatory myopathy. Among these patients, infection-related mortality rates were identified to range from 2.2% to 7.2% (). Notably, a significant proportion of deaths (22.0–83.3%) in patients with idiopathic inflammatory myopathy were primarily attributed to infections. Our study also provides a summary of infection rates observed in different IIM subtypes and infection types ( and ). The most common infection agents are cytomegalovirus, Herpes Simplex Virus/Varicella Zoster Virus, Candidiasis, pneumocystis Carinii pneumonia (). Among all patients, those with melanoma differentiation associated gene 5 antibody-positive dermatomyositis (Anti-MDA-5+DM) have the highest susceptibility to infections, with an infection rate ranging from 42.1 to 83.3% ().
Table 1 The Clinical Data and Mortality Rates in Patients with IIM
Table 2 The Clinical Data and Prevalence of Infections in Patients with Different IIM Subtypes
Table 3 The Clinical Data and Prevalence of Infections in Patients with Different Infection Types
Conclusion
Our case report highlights the potential for pyomyositis as a complication of DM resulting from localized cellulitis, while our review underscores the importance of identifying and managing infections in reducing infection-related mortality in IIM patients. These findings have important implications for the clinical management of DM and IIM and underscore the need for further research into infection-related complications in these patient populations. A better understanding of the relationship between infections and DM/IIM could help inform the development of more targeted interventions aimed at improving patient outcomes.
Abbreviations
ARS, Anti-Aminoacyl-tRNA Synthetase; ASS, Antisynthetase syndrome; BT, Biologic therapy; CMV, Cytomegalovirus; CYC, Cyclophosphamide; DM, dermatomyositis; GCs, Glucocorticosteroid; HSV, Herpes Simplex Virus; IIM, idiopathic inflammatory myositis; ILD, interstitial lung diseases; ISD, Immunosuppressive drug; IVIG, Intravenous immunoglobulin; MDA5, melanoma differentiation associated gene 5; MMF, mycophenolate mofetil; NTM, Non-tuberculosis mycobacteria; OM, overlap myositis; P, Prospective; PCP, Pneumocystis carinii pneumonia; PM, polymyositis; R, Retrospective; RTX, Rituximab; TAC, Tacrolimus; TIF1-γ, Transcription Intermediary Factor 1 Gamma; TOF, Tofacitinib; VZV, Varicella-Zoster Virus.
Data Sharing Statement
The data that support the findings of this study are available from the corresponding author upon reasonable request.
Patient Perspective
The use of Vancomycin has been instrumental in my recovery, and I have noticed a significant improvement in my symptoms since starting the medication.
Ethical Approval and Consent to Participate
The Institutional Review Board (IRB) has approved our research application. The patient provided informed consent for the publication of their case.
Author Contributions
All authors made a significant contribution to the work reported, whether that is in the conception, study design, execution, acquisition of data, analysis and interpretation, or in all these areas; took part in drafting, revising or critically reviewing the article; gave final approval of the version to be published; have agreed on the journal to which the article has been submitted; and agree to be accountable for all aspects of the work.
Disclosure
The authors report no conflicts of interest in this work.
Acknowledgments
No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.
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