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a Heliotrope eruption: a blue-purple discoloration on the upper eyelids can be observed. b Shawl sign/V-sign skin rash around the neck.
Source publication
A 76-year-old woman with muscle ache, weakness of the extremities, and skin rash was diagnosed with dermatomyositis (DM). Upon the diagnosis of DM, a systemic survey of malignancy revealed an advanced carcinoma of the ascending colon. The patient underwent right hemicolectomy approximately 2 months after the onset of DM. The symptoms and signs of D...
Contexts in source publication
Context 1
... Her heart had a regular rate and rhythm without murmur. The lungs were clear to auscultation without wheezing or rales. Her abdomen was soft and flat with a normal bowel sound. She had a purple rash on her arms, chest, lumbar back, and hip. In summary, she had a heliotrope eruption (a blue-purple discoloration on the upper eyelids; fig. 1a), Gottron's sign (a raised violaceous rash or papules on the knuckles prominent in the metacarpophalangeal and in- terphalangeal joints), a skin eruption on the extensor surfaces of the extremities, and a shawl sign/V-sign skin rash around the neck (fig. ...
Context 2
... summary, she had a heliotrope eruption (a blue-purple discoloration on the upper eyelids; fig. 1a), Gottron's sign (a raised violaceous rash or papules on the knuckles prominent in the metacarpophalangeal and in- terphalangeal joints), a skin eruption on the extensor surfaces of the extremities, and a shawl sign/V-sign skin rash around the neck (fig. ...
Citations
... DM is considered a part of paraneoplastic syndrome [3,6,7], meaning it is a clinical syndrome associated with an underlying malignancy owing to the production of antibodies [6]. Paraneoplastic syndromes affect any part of the nervous system, neuromuscular junction, and muscles. ...
... DM is considered a part of paraneoplastic syndrome [3,6,7], meaning it is a clinical syndrome associated with an underlying malignancy owing to the production of antibodies [6]. Paraneoplastic syndromes affect any part of the nervous system, neuromuscular junction, and muscles. ...
... The antibodies which indicated a higher risk of malignancy were: transcription intermediary factor 1γ (TIF-1γ) [5], and nuclear matrix protein NXP-2 [3,5,8,9]. About a third of patients diagnosed with DM are diagnosed with cancer during its clinical course [6], with adenocarcinomas of the lung, stomach, pancreas, colon, and pelvis being most common [2,4,6,10]. The malignancy is most likely diagnosed simultaneously with or shortly after the diagnosis of myopathy has been made [10][11][12]. ...
Dermatomyositis (DM) is a rheumatological disorder characterized by proximal myositis and distinctive dermatological manifestations. It can be an isolated clinical syndrome or, in rarer cases, can be the initial presentation for an underlying malignancy as a part of a paraneoplastic syndrome. In this case report, we describe a case of a 51-year-old lady who presented with proximal myopathy, typical DM skin rash, dysphagia, and markedly elevated creatine kinase. She was diagnosed with a seronegative DM and her malignancy screening revealed a mass in the ascending colon. During her hospital course, she also developed microangiopathic hemolytic anemia, another paraneoplastic disorder typically associated with late stages of malignancy, manifested as hemolytic anemia, thrombocytopenia, and low fibrinogen. The patient received intravenous corticosteroids and underwent tumor resection with following resolution of her both rheumatological and hematological manifestation. Unfortunately, due to her general poor health, she developed sepsis and died in the hospital.
... Su incidencia en Estados Unidos es de 5 a 10 por 1 millón de habitantes y tiene predilección por el sexo femenino en relación 3:1. El pico de incidencia se da entre los 40 y 60 años de edad (8). La asociación de dermatomiositis y malignidad fue establecida por primera vez en 1916 en un paciente con cáncer gástrico. ...
Los pacientes con dermatomiositis presentan un mayor riesgo de asociación con patología maligna subyacente, sin conocerse una causa precisa de esta relación. Son múltiples las posibles localizaciones anatómicas, entre las que se incluyen los ovarios, mamas, zona gástrica, zona colorrectal, sangre, pulmones y próstata. Se presenta el caso de una mujer de 58 años que durante el estudio de pérdida anormal de peso manifestó debilidad muscular grave y alteraciones dermatológicas asociadas con el hallazgo de adenocarcinoma del colon transverso.
... Dermatomyositis (DM) is an inflammatory myopathy characterized by skin rash and progressive, symmetrical weakness of the proximal muscles [1,2]. DM has been shown to be associated with malignant disease [3]. ...
Background
Anti-transcriptional intermediary factor 1 gamma (TIF1γ) antibody is a marker for predicting cancer association in patients with dermatomyositis (DM). The overall survival rate in DM patients with cancer was reported to be considerably worse than that in DM patients without cancer. However, the treatment for cancer-associated DM remains controversial, because the treatment priority between surgical resection for the tumor and internal treatments, including glucocorticoids, immunosuppressive agents, and intravenous immune globulin, has not been established.
Case presentation
We report the case of a 57-year-old Japanese man diagnosed with anti-TIF1γ antibody-positive DM associated with ascending colon cancer. His clinical symptoms included facial and brachial edema, muscle weakness, dysphagia, myalgia, and rash. Physical examination revealed periorbital edema and Gottron's papules over his knuckles with brachial edema, and tenderness and weakness of the proximal limb muscles. The findings of hyperintense muscles in T2-weighted sequences of brachial contrast-enhanced magnetic resonance imaging and the infiltration of lymphocytic cells and CD4-positive lymphocytes from muscle biopsy were compatible with the diagnostic criteria for dermatomyositis. Anti-TIF1γ antibody was positive by immunoprecipitation assay. He first started internal treatment including intravenous immunoglobulin, steroid pulse, prednisolone, and azathioprine, followed by surgical resection for the tumor because of the elevation of creatine kinase and progression of dysphagia. However, clinical symptoms did not improve, and the patient died 6 months later.
Conclusions
We faced difficulties in determining the treatment priority between surgical resection and internal treatment for our case; therefore, this case would be educational for readers. We searched PubMed to identify English-language case reports of anti-TIF1γ antibody-positive dermatomyositis with malignancy and found 21 reported cases. We herein review and summarize previously reported cases of anti-TIF1γ antibody-positive DM with malignancy. Cancer screening is essential in patients with anti-TIF1γ antibody-positive dermatomyositis because it is associated with a high prevalence of malignancies. Our review revealed that initial surgical treatment should be recommended for better prognosis if the general condition allows.
... The principal characteristics are skin rash, muscle ache as well as muscle weakness, while the absence of cutaneous manifestations are recognized as polymyositis [1]. The incidence of DM is 5 to 10 per one million in the USA, with a predilection to the female sex (3:1) and with a major age peak at 40 to 60 years old [2]. The principle trigger of the inflammatory process is the activation and deposition of components of the complement system, causing lysis of endomuscular capillaries, subsequently leading to muscle ischemia and further inflammation [1]. ...
... The available data from case reports referring to patients with DM, and colorectal cancer are summarized in Table 1; data for 27 patients from 27 case reports are presented [2,. The median age of the patients was 65 years (range 40-82), the majority of which were female (17 out of 27, 63%). ...
Background:
Dermatomyositis (DM) is an idiopathic inflammatory myositis. The principal characteristics are cutaneous rash, muscle ache, and muscle weakness. In the past, associations have been established between DM and malignancy, including colorectal cancer.
Methods:
A systematic PubMed and Scopus search was conducted.
Results:
The median age of the patients was 65 years (range 40-82). The majority were female (17 out of 27, 63%). Adenocarcinoma was the most frequent histological type of colorectal neoplasm. DM manifested before the diagnosis of colorectal cancer in 21 out of 27 patients (77.8%). At the time of the first presentation, creatine kinase was at a median level of 514.5 U/L (range 50-11,744), and serum antibodies were present in 11 out of 27 patients (40.7%). Immediate improvement of DM symptoms after surgery occurred in 14 out of 26 patients (53.8%). Recurrence of cancer in the form of distal metastasis was present in 5 out of 26 patients (19.2%). Cancer recurrence occurred within a median of 7.9 months (range 2-21) after surgery. In 7 out of 26 patients (26.9%), DM symptoms recurred during the post-operative period. Death was reported in 23 out of 27 patients (85.2%).
Conclusion:
It is of paramount importance to perform a systematic diagnostic workup for malignancy, always including colonoscopy, in DM patients, since there is a high incidence of cancer in DM patients. Surgical treatment of colorectal tumors should precede the treatment of DM, as DM will frequently regress after a successful resection of malignancy.
... Prior case reports have documented resolution of DM after treatment of underlying neoplasm [24,25]. Unfortunately, given the poor clinical condition many patients with paraneoplastic DM present with, surgical resection or chemotherapy may not be tolerated. ...
Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association.
Background:Several studies have reported a clinical association between gastric cancer(GC) and dermatomyositis (DM), but the molecular features and underlying mechanisms between the two diseases have not been investigated.
Methods：We obtained the strongly associated genes of DM and GC and the clinical characteristics from the Gene Expression Omnibus (GEO), The Comparative Toxicogenomics Database (CTD), GeneCards, and DisGeNET databases. We next screened hub genes, constructed co-expression and interaction networks, transcription factor-gene-miRNA regulatory networks, and performed enrichment analysis of cell signaling pathways and candidate drugs prediction. Finally, a single-gene immune infiltration assay was performed on the hub genes.
Results：Our study revealed commonalities at the genetic level between DM and GC. A deep dive into the 8 hub genes revealed the role in immune response, especially cytokines, which were involved in the co-development of the two diseases. The obtained hub genes have the potential to be biomarkers as well as therapeutic targets for DM patients with a potential predisposition to GC tumorigenesis.
Dermatomyositis is a rare autoimmune disorder that can be idiopathic or present as a paraneoplastic syndrome. We report the case of an 83-year-old with anti-TIF-1-antibody-positive dermatomyositis, which led to the identification of a presumed left lower lobe T3 malignancy. Dramatic symptom improvement was noted after stereotactic body radiotherapy to the lesions, after no initial response to prednisone and IVIG. Recurrence of his skin findings at 6 month follow-up coincided with recurrent disease, biopsy proven for small cell lung cancer.
A 65-year-old man presented with muscle pain and finger swelling, and was given a diagnosis of anti-MDA5 antibody-positive dermatomyositis and interstitial pneumonia. Colonoscopy, as a detailed examination for malignancy detected descending colon cancer (cStage IIIa), and laparoscopic left hemicolectomy was performed. On postoperative day 4, hypoxemia developed and acute exacerbation of the interstitial pneumonia was diagnosed. We started steroid pulse therapy with cyclosporin A and cyclophosphamide. On postoperative day 7, he developed abdominal pain. CT revealed retroperitoneal and intra-abdominal gas. We considered the possibility of anastomotic leakage, nevertheless, there was no tenderness or change in vital signs. We diagnosed this as intra-abdominal gas spreading from mediastinal emphysema and gave conservative treatment with antibiotics. The clinical course was uneventful and the patient was discharged on postoperative day 41. Anti-MDA5 antibody-positive dermatomyositis is often associated with rapidly progressive interstitial lung disease. Surgical cases for malignancy with this disease should be carefully treated.
