Paraneoplastic Dermatomyositis Research Articles

Paraneoplastic dermatomyositis and Stevens-Johnsonsyndromerelated to immunotherapy.

Paraneoplastic syndromes such as dermatomyositis, often emerge as the initial clinical manifestation across various cancer types and are characterized by the development of B-cell responses targeting cancer-cell antigens that cross-react with normal skin and muscle cells. While these syndromes may alleviate following antineoplastic intervention, their response to immunotherapy remains elusive due to the exclusion of patients with autoimmune phenomena from clinical trials. In this report, we present the case of a female patient with advanced urothelial cancer presenting with dermatomyositis, who subsequently underwent treatment with anti-PD1 immunotherapy and experienced the onset of Stevens-Johnson syndrome. We discuss these two autoimmune entities and provide a comprehensive review of the existing literature to elucidate similar associations.

Sequence Alignment between TRIM33 Gene and Human Noncoding RNAs: A Potential Explanation for Paraneoplastic Dermatomyositis.

This computational analysis investigated sequence complementarities between the TRIM33 gene and human noncoding (nc)RNAs and characterized their interactions in the context of paraneoplastic dermatomyositis. TRIM33 FASTA sequence (NCBI Reference Sequence: NC_000001.11) was used for BLASTN analysis against Human GRCh38 in the Ensembl.org database. Retrieved ncRNAs showing hits to TRIM33 were searched in the GeneCards.org database and further analyzed through RNAInter, QmRLFS-finder, Spliceator, and NcPath enrichment analysis. A total of 100 hits were found, involving the lncRNAs NNT-AS1, MKLN1-AS, LINC01206, and PAXBP1-AS1, whose dysregulation has been reported in either cancer or dermatomyositis. Additionally, the lncRNAs NNT-AS1 and PAXBP1-AS1 may interact with microRNA-142-3p, reducing its expression and increasing that of TRIM33. Sequence complementarity affected only TRIM33 intron 1, possibly resulting in alternatively spliced isoforms of TIF1γ with increased immunogenicity. The results also revealed nucleotide alignment between TRIM33 and the gene regulatory elements of 28 ncRNA genes involved in immune pathways. This pivotal study demonstrates sequence complementarity between TRIM33 and human ncRNAs dysregulated in cancer and dermatomyositis. This scenario may lead to the overproduction of more immunogenic TIF1γ variants in tumors and the stimulation of autoimmunity. Further experimental analyses using targeted methods such as Western blot or Chip-Seq are required to confirm these data.

Anti-transcription intermediary factor 1 gamma antibody-associated paraneoplastic dermatomyositis: A case report

Dermatomyositis (DM) is an inflammatory myopathy characterized by distinctive cutaneous manifestations. Recent advancements have led to the identification of a novel myositis-specific autoantibody called anti-transcription intermediary factor 1 gamma (anti-TIF-1-γ) which presents with unique cutaneous manifestations and a heightened risk for malignancy. We report a case of a 55-year-old female who presented with proximal muscle weakness, an erythematous rash over the face, blanching erythema over the nape of the neck, and red-on-white lesions over the chest. She was diagnosed with anti-TIF-1-γ positive DM and found to have a palpable mass in the right breast. Biopsy showed triple negative invasive breast carcinoma no special type (NST) grade 3. She showed poor response to steroids and was referred for cancer staging and treatment. The anti-TIF-1-γ antibody serves as a novel tool to identify a subset of DM patients at high risk for cancer. At present, it’s role in the prognosis or surveillance of cancer recurrence remains uncertain.

A Case of a 50-Year-Old Female with Dermatomyositis Secondary to a Paraneoplasm (Infiltrating Ductal Carcinoma, Grade 2)

Introduction: Dermatomyositis is an autoimmune disorder with an incidence of 5 to 10 per 1 million per year. 10 to 20% of patients with adult-onset Dermatomyositis have an underlying malignancy. There are currently no specific cutaneous markers for Paraneoplastic Dermatomyositis. This case highlights a patient with uncommon features of paraneoplasm after breast malignancy. Case Report: A 50-year-old female with Infiltrating Ductal Carcinoma, presented with patches on the face, neck, chest, upper back, outer arms, dorsal hands, thighs, hair loss and weakness. Histopathology revealed widened basal layer vacuolization with widened spaces between collagen bundles filled with mucin deposition, and pigment incontinence in the papillary dermis. Direct immunofluorescence showed granular deposits of IgG, C3, IgM and IgA in the basement membrane zone, which are consistent with Dermatomyositis. The patient was prescribed with Clobetasol ointment mixed equally with petrolatum on the body and Mometasone cream on the face. She was subsequently started on Prednisone at 0.5 mkd with eventual tapering, Omeprazole, and Calcium + Vitamin D tablet daily. Hydroxychloroquine 200 mg/tab daily was added. Referral to Rheumatology for co-management and Surgery for management of the breast mass was also done. Conclusion: This case emphasizes the rare occurrence of a Paraneoplastic Dermatomyositis following a diagnosis of breast malignancy. A careful review upon diagnosis may detect occult malignancies especially in patients who present primarily with cutaneous Dermatomyositis. Clinicopathologic correlation and a multi-disciplinary approach, with Surgery, Oncology, Rheumatology and Dermatopathology are needed to identify the underlying cause and facilitate successful treatment of a neoplasm followed by improvement of Dermatomyositis.

Paraneoplastic dermatomyositis associated with urothelial cancer: report of a case and systematic review of the literature.

The idiopathic inflammatory myopathies (IIM) are a collection of autoimmune diseases that have a substantial impact on the entire body and include conditions such as dermatomyositis (DM), polymyositis (PM), sporadic inclusion body myositis, and immune-mediated necrotizing myopathy. These disorders are characterized by symptoms such as muscular weakness, pain, and dermal rash. This systematic review is intended to explore the potential link between bladder cancer and DM/PM. We performed a comprehensive systematic search on PubMed and Scopus until August 2022 to identify relevant research studies. The studies that met our inclusion criteria focused on patients with urinary bladder cancer and dermatomyositis, and/or polymyositis. The patients' median age was 65.5 years (47-79), with the majority being male (15, 39.47%). Bladder cancer manifested before PM/DM in 5 (13.15%) patients, while in the majority of cases occurred after the cancer diagnosis. The stage of cancer at the time of the initial PM/DM diagnosis were mostly locally (11/20, 50%).During the first presentation, the patients had a median creatine kinase level of 2227 U/L, ranging between 44 and 10471. In one case, anti-TIF-1γ antibodies were found to be present. Among the cases with reported medical history (20/38), treatment immediately improved DM symptoms in 16 patients(53.8%) and in 3 patients(15%), symptoms of DM resurfaced during the period after the operation. Death was reported in 14 (36.8%) patients. In conclusion, our study provides knowledge and understanding for identifying specific risk factors in patients with the coexistence of bladder cancer and DM/PM and their management. During the initial and follow-up screening, age, gender, and the clinicopathological subgroup of myositis should be considered to ensure proper management of the condition.

Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder: A clinical and histologic retrospective cohort study

Clonally restricted, non-epidermotropic, low-grade, CD8-positive T-cell infiltrates of the skin was recognized as a unique form of indolent CD8-positive lymphoproliferative disease in 2007 when it was first called primary cutaneous indolent CD8-positive lymphoid proliferation. More recently, the designation of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder has been used. It is unique as a cutaneous lymphoproliferative disorder because of relative uniformity in its clinical presentation and histomorphology. It has been recognized as having an interesting predilection for the ear and acral sites, characteristically presenting as a solitary lesion. The basic morphology is one characterized by a non-epidermotropic, tumefactive infiltrate of well-differentiated, noncerebriform, atypical, small- to intermediate-sized lymphocytes that exhibit a specific phenotype characterized by CD8 and TIA positivity in concert with a distinct perinuclear Golgi staining pattern for CD68. The typical presentation is in the context of a solitary lesion, which can be treated surgically or with local irradiation. We describe in detail two very unusual cases that expand the clinical spectrum of this condition given the non-acral localization, the multiplicity of lesions to involve the trunk and extremities, and, in one case, the stable but recalcitrant course over 30 years. In addition, the second patient developed paraneoplastic dermatomyositis. We also retrospectively review our database for other cases that represent the entity of primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder and review the literature focusing on non-acral cases. Nomenclature evolution from its first recognition in 2007 to the present is discussed.

PARANEOPLASTIC DERMATOMYOSITIS REVEALING OVARIAN CANCER: ABOUT A CASE

Dermatomyositis (DMs) is a systemic autoimmune disease affecting mainly muscle and skin. It may be associated with cancer in a paraneoplastic setting with a frequency ranging from 18 to 32% [1]. various types of cancer are described in the literature however, the association with gynecological cancers, especially in women, is more frequent. the incidence of ovarian cancer in dermatomyositis in women is 13.3% it is higher than in the general population (1%) [2]. We present the case of a patient with paraneoplastic dermatomyositis, who presented with a typical clinical symptomatology of paraneoplastic dermatomyositis, confirmed by other complementary examinations (creatine phosphokinase (CPK) + EMG (electromyogram) + skin biopsy). Etiological exploration by the ovarian specific tumor marker CA 125, and pelvic ultrasound completed by pelvic MRI oriented the diagnosis towards ovarian adenocarcinoma. Our results suggest that a complete gynecological workup including gynecological clinical examination, CA 125, CA 15-3 and pelvic ultrasound should be routinely performed in any woman over 40 years of age with DM.

A Rare Case of Paraneoplastic Dermatomyositis in a Patient With Metastatic Endometrial Cancer

Dermatomyositis (DM) is a rare autoimmune myopathy that presents with muscle weakness and characteristic skin manifestations. Certain malignancies are associated with DM, such as stomach, colorectal, ovarian, lung, pancreatic, and non-Hodgkin lymphoma. However, there are only rare reports of endometrial cancer with DM. In this report, we describe a case of anti–TIF-1-gamma and anti-Mi-2 antibody positive DM in a patient with stage IV endometrial cancer. This case illustrates the importance of considering DM as a paraneoplastic syndrome regardless of both stage and type of malignancy.

S2309 Esophageal Adenocarcinoma Causing Paraneoplastic Dermatomyositis: Preventing Delayed Diagnosis Is Key

S2309 Esophageal Adenocarcinoma Causing Paraneoplastic Dermatomyositis: Preventing Delayed Diagnosis Is Key

Is a Triggering Role or a Causative Role of PD-1/PDL-1 Inhibitors in the Development of Paraneoplastic Dermatomyositis?

Is a Triggering Role or a Causative Role of PD-1/PDL-1 Inhibitors in the Development of Paraneoplastic Dermatomyositis?

Paraneoplastic Dermatomyositis Revealing Tardly an Ovarien Cancer at Metastatic Stage who Responded Favorably to Treatment

Dermatomyositis is a type of inflammatory myopathy characterized by inflammatory and degenerative changes of the muscles and skin. The paraneoplastic dermatomyositis is a subtype associated with cancer. The pathophysiothogy remains unfully understood. The diagnosis is supported by both the clinical and serological features. The treatment involves both the DM and the malignancy. Multi-disciplinary management is fundamental for patients. However the pronosis remains usually poor. We report a case of paraneoplastic dermatomyositis revealing an ovarian cancer, the purpose is to illustrate the diagnostic, therapeutic and pronostic of this entity.

Dermatomyositis Triggered by Metastatic Subungueal Melanoma: A Case Report and Review of Literature

Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized predominantly by cutaneous and muscular abnormalities that can be associated with neoplasia. The association of dermatomyositis with nail melanoma is very rare, if not exceptional. We report the case of a paraneoplastic dermatomyositis due to an undiagnosed subungueal melanoma in a 70-year-old patient.

The prognosis of cutaneous malignant melanoma associated with paraneoplastic dermatomyositis

The prognosis of cutaneous malignant melanoma associated with paraneoplastic dermatomyositis

Paraneoplastic Dermatomyositis

A Hispanic man in his 50s—recently diagnosed with acral lentiginous melanoma—presented for evaluation of a tender, photodistributed rash that was first appreciated approximately 10 weeks prior. Review of systems was notable for difficulty lifting objects above his head.

Paper Title of Case Report: Fatal Skin Lesions

We present a clinical case of a 75-year-old woman hospitalized for localized edema and erythema on the right hemiface, with progressive extension over 30 days. On objective examination, a heliotropic erythema, Gottron's papules and an ulcerated lesion on the right breast were founded. It's important to point out an increase of muscle enzymes in laboratory tests. According to classification criteria of the European League against Rheumatism, and taking into consideration the clinical suspicion of breast cancer, a paraneoplastic dermatomyositis was diagnosed. Highlight that dermatomyositis lesions are pathognomonic despite that they are not always recognized in the patient's first approach.

Paraneoplastic dermatomyositis revealing undifferentiated nasopharyngeal carcinoma at early stage: a case report

ContextDermatomyositis is a rare autoimmune disease characterized by noninfectious inflammatory damage of skin and predominant muscles in the belts. It is believed to be associated with about 1 in 1000 cases of nasopharyngeal carcinoma. This association has been described for locally advanced stages II and III nasopharyngeal carcinoma. It has rarely been described in the early stages (stage I).Case presentationA 65-year-old Moroccan patient residing in Casablanca, with no particular history was referred to the Mohamed VI Center for the treatment of cancers of the University Hospital Center IBN ROCHD in Casablanca, for treatment of nasopharyngeal cancer. He was admitted in poor general condition, performance status 3, with erythema on the face, neck, and extremities. The diagnosis of paraneoplastic dermatomyositis was made owing to progressive muscle weakness and elevation of muscle enzymes associated with the typical rash of the face and hands. He received corticosteroid therapy and then radiotherapy to the nasopharynx with good clinical outcome, disappearance of skin lesions, and recovery of muscle strength.ConclusionsWe report this case of dermatomyositis in early-stage nasopharyngeal carcinoma, which is a rarely described entity. Rapid treatment of dermatomyositis improved the patient’s quality of life and enabled him to support specific cancer treatments. This can be used as an element of early diagnosis and monitoring after treatment.

Paraneoplastic Dermatomyositis Revealing Tardly an Ovarien Cancer at Metastatic Stage Who Responded Favorably to Treatment

Dermatomyositis is a type of inflammatory myopathy characterized by inflammatory and degenerative changes of the muscles and skin. The Para neoplastic dermatomyositis is a subtype associated with cancer. The pathophysiothogy remains unfully understood. The diagnosis is supported by both the clinical and serological features. The treatment involves both the DM and the malignancy. Multi-disciplinary management is fundamental for patients. However the pronosis remains usually poor. We report a case of Para neoplastic dermatomyositis revealing an ovarian cancer; the purpose is to illustrate the diagnostic, therapeutic and pronostic of this entity

Paraneoplastic dermatomyositis and prostate cancer: Myopathy regression under cancer-directed therapy.

Prostate cancer is the second most frequent malignancy in men worldwide and the fifth leading cause of death. Dermatomyositis (DM) is a rare idiopathic inflammatory myopathy characterized by musculocutaneous manifestations. However, DM can also present as a paraneoplastic syndrome of an underlying neoplasm. We report a case of a 65-year-old man diagnosed with prostate adenocarcinoma in the setting of severe dysphagia, muscle weakness and a facial erythematous rash. At first, the DM-related symptoms resolved with the initial treatment for the underlying malignancy. Yet, they flared up as the tumor progressed. To sum up, DM is a rare systemic disorder with unknown etiology. There is a well-established association between DM and malignancy. Malignancy-headed therapy can improve DM manifestations and the recurrence of DM symptoms may act as an early warning of malignancy relapse.

Carcinome indifférencié du nasopharynx associé à une dermatomyosite paranéoplasique à un stade précoce T1N0M0 : A propos d’un cas et revue de la littérature

La dermatomyosite (DM) est une maladie auto-immune rare, caractérisée par une atteinte inflammatoire non infectieuse de la peau et des muscles prédominants aux ceintures. Elle serait associée à environ un cas sur 1.000 au carcinome nasopharyngé (NPC). Cette association a été décrite pour les stades localement avancés, stade II et stade III du NPC. Peu décrite à des stades précoces (stade I), sa symptomatologie est souvent révélatrice du cancer dont le diagnostic repose sur cinq critères : la faiblesse musculaire des membres proximaux, l’élévation des enzymes musculaires sériques, les anomalies musculaires histologiques à la biopsie musculaire, l’anomalie électromyographique et la manifestation dermatologique inflammatoire clinique. Le traitement repose sur la corticothérapie et le traitement étiologique du cancer. Le pronostic des NPC chez les patients atteints de DM paranéoplasique est comparable aux patients non atteints. Nous rapportons ce cas de DM révélant un NPC à un stade précoce et nous faisons le point sur l’état des connaissances sur la DM paranéoplasique associée à un NPC rarement décrite. En effet, au Maroc la dernière description d’un cas remonte en 2016.

Ulcerative Paraneoplastic Dermatomyositis in the Setting of Positive Transcriptional Intermediary Factor 1-γ Antibody.

Dermatomyositis (DM) is an autoimmune inflammatory disease of the skin with or without muscle involvement1 One-third of patients present with malignant disease within 3 years2.