Manifestations Of Dermatomyositis Research Articles

S892 Prevalence of Severe Gastrointestinal Manifestations in Dermatomyositis

S892 Prevalence of Severe Gastrointestinal Manifestations in Dermatomyositis

Erythematous, annular, atrophic patchy lesions in bilateral axillae.

Cutaneous manifestations in dermatomyositis often lead to a reduced quality of life. In this article, we report a patient with axillary dermatomyositis, a previously unreported cutaneous involvement of dermatomyositis, to show that we may encounter different sites of involvement.

Deep RNA sequencing of muscle tissue reveals absence of viral signatures in dermatomyositis.

Objective: To explore a possible connection between active viral infections and manifestation of dermatomyositis (DM). Methods: Skeletal muscle biopsies were analyzed from patients diagnosed with juvenile (n=10) and adult (n=12) DM. Adult DM patients harbored autoantibodies against either TIF-1γ (n=7) or MDA5 (n=5). Additionally, we investigated skeletal muscle biopsies from non-diseased controls (NDC, n=5). We used an unbiased high-throughput RNA sequencing (HTS) approach to detect viral sequences. To further increase sequencing depth, a host depletion approach was applied. Results: In this observational study, no relevant viral sequences were detected either by native sequencing or after host depletion. The absence of detectable viral sequences makes an active viral infection of the muscle tissue unlikely to be the cause of DM in our cohorts. Discussion: Type I interferons (IFN) play a major role in the pathogenesis of both juvenile and adult DM. The IFN response is remarkably conserved between DM subtypes classified by specific autoantibodies. Certain acute viral infections are accompanied by a prominent type I IFN response involving similar downstream mechanisms as in DM. Aiming to elucidate the pathogenesis of DM in skeletal muscle tissue, we used deep RNA sequencing and a host depletion approach to detect possible causative viruses.

Complete atrioventricular block as the first manifestation of dermatomyositis

Dermatomyositis is a rare systemic autoimmune disease characterized by primary damage of the skin and striated muscles with the development of chronic muscle weakness. Due to the polymorphic nature of the clinical picture, early diagnosis of dermatomyositis is challenging since patients may visit different specialists depending on predominating symptoms. This entails a late diagnosis and, as a result, the absence of necessary treatment. The article presents a clinical case of a late diagnosis of dermatomyositis, which resulted in the development of autoimmune myocarditis with complete atrioventricular block in a female patient.

First report of an adult patient with Clinical amyopathic dermatomyositis associated rapid progressive interstitial lung disease triggered by COVID19

ILD is a common manifestation of dermatomyositis (DM) with indolent course but a unique variant of DM called clinically amyopathic DM (CADM) presents with rapid progressive interstitial lung disease (RP-ILD) and is called CADM associated RP-ILD. Here, we report a case of a 52 year old woman with CADM associated RP-ILD occurred 2 weeks after recovery from COVID19 infection but unfortunately died from RP-ILD, almost 5 months later. This is the first report of CADM associated RP-ILD triggered by COVID19 in an adult patient

Do histopathological features correlate with systemic manifestations in dermatomyositis? Analysis of 42 skin biopsy specimens from 22 patients.

Understanding whether specific histopathologic features on skin biopsy are predictive of systemic associations in dermatomyositis (DM) would be useful to guide clinical screening. Through retrospective medical record search, clinical and laboratory findings of patients with DM were documented. Existing skin biopsy slides were re-reviewed blindly. Of all biopsy specimens (n=42), the most frequent histopathological finding was vacuolar interface dermatitis (95%). Other features included perivascular lymphocytic infiltrate (71%), increased dermal mucin (40%), vessel wall thickening (12%), follicular plugging (9.5%), and dermal sclerosis (7%). Neutrophilic infiltrate was observed in three biopsies from a patient with adalimumab-associated DM. Vasculitis was not observed. There was no statistically significant difference in the presence of any histopathological feature and that of various systemic manifestations (i.e., myopathy, interstitial lung disease [ILD] and malignancy). However, we observed that dense lichenoid infiltrate rather than pauci-inflammatory changes correlated with severe itching (p < 0.001). Patients with MDA-5 antibodies were significantly more likely to have vasculopathy than those without (p= 0.029*). No dermatopathologic feature was reliably predictive of myopathy, ILD, or malignancy. This finding implies that, regardless of histopathologic findings, patients should be screened for associated conditions as clinically indicated.

Hyperbaric oxygen therapy in the adjuvant treatment of cutaneous manifestations in dermatomyositis: a case report

Cutaneous manifestations of dermatomyositis are unusual and difficult to treat. This study aimed to report a case of cutaneous manifestations of dermatomyositis treated with hyperbaric oxygen. We present a case of dermatomyositis in a 44-year-old female with pain ulcers in her left leg for 17 months, refractory to an exclusive clinical treatment, who underwent a hyperbaric oxygen therapy (HBOT) breathing O2 100%, 90 minute sessions, six days a week, at 2.4 ATA. HBOT therapy proved to be highly efficacious in wound healing in this case and HBOT should be considered as a treatment in the assistance given to such patients.

Skin Manifestations in Dermatomyositis and Antisynthetase Syndrome

Dermatomyositis and antisynthetase syndrome are among idiopathic inflammatory myopathies (myositis) that have skin lesions. In these disorders, skin symptoms often appear as the initial manifestations, and thus, their evaluation is critical for diagnosis. Two components, "site" and "properties," are important for understanding the skin manifestations in myositis. The rash in these disorders is induced by Koebner's phenomenon, and is most frequently observed on the hands and face. Recent studies have shown that myositis-specific autoantibodies are useful tools for the diagnosis and classification of these diseases, as they are closely correlated with the clinical and histopathological phenotypes of myositis. It has recently become clear that each antibody is associated with characteristic skin manifestations, especially related to the properties of the skin lesions.

Anti‐Mi‐2 antibody titers and cutaneous manifestations in dermatomyositis

AbstractThe anti‐Mi‐2 antibody is a dermatomyositis (DM)‐specific autoantibody. It is thought to be associated with photosensitivity in patients with DM because higher expression levels of Mi‐2 antigens have been found in UV‐irradiated cultured cells and because epidemiological studies have supported this association. This study aims to identify the relations between anti‐Mi‐2 antibody titers measured by ELISA and clinical characteristics in patients with DM. We focused on analyzing associations between anti‐Mi‐2 antibody titers and cutaneous manifestations. This retrospective analysis covered 160 adult DM patients. Anti‐Mi‐2 antibodies were detected by an in‐house ELISA, and anti‐Mi‐2 titers were measured by a manufactured ELISA. Twelve of the 160 (7.5%) patients were anti‐Mi‐2 positive. The anti‐Mi‐2‐positive patients showed extremely elevated muscle enzyme levels (P &lt; 0.0001), less frequent interstitial lung disease complication (P = 0.031), and favorable survival rate (100%) (P = 0.07). No correlations were found between anti‐Mi‐2 antibody titers and clinical characteristics or skin manifestations, including photosensitive rashes. Further investigation is necessary to elucidate the pathological significance of the anti‐Mi‐2 antibody in DM.

Juvenile Onset Clinically Amyopathic Dermatomyositis Presenting as Calcinosis Cutis

Dermatomyositis (DM) is an autoimmune inflammatory disorder characterized by involvement of muscles and skin. Classical dermatomyositis (CDM) patients display the hallmark cutaneous manifestations of dermatomyositis (DM), proximal muscle weakness, and laboratory evidence of myositis. Rarely, DM presents with cutaneous features of the disease without muscle involvement for a period of more than six months. Such cases are classified into a category of clinically amyopathic dermatomyositis (CADM) which includes amyopathic DM and hypomyopathic DM. We present a case of a 14-year-old child who presented with calcinosis cutis and cutaneous findings suggestive of the disease without muscle involvement. The child was diagnosed as a case of juvenile onset CADM.J Enam Med Col 2018; 8(2): 101-104

Case Report: Acute amyopathic dermatomyositis presenting with isolated facial edema.

A 45-year-old Asian man presented with acute-onset periorbital and facial edema associated with pyrexia. Muscle weakness was absent. Initial laboratory investigations showed an inflammatory reaction, while screening for infections was negative. Serum muscle enzyme levels were normal. He was hospitalized and treated empirically with antibiotics and corticosteroids, pending the result of facial skin and muscle biopsy. He showed a good clinical and laboratory response but an attempt to discontinue corticosteroids led to a prompt relapse of facial edema and pyrexia, associated with rising laboratory indices of inflammation. Biopsy findings were typical of dermatomyositis. Reintroduction of corticosteroid treatment resulted in complete clinical and laboratory remission. Facial edema as the sole clinical manifestation of dermatomyositis is extremely rare. There have been no previous reports of isolated facial edema in the setting of acute amyopathic dermatomyositis. A high level of suspicion is required to make the diagnosis in the absence of myopathy and the hallmark cutaneous manifestations of the disease (heliotrope rash, Gottron papules).

Heart attacks during dermatomyositis: case of a young Guinean 30 years

Abstract Summary: The objective of this study is to describe the cardiovascular manifestations of dermatomyositis in a 30-year-old male and the therapeutic management. Observation: This is

Hydroxychloroquine for autoimmune diseases

Hydroxychloroquine (HCQ) is by far the most frequently used antimalarial for the management of Systemic Autoimmune Diseases. It has immunomodulatory, hypolipidemic, hypoglycemic and antithrombotic properties and it diminishes the risk of malignancies. The most important mechanisms to explain the immunomodulatory actions are its ability to reduce inflammatory pathways and Toll-like receptors activation. The safety profile is favorable. In spite of its low frequency, retinal toxicity is potentially severe. In systemic lupus erythematous HCQ therapy reduces activity, the accrual of organ damage, risk of infections and thrombosis and improves the cardiometabolic profile. It contributes to induce lupus nephritis remission, spares steroid use and increases survival rates. In rheumatoid arthritis, it improves cardiometabolic risk and has a favorable effect in joint inflammation. In Sjögren's syndrome, an increased lacrimal quality as well as an improvement in objective and subjective inflammatory markers has been demonstrated with HCQ. In Antiphospholipid Syndrome, HCQ is effective in primary and secondary thrombosis prevention. The effectiveness of the drug in other systemic autoimmune diseases is less established. HCQ therapy may improve dermatological manifestations in Dermatomyositis and may have a positive effects in the treatment of Sarcoidosis and Still disease.

Cutaneous Manifestations in Dermatomyositis: Key Clinical and Serological Features-a Comprehensive Review.

Dermatomyositis (DM) is a common idiopathic inflammatory myopathy. The pathogenesis is considered to be microangiopathy affecting skin and muscle. The cutaneous manifestations of DM are the most important aspect of this disease, and their correct evaluation is important for early diagnosis. The skin signs are various: Some are pathognomonic or highly characteristic, and others are compatible with DM. Recently, DM has been categorized into several disease subsets based on the various autoantibodies present in patients. Sometimes, characteristic cutaneous manifestations are strongly associated with the presence of specific autoantibodies. For example, anti-Mi-2 antibody is associated with the classic features of DM, including heliotrope rash, Gottron's papules, the V-neck sign, the shawl sign, cuticular overgrowth, and photosensitivity. Frequent cutaneous features in anti-transcriptional intermediary factor 1 gamma (TIF1γ)-positive patients are diffuse photoerythema, including "dusky red face," while skin ulcerations, palmar papules (inverse Gottron), diffuse hair loss, panniculitis, and oral pain and/or ulcers are sometimes associated with anti-melanoma differentiation-associated gene 5 product(MDA5) antibody. Here, we review important cutaneous manifestations seen in patients with DM, and we examine the relationship between the skin changes and myositis-associated autoantibodies. Correct evaluation of cutaneous manifestations and myositis-associated autoantibodies should help the clinician in the early diagnosis of DM, for a quick recognition of cutaneous signs that may be the symptom of onset before muscle inflammation.

Calcific panniculitis and nasopharyngeal cancer-associated adult-onset dermatomyositis: a case report and literature review.

Panniculitis is an uncommon cutaneous manifestation in dermatomyositis. It not only occurs in idiopathic dermatomyositis, but also rarely occurs in the setting of malignancy-associated dermatomyositis. Calcinosis cutis is also less likely to be found in adult-onset dermatomyositis. In malignancy-associated dermatomyositis, panniculitis can precede, concur, or follow the diagnosis of malignancy. We report the case of a 26-year-old Thai female with calcific panniculitis in the setting of adult-onset dermatomyositis associated with nasopharyngeal cancer. The clinical course of calcific panniculitis in our case was not parallel to the course of malignancy. Calcific panniculitis can appear many years after, despite the remission of the cancer. A thorough review of the previously reported literature is also provided.

Interstitial lung disease as an initial manifestation of dermatomyositis

To explore the clinical features and prognosis of dermatomyositis patients with interstitial lung disease (ILD) as an initial manifestation. Medical records of 184 dermatomyositis inpatients complicated with ILD, admitted into Peking Union Medical College Hospital from January 1999 to January 2013, were retrospectively analyzed. The clinical features, biochemical parameters, positive rates of autoantibodies, radiology, pulmonary function tests, pathology, treatments and prognosis were compared between two subgroups of ILD-initial and non-ILD-initial dermatomyositis. The incidence of ILD of dermatomyositis inpatients was 17%. The average age was 48 ± 12 years and the gender ratio of male-to-female was 63: 121. Eighty eight (47.8%) dermatomyositis patients had ILD as an initial manifestation, including (n = 42, 22.8%) of ILD concomitant dermatomyositis (within 1 month) and (n = 46, 25.0%) of ILD before dermatomyositis with an average ahead time of (11 ± 3) months. Patients of ILD-initial dermatomyositis had a higher incidence of dyspnea on exertion, cough and lung crackles, but there were lower incidences of heliotrope rash, chest V area rash, shawl sign and joint involvement than non-ILD-initial dermatomyositis (P < 0.05). The positive rate of anti-Jo-1 antibodies of ILD-initial dermatomyositis group was 13.6%. The main performances of ILD-initial dermatomyositis on pulmonary function tests were diffusing and restrictive ventilation impairment. And there was a lower diffusing rate of carbon monoxide than non-ILD-initial dermatomyositis group (P < 0.01). Organic and non-specific interstitial pneumonias were the major clinical pathology types of ILD-initial dermatomyositis. The mortality rate of ILD-initial dermatomyositis patients was 19.3% and there was no significant difference from non-ILD-initial dermatomyositis (P > 0.05). The main course of ILD-initial dermatomyositis was respiratory failure due to progressive ILD (n = 13, 76.5%). ILD as an initial manifestation is a common complication and a major mortality cause of dermatomyositis inpatients. And the frequent clinical pathology types are organic and non-specific interstitial pneumonias. Symptoms of skin and muscle, creatine kinase and anti-synthetase antibodies should be closely monitored.

Cutaneous manifestations of dermatomyositis in male patient: a rare report

Introduction: Dermatomyositis is an idiopathic inflammatory myopathy. It is a systemic disorder that most frequently affects the skin and muscles, but may also affect the joints, the esophagus, the lungs, and, less commonly, the heart. Case report: It is presented with characteristic cutaneous findings like skin rash as well as progressive symmetrical proximal muscle weakness. Its prevalence rate is approximately one per 100,000 in the general population with a female to male predominance of about 2:1. Conclusion: We report a male patient with the classical features of dermatomyositis in whom cutaneous changes preceded muscle weakness.

Manifestations of dermatomyositis in otorhinolaryngology

皮肌炎（dermatomyositis，DM）是一种自身免疫性结缔组织疾病，横纹肌呈坏死性病变，同时伴有多种形态的皮肤损害，可并发各种恶性肿瘤，尤以合并鼻咽癌多见。一直以来，自身免疫性疾病合并鼻腔、咽喉部损害并未得到足够的重视。本文回顾性分析97例DM患者鼻、鼻咽、咽喉纤维内镜检查资料，了解皮肌炎患者鼻、鼻咽、咽喉部的表现，为临床诊治提供一定的参考依据。

Dermatomyositis with a Pityriasis Rubra Pilaris‐like Eruption: An Uncommon Cutaneous Manifestation in Dermatomyositis

A pityriasis rubra pilaris-like eruption has been described in patients with dermatomyositis. We describe an 11-year-old girl with dermatomyositis who had additional clinical findings of pityriasis rubra pilaris. Over a year, she developed muscle weakness, increasing fatigue, and a markedly elevated creatinine kinase level in addition to her cutaneous eruption and was seen in our clinic for these complaints. A year earlier, when a generalized, scaly erythematous eruption had appeared, she had been diagnosed as pityriasis rubra pilaris clinically and histopathologically. Dermatologic examination found scaling erythematous plaques involving the trunk and upper and lower extremities. Islands of unaffected skin were intermingled with erythematous plaques that were characteristic of pityriasis rubra pilaris. A skin biopsy specimen showed the findings of dermatomyositis and that diagnosis was made. The laboratory findings, electromyographic pattern, and muscle biopsy were also consistent with dermatomyositis. Her presentation is interesting, as she had been diagnosed as pityriasis rubra pilaris both clinically and histopathologically 1 year earlier and, although the cutaneous lesions had not changed, a diagnosis of dermatomyositis was made a year later.

Incidental Granular Parakeratosis Associated With Dermatomyositis

A 43-year-old patient presented with a 4-month history of intermittently occurring symmetrical and slightly scaling erythematous plaques in his zygomatic regions. Because of polymyositis in the patient's history, the skin lesions were thought to be a manifestation of dermatomyositis. The skin biopsy revealed a sparse superficial perivascular infiltrate of lymphocytes, which was associated with telangiectases and both discrete vacuolar alteration and smudging of the dermal-epidermal junction. These findings were consonant with dermatomyositis. In addition to these features focal granular parakeratosis was present. The histopathologic presence of granular parakeratotic corneocytes above a zone of orthokeratosis and the absence of clinical features of granular parakeratosis indicate the granular parakeratosis is incidental.