scholarly journals Anasarca as the presenting symptom of juvenile dermatomyositis: a case series

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Emily E. Schildt ◽  
Deirdre De Ranieri
Keyword(s):  
Juvenile Dermatomyositis ◽  
Early Recognition ◽  
Case Series ◽  
Atypical Presentation ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rare Presentation ◽  
Case Presentation ◽  
Patients Âgés ◽  
Rare Manifestation

Abstract Background Juvenile Dermatomyositis (JDM) is an autoimmune disease that typically presents with classic skin rashes and proximal muscle weakness. Anasarca is a rare manifestation of this disease and is associated with a more severe and refractory course, requiring increased immunosuppression. Early recognition of this atypical presentation of JDM may lead to earlier treatment and better outcomes. Case presentation We present two female patients, ages 11 years old and 4 years old, who presented to the ED with anasarca and were subsequently diagnosed with JDM. Both patients required ICU-level care and significant immunosuppression, including prolonged courses of IV methylprednisolone, IVIG, and Rituximab. Conclusions Anasarca is a rare presentation of Juvenile Dermatomyositis, but it is important for clinicians to recognize this manifestation of the disease. Early recognition and treatment will lead to better outcomes in these children and hopefully decrease the need for prolonged hospitalization and ICU level care.

scholarly journals The Significance of Autoantibodies in Juvenile Dermatomyositis

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Dominika Kwiatkowska ◽  
Adam Reich
Keyword(s):  
Juvenile Dermatomyositis ◽  
Immunosuppressive Agents ◽  
Treatment Strategies ◽  
Case Series ◽  
Autoimmune Disorder ◽  
Current Treatment ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Cutaneous Lesions

Juvenile dermatomyositis is a chronic and rare autoimmune disorder classified into the spectrum of idiopathic inflammatory myopathies. Although this entity is mainly characterized by the presence of pathognomonic cutaneous lesions and proximal muscle weakness, the clinical manifestation can be highly heterogeneous; thus, diagnosis might be challenging. Current treatment recommendations for juvenile dermatomyositis, based mainly upon case series, include the use of corticosteroids, immunomodulatory, and immunosuppressive agents. Recently, several specific autoantibodies have been shown to be associated with distinct clinical phenotypes of classic dermatomyositis. There is a need to further evaluate their relevance in the formation of various clinical features. Furthermore, while providing more personalized treatment strategies, one should consider diversity of autoantibody-related subgroups of juvenile dermatomyositis.

Juvenile dermatomyositis: a case of delayed recognition with unusual complication of nephrocalcinosis

2021 ◽  
Vol 14 (4) ◽  
pp. e241152
Author(s):  
Geminiganesan Sangeetha ◽  
Divya Dhanabal ◽  
Saktipriya Mouttou Prebagarane ◽  
Mahesh Janarthanan
Keyword(s):  
Muscle Weakness ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathy ◽  
Unusual Complication ◽  
Ultrasound Screening ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Calcinosis Cutis ◽  
First Case ◽  
Medullary Nephrocalcinosis

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children and is characterised by the presence of proximal muscle weakness, heliotrope dermatitis, Gottron’s papules and occasionally auto antibodies. The disease primarily affects skin and muscles, but can also affect other organs. Renal manifestations though common in autoimmune conditions like lupus are rare in JDM. We describe a child whose presenting complaint was extensive calcinosis cutis. Subtle features of proximal muscle weakness were detected on examination. MRI of thighs and a muscle biopsy confirmed myositis. Nephrocalcinosis was found during routine ultrasound screening. We report the first case of a child presenting with rare association of dermatomyositis, calcinosis cutis and bilateral medullary nephrocalcinosis.

scholarly journals Juvenile dermatomyositis - A case report with review on oral manifestations and oral health considerations

2018 ◽  
Vol 44 (1) ◽  
pp. 52-61
Author(s):  
Pritesh Ruparelia ◽  
Oshin Verma ◽  
Vrutti Shah ◽  
Krishna Shah
Keyword(s):  
Oral Health ◽  
Muscle Weakness ◽  
Juvenile Dermatomyositis ◽  
Oral Manifestations ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Inflammatory Myositis ◽  
Life Threatening

Juvenile Dermatomyositis is the most common inflammatory myositis in children, distinguished by proximal muscle weakness, a characteristic rash and Gottron’s papules. The oral lesions most commonly manifest as diffuse stomatitis and pharyngitis with halitosis. We report a case of an 8 year old male with proximal muscle weakness of all four limbs, rash, Gottron’s papules and oral manifestations. Oral health professionals must be aware of the extraoral and intraoral findings of this rare, but potentially life threatening autoimmune disease of childhood, for early diagnosis, treatment, prevention of long-term complications and to improve the prognosis and hence, the quality of life for the patient.

A Case Report on the Surgical Management of Subhepatic Perforated Appendicitis: A Rare Presentation

2020 ◽  
Author(s):  
Mumin Hakim ◽  
Rania Mostafa ◽  
Mohammed Al Shehri ◽  
Sherif Sharawy
Keyword(s):  
Case Report ◽  
Acute Appendicitis ◽  
Female Patient ◽  
Surgical Management ◽  
Perforated Appendicitis ◽  
Exploratory Laparotomy ◽  
Atypical Presentation ◽  
Review Of Literature ◽  
Rare Presentation ◽  
Case Presentation

Abstract Background: Subhepatic appendicitis is an exceedingly rare presentation accounting for 0.01% of Acute appendicitis. It is of prime importance to be aware of various variants and thereby managing such challenging cases accordingly.Case presentation: We present a middle-aged female patient with subhepatic perforated appendicitis and peritonitis who underwent an exploratory laparotomy and appendectomy.Conclusions: Surgical management of such patients is challenging due to an atypical presentation. The surgical management of such patients is discussed with a brief review of literature.

Systemic lupus and catastrophic antiphospholipid syndrome manifesting as cardiogenic shock

Lupus ◽  
2019 ◽  
Vol 28 (11) ◽  
pp. 1350-1353 ◽  
Author(s):  
M Schultz ◽  
K Wimberly ◽  
M Guglin
Keyword(s):  
Cardiogenic Shock ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Early Recognition ◽  
Case Series ◽  
Young Female ◽  
Systemic Lupus ◽  
Rare Manifestation ◽  
Autoimmune Condition ◽  
Peripartum Period

In this case series we present two young female patients presenting in the peripartum period with cardiogenic shock. Both patients had underlying autoimmune diseases, one with systemic lupus erythematosus (SLE) and the other with antiphospholipid syndrome (APS). In both cases cardiogenic shock was a direct manifestation of their autoimmune condition, and with prompt diagnosis and management both patients were able to recover. This case series illustrates the importance of early recognition of cardiogenic shock as a rare manifestation both of SLE and APS.

Juvenile Dermatomyositis: A Clinical Overview

1990 ◽  
Vol 12 (4) ◽  
pp. 117-124
Author(s):  
Lauren M. Pachman
Keyword(s):  
Medical Care ◽  
Causal Relationship ◽  
Medical Therapy ◽  
Muscle Weakness ◽  
Genetic Factors ◽  
Juvenile Dermatomyositis ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Coxsackievirus B ◽  
Clinical Overview

The child who develops the symptoms of the specific rash, proximal muscle weakness, and fatigue should seek medical care promptly. With the advances in physical and medical therapy, many of the consequences of the disease can now be ameliorated. There are suggestive data that JDMS and PM may each have a different pathophysiology, but more evidence is needed. The next few years should be exciting as there is increased effort to determine if there is, in fact, a causal relationship between Coxsackievirus B or other enterovirus and genetic factors that alter the susceptibility to or severity of the course of the disease—JDMS.

scholarly journals Paediatric burns secondary to nail adhesives: a case series

2016 ◽  
Vol 4 ◽  
pp. 1-3 ◽  
Author(s):  
Claire Coles ◽  
Muhammad Umair Javed ◽  
Sarah Hemington Gorse ◽  
Dai Nguyen
Keyword(s):  
Early Recognition ◽  
Healthcare Providers ◽  
Case Series ◽  
Chemical Agent ◽  
Chemical Burns ◽  
Case Presentation ◽  
The Public ◽  
Cosmetic Industry ◽  
Paediatric Burns ◽  
Beauty Products

Abstract Background Nail adhesives are widely available beauty products that are used in the cosmetic industry and households to attach false nails. Nail adhesive burns are uncommon, and it is crucial that healthcare providers and the public are aware of its potential to cause chemical burn. Case Presentation Case series of accidental burns secondary to cyanoacrylate nail glue treated at the Welsh Centre for Burns and Plastic Surgery (WCBPS) in Swansea, United Kingdom. Conclusion All of the burns were observed in children and occurred due to accidental spillage. Therefore, it is important for the public to be aware that nail glue is a chemical agent which should be stored safely away from the reaches of young children. The case series highlights the importance of first aid in reducing the severity of chemical burns secondary to the nail adhesives, and its early recognition and treatment are emphasised.

scholarly journals Surgical management of subhepatic perforated appendicitis: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Mumin Hakim ◽  
Rania Mostafa ◽  
Mohammed Al Shehri ◽  
Sherif Sharawy
Keyword(s):  
Case Report ◽  
Acute Appendicitis ◽  
Surgical Management ◽  
Perforated Appendicitis ◽  
Exploratory Laparotomy ◽  
Atypical Presentation ◽  
Review Of The Literature ◽  
Middle Aged ◽  
Rare Presentation ◽  
Case Presentation

Abstract Background Subhepatic appendicitis is an exceedingly rare presentation, accounting for 0.01% of acute appendicitis cases. It is of prime importance to be aware of variants and manage such challenging cases accordingly. Case presentation We present a case of a middle-aged Saudi woman with subhepatic perforated appendicitis and peritonitis who underwent an exploratory laparotomy and appendectomy. Conclusions The initial diagnosis and surgical management of such patients is challenging due to an atypical presentation. The surgical management of such patients is discussed with a brief review of the literature.

scholarly journals AB0733 DADA2 VASCULITIS PRESENTING AS POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES): AN ATYPICAL PRESENTATION

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1396.3-1397
Author(s):  
M. Mamadapur ◽  
S. Mahadevan ◽  
A. Singh ◽  
R. Chakravarthy C H ◽  
R. S ◽  
...  
Keyword(s):  
Polyarteritis Nodosa ◽  
Early Recognition ◽  
Ulnar Artery ◽  
Monogenic Disease ◽  
Normal Male ◽  
Atypical Presentation ◽  
Rare Presentation ◽  
Diffusion Restriction ◽  
Mri Brain ◽  
Left Insula

Background:Deficiency of Adenosine Aminase deficiency 2 is monogenic disease presenting with multisystem involvement of vasculitis,Stroke1,hematological manifestations. We hereby present a young child who presented with PRES initially and later diagnosed as DADA2.Only one case of DADA2 presenting as PRES is reported so far2.This case highlights the atypical presentation of DADA2.Objectives:A case report to highlight the rare presentation of DADA2 vasculits.Methods:8 year old developmentally normal male child,born out of consanguineous marriage presented with with fever,abdominal pain, seizures 1 year back.Ultrasound of abdomen revelaed mesenteric lymphadenitis and MRI Brain was suggestive of Meningoencephalitis.He was managed with antivirals and antibiotics then.6 months later,he had fever,skin rash,pain abdomen,status epilepticus,hypertension followed by gangrene of fingers and toes.APLA,ANA,ANCA workup was negative. Complete Hemogram was normal. CRP was 130mg/dl. CT Abdomen was normal and no evidence of aneurysms. Renal Doppler Ultrasound was normal.CT upper limb showed left radial and ulnar artery significant narrowing with patchy occlusion. MRI Brain showed bilateral fronto parietal,occipital,putamen,left insula and subcortical and cortical T2W FLAIR hyperintensity without diffusion restriction suggestive of PRES. He was diagnosed as Childhood Polyarteritis Nodosa and treated with cyclophosphamide. Genetic Analysis of ADA2(CECR1) gene mutation by Sanger Sequencing of exons 2 to 10 showed a known variant (rs202134424) in ADA2 gene.Figure 1.Gangrene of left 1-5th digits and right 4th and 5th digit resorptionFigure 2.T2W FLAIR image showing bilateral fronto parietal,occipital,putamen,left insula and subcortical and cortical hyperintensity without diffusion restriction suggestive of PRES.Results:He was started on Infliximab and is on follow up. No further events noted so far.Rehabilitation of left upper hand was done.Conclusion:DADA2 can present with varied CNS manifestations like infarct,hemorrhage,aneurysms and PRES.High index of suspicion and early recognition can help in maintaining vascular integrity.References:[1]Ganhão S, Loureiro G, Oliveira D, dos-Reis-Maia R, Aguiar F, Quental R et al. Two cases of ADA2 deficiency presenting as childhood polyarteritis nodosa: novel ADA2 variant, atypical CNS manifestations, and literature review. Clinical Rheumatology. 2020;39(12):3853-3860.[2]Sharma A, Naidu G, Sharma V, Jha S, Dhooria A, Dhir V et al. Deficiency of Adenosine Deaminase 2 in Adults and Children: Experience From India. Arthritis & Rheumatology. 2020;73(2):276-285.Disclosure of Interests:None declared

scholarly journals Juvenile Dermatomyositis and the Inflammatory Myopathies

2020 ◽  
Vol 40 (03) ◽  
pp. 342-348
Author(s):  
Collin Swafford ◽  
E. Steve Roach
Keyword(s):  
Muscle Weakness ◽  
Muscle Injury ◽  
Juvenile Dermatomyositis ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Rimmed Vacuoles ◽  
Immune Mediated ◽  
Primary Disorder ◽  
Tissue Changes

AbstractThe inflammatory myopathies comprise disorders of immune-mediated muscle injury. The histopathology and clinical features help distinguish them. Juvenile dermatomyositis (JDM) is the most common form of myositis in children and adolescents. Children with JDM present with proximal muscle weakness and characteristic rashes. The presentation is similar in children and adults, but JDM is a primary disorder and the adult form often is concerning for a paraneoplastic syndrome. Proximal muscle weakness occurs with dermatomyositis, polymyositis, and immune-mediated necrotizing myopathy, but the latter two conditions have no dermatologic findings or distinct tissue changes which set them apart from dermatomyositis. Inclusion body myositis, also included in the inflammatory myopathies, presents with more distal involvement, and microscopically exhibits identifiable rimmed vacuoles. We review key features of these disorders, focusing in more detail on JDM because it is more often encountered by the child neurologist.

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