scholarly journals Unilateral Heliotrope Rash in Juvenile Dermatomyositis: An Unusual Presentation of an Underlying Serious Disease

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Ghada Al-Janobi ◽  
Hisham Alkhalidi ◽  
Mohammed A. Omair
Keyword(s):  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Juvenile Dermatomyositis ◽  
Unusual Presentation ◽  
Inflammatory Myositis ◽  
Case Presentation ◽  
Excellent Response ◽  
Myositis Patient ◽  
Underlying Malignancy ◽  
Serious Disease

Background. Heliotrope rash is one of the characteristic skin manifestations of juvenile dermatomyositis. It is a reddish-purple rash on the upper eyelids that is usually bilateral.Case Presentation. We report a boy who presented with unilateral heliotrope rash, Gottron’s papules, and muscle weakness. Muscle biopsy was consistent with inflammatory myositis. Patient was started on prednisolone and methotrexate with an excellent response in both the skin and muscles.Conclusion. Unilateral heliotrope rash can occur in patients with juvenile dermatomyositis. Being a paraneoplastic condition caution should be taken not to miss any underlying malignancy.

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.

Clinical features, muscle biopsy scores, myositis specific antibody profiles and outcome in juvenile dermatomyositis

2021 ◽  
Vol 51 (1) ◽  
pp. 95-100
Author(s):  
Erdal Sag ◽  
Selcan Demir ◽  
Yelda Bilginer ◽  
Beril Talim ◽  
Goknur Haliloglu ◽  
...  
Keyword(s):  
Clinical Features ◽  
Muscle Biopsy ◽  
Specific Antibody ◽  
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 Unusual Case of Localized Upper Limbs and Truncal Edema Associated with Fever, Anemia, and Acute Kidney Injury

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Mohammad Tinawi
Keyword(s):  
Acute Kidney Injury ◽  
Creatine Kinase ◽  
Muscle Biopsy ◽  
Unusual Case ◽  
Kidney Injury ◽  
The Other ◽  
Upper Limbs ◽  
Inflammatory Myositis ◽  
Arm Pain ◽  
Extensive Evaluation

The patient is a 75-year-old man who presented with right arm pain, edema, and erythema. The same manifestations appeared in the other arm 3 weeks later. He also developed fever, acute kidney injury, anemia, and truncal edema. Initial extensive evaluation was unrevealing. He was noted to have elevated creatine kinase, and a diagnostic muscle biopsy lead to diagnosis of inflammatory myositis. He improved with corticosteroids.

scholarly journals A rare case presentation of an anomalous uterus mimicking ovarian tumour

2017 ◽  
Vol 6 (6) ◽  
pp. 2638
Author(s):  
Mohanambal M. ◽  
Wills G. Sheelaa
Keyword(s):  
Rare Case ◽  
Pregnancy Loss ◽  
Recurrent Pregnancy Loss ◽  
Ovarian Tumour ◽  
Left Ovary ◽  
Unusual Presentation ◽  
Case Presentation ◽  
Young Girls ◽  
Uterus Didelphys ◽  
Operative Findings

Mullerian anomalies occur in 1:1000-3000 females. Uterus didelphys and obstructed hemangioma with a septum contribute to 10% anomalies. Young girls present with severe dysmenorrhea, hematometra, hematocolpos and recurrent pregnancy loss. A 16-year-old teenager presented like a torsion of complex ovarian tumour is presented here. Intra operative findings was uterus didelphys with well-developed 2 horns, tubes and ovaries. On left ovary, a hemorrhagic corpus luteal cyst of size 5.2*4cm was seen with 50ml of hemoperitoneum. Diagnosis was confirmed histopathologically. This case is reported for the unusual presentation of an anomalous uterus mimicking torsion ovarian tumour.

scholarly journals Physical therapy improves motion in a patient with inclusion body myositis - a case report

2020 ◽  
Vol 77 (11) ◽  
pp. 1216-1220
Author(s):  
Jelena Stevanovic ◽  
Maja Vulovic ◽  
Danijela Pavicevic ◽  
Mihailo Bezmarevic ◽  
Andjelka Stojkovic ◽  
...  
Keyword(s):  
Case Report ◽  
Physical Therapy ◽  
Inclusion Body ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Inclusion Body Myositis ◽  
Clinical Symptoms ◽  
Inflammatory Myopathy ◽  
Functional Abilities ◽  
Progressive Muscle Weakness

Introduction. Inclusion body myositis (IBM) is a rare form of inflammatory myopathy with a slowly progressive course. It is manifested by early weakness and atrophy of skeletal muscles, especially forearm muscles and the quadriceps. At the very beginning of the disease, clinical symptoms are not pronounced, therefore it is difficult to diagnose. Case report. A forty-eight-year-old female patient visited her doctor due to the weakness of muscles in arms and legs. Five years prior to this, she was treated by a neurologist and a physiatrician on several occasions with different diagnoses for progressive muscle weakness. During the last hospitalization, IBM was diagnosed after the muscle biopsy findings. After the diagnosis, the patient underwent intensive physical therapy in order to preserve the ability to independently perform everyday activities and stability of walk. Conclusion. IBM is a rare clinical entity which often takes several years to be diagnosed. Progressive muscle weakness in elderly should point to possible IBM diagnosis, which is only confirmed by muscle biopsy. Physical therapy has a significant role in the treatment as it leads to improvement of functional abilities of the patients in their daily activities, thus reducing the disability degree.

scholarly journals Validation of a score tool for measurement of histological severity in juvenile dermatomyositis and association with clinical severity of disease

2013 ◽  
Vol 74 (1) ◽  
pp. 204-210 ◽  
Author(s):  
Hemlata Varsani ◽  
Susan C Charman ◽  
Charles K Li ◽  
Suely K N Marie ◽  
Anthony A Amato ◽  
...  
Keyword(s):  
Disease Activity ◽  
Muscle Biopsy ◽  
Juvenile Dermatomyositis ◽  
Intraclass Correlation ◽  
Latin Square ◽  
Quadriceps Muscle ◽  
Clinical Severity ◽  
Intraobserver Agreement ◽  
Biopsy Tissue ◽  
Clinical Measures

ObjectivesTo study muscle biopsy tissue from patients with juvenile dermatomyositis (JDM) in order to test the reliability of a score tool designed to quantify the severity of histological abnormalities when applied to biceps humeri in addition to quadriceps femoris. Additionally, to evaluate whether elements of the tool correlate with clinical measures of disease severity.Methods55 patients with JDM with muscle biopsy tissue and clinical data available were included. Biopsy samples (33 quadriceps, 22 biceps) were prepared and stained using standardised protocols. A Latin square design was used by the International Juvenile Dermatomyositis Biopsy Consensus Group to score cases using our previously published score tool. Reliability was assessed by intraclass correlation coefficient (ICC) and scorer agreement (α) by assessing variation in scorers’ ratings. Scores from the most reliable tool items correlated with clinical measures of disease activity at the time of biopsy.ResultsInter- and intraobserver agreement was good or high for many tool items, including overall assessment of severity using a Visual Analogue Scale. The tool functioned equally well on biceps and quadriceps samples. A modified tool using the most reliable score items showed good correlation with measures of disease activity.ConclusionsThe JDM biopsy score tool has high inter- and intraobserver agreement and can be used on both biceps and quadriceps muscle tissue. Importantly, the modified tool correlates well with clinical measures of disease activity. We propose that standardised assessment of muscle biopsy tissue should be considered in diagnostic investigation and clinical trials in JDM.

Bilateral diffuse uveal melanocytic proliferation: A case report and literature review

2020 ◽  
Author(s):  
Nianting Tong ◽  
Liangyu Wang ◽  
Nan Wang ◽  
Zhanyu Zhou
Keyword(s):  
Retinal Detachment ◽  
Exudative Retinal Detachment ◽  
Histopathologic Examination ◽  
Cataract Formation ◽  
Case Presentation ◽  
Melanocytic Tumors ◽  
Ophthalmic Manifestations ◽  
Underlying Malignancy ◽  
History Of ◽  
Shallow Anterior Chamber

Abstract Background Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic intraocular disease that causes progressive visual loss in patients driven by an IgG factor associated with an underlying malignancy. Characteristic ocular findings include exudative retinal detachment, rapid cataract formation and uveal melanocytic tumors. Case presentation Here, we presented a patient, whose clinical manifestation was diffusely thickened choroid, shallow anterior chamber, cataract formation and exudative retinal detachment. Histopathologic examination for the biopsies from the choroid during the surgery showed the the tissue might be originated from melanocytes and with the benign biologic behavior. Therefore, the diagnosis for this patient was BDUMP, although there was no obvious history of malignancy until we prepared for this article. Conclusions This was a rare BDUMP clarified by ophthalmic manifestations and histopathologic examination, without clear history of systematic malignancy.

scholarly journals Hypothyroidism Presenting as Hoffman’s Syndrome – A Case Report

2015 ◽  
Vol 16 (2) ◽  
pp. 112-114
Author(s):  
NS Neki ◽  
Ishu Singh ◽  
Jasbir Kumar ◽  
Ankur Jain ◽  
Tamil Mani
Keyword(s):  
Case Report ◽  
Thyroid Function ◽  
Muscle Weakness ◽  
Muscle Biopsy ◽  
Muscle Hypertrophy ◽  
Thyroid Function Tests ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzyme ◽  
Marked Elevation

Hoffman syndrome is characterized by pseudohypertrophy of muscles, muscle’s weakness & stiffness complicating hypothyroidism. We describe the disorder in a 45 years old female admitted with complaints of myalgia, proximal muscle weakness & calf muscle hypertrophy since 11 months. Thyroid function tests, marked elevation of muscle enzyme, electromyogram & muscle biopsy established the diagnosis of thyroid myopathy with Hoffman’s syndrome. Therapy with levothyroxine resulted in marked clinical & biochemical improvements.J MEDICINE July 2015; 16 (2) : 112-114

scholarly journals Late-onset multiple acyl-CoA dehydrogenase deficiency mimicking myositis in an elderly patient: a case report

BMC Neurology ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Yiming Zheng ◽  
Yawen Zhao ◽  
Wei Zhang ◽  
Zhaoxia Wang ◽  
Yun Yuan
Keyword(s):  
Elderly Patient ◽  
Muscle Weakness ◽  
Dehydrogenase Deficiency ◽  
Late Onset ◽  
Lipid Storage ◽  
Exercise Intolerance ◽  
Lipid Storage Myopathy ◽  
Case Presentation ◽  
Acylcarnitine Profile ◽  
Progressive Weakness

Abstract Background Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare and treatable inherited lipid storage myopathy. Here, we report an elderly patient with MADD mimicking myositis. Case presentation An 80-year-old woman had progressive weakness in her limbs, exercise intolerance, and no muscle pain for 3 months. The patient’s serum creatine kinase level was slightly elevated. The initial diagnosis was myositis. However, muscle biopsy showed many cytoplasmic vacuoles stained with oil red O, indicating the presence of lipid storage myopathy. The plasma acylcarnitine profile showed increased medium-chain and long-chain acylcarnitine species, consistent with the diagnosis of MADD. Riboflavin treatment dramatically improved muscle weakness. Conclusions MADD should be considered when evaluating elderly patients with subacute muscle weakness.

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