scholarly journals Anti-MDA5 Antibody-Positive Interstitial Pneumonia with Autoimmune Features Presenting as Amyopathic Hypodermatitic Dermatomyositis: A Case Report

2021 ◽  
pp. 222-229
Author(s):  
Maria L. Mihailescu ◽  
Cuoghi Edens ◽  
Mark D. Hoffman
Keyword(s):  
Case Report ◽  
Interstitial Lung Disease ◽  
Specific Antibody ◽  
Early Recognition ◽  
Clinical Findings ◽  
Muscle Disease ◽  
Skin Involvement ◽  
Unique Form ◽  
Multidisciplinary Assessment ◽  
Positive Individual

Dermatomyositis (DM) and its variant, clinically amyopathic DM, are widely recognized entities. DM sine dermatitis, a variant without skin involvement, is less widely reported. DM with neither muscle nor skin manifestations has not been reported. We herein describe the first account of a patient with a myositis-specific antibody presenting with an array of clinical findings in the absence of both muscle and pathognomonic skin disease. This case report details the multidisciplinary assessment of an anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive individual with inflammatory polyarthropathy, mucocutaneous capillary changes, and evidence of interstitial lung disease but lacking overt skin and muscle disease. This presentation is paradoxically but appositely deemed to represent a unique form of DM, which may be best described as “amyopathic hypodermatitic dermatomyositis.” Early recognition and documentation of these cases will help to characterize this variant in the future, determine its frequency, and guide management.

scholarly journals MANIFESTASI DAN PENATALAKSANAAN PEMFIGUS VULGARIS DI RONGGA MULUT

2018 ◽  
Vol 2 (2) ◽  
pp. 117-124
Author(s):  
Indra Gunawan ◽  
Riani Setiadhi
Keyword(s):  
Case Report ◽  
Early Recognition ◽  
Pemphigus Vulgaris ◽  
Oral Lesions ◽  
Oral Manifestations ◽  
Skin Involvement ◽  
Mucous Membranes ◽  
Blistering Disease ◽  
Mouth Wash ◽  
Desmoglein 3

Background : Pemphigus Vulgaris (PV) is an autoimmune intraepithelial blistering disease involving the skin and mucous membranes. Oral lesions could be the first sign of the disease followed by the involvement of skin and other mucosa sites.Objectives : This is oral manifestations of pemphigus vulgaris case report, intended to help clinicians to recognize and treat the oral lesions.Case Reported : Male 27 yo admitted to RSUP Dr.Hasan Sadikin hospital with multiple blisters on the skin and oral mucosa, was diagnosed with Pemphigus Vulgaris.Case Management : Patient was treated with steroid mouth wash and oral paste. After two months of treatment, the patient was fully recovered from oral lesion.Discussion : In PV, autoantibodies are produced against desmosomes spesifically desmoglein 3 which responsible for holding the cells of the epithelium together. The loss of adhesive function due to anti Dsg 3 antibodies result in bulla formation on the oral mucosa.The aetiology for PV is still uncertain. Conclusion :Early recognition and treatment of oral lesions is important as it may prevent skin involvement. Early treatment, patient’s compliance and multi disciplinal teamwork ensure the treatment succes for this disease

scholarly journals Rapidly progressive interstitial lung disease due to anti-MDA5 antibodies without skin involvement: a case report and literature review

2018 ◽  
Vol 38 (7) ◽  
pp. 1293-1296 ◽  
Author(s):  
Juan González-Moreno ◽  
Manuel Raya-Cruz ◽  
Ines Losada-Lopez ◽  
Ana Paula Cacheda ◽  
Cristina Oliver ◽  
...  
Keyword(s):  
Case Report ◽  
Interstitial Lung Disease ◽  
Literature Review ◽  
Lung Disease ◽  
Skin Involvement

scholarly journals Allopurinol causing generalized exfoliative dermatitis: a case report

2017 ◽  
Vol 6 (7) ◽  
pp. 1816
Author(s):  
Mila Nu Nu Htay ◽  
Wai Wai Myint ◽  
Htay Lwin ◽  
Win Htay
Keyword(s):  
Case Report ◽  
Drug Allergy ◽  
Early Recognition ◽  
Supportive Treatment ◽  
Upper Limbs ◽  
Skin Involvement ◽  
Exfoliative Dermatitis ◽  
Culprit Drug ◽  
Mortality Case ◽  
The Face

Erythroderma is a scaly, erythematous dermatitis of the skin, which occurs in drug allergy, malignancy and underlying skin disorders. The diagnosis is challenging because the extent of skin involvement does not always correlate with the extent of internal organ involvement. Therefore, early recognition of symptoms is vital to minimize morbidity and mortality. Case report: A 52 years old man had asymptomatic hyperuricemia and prescribed allopurinol 300mg, daily. One month later, the rashes started to appear on his trunk and then progressed to the face and upper limbs. Then it continued to spread to the lower extremities. Management involves prompt cessation of the culprit drug, administration of corticosteroids and supportive treatment. It is Concluded that Allopurinol is commonly used in clinical practice for the treatment of symptomatic hyperuricemia and gout. It has been associated with erythroderma especially when used indiscriminately.

Acceleration of interstitial lung disease induced by raltitrexed

2019 ◽  
Vol 26 (5) ◽  
pp. 1241-1243 ◽  
Author(s):  
Sarah Howlett ◽  
Raminder Aul ◽  
Mark Hill ◽  
David J Pinato
Keyword(s):  
Case Report ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Thymidylate Synthase ◽  
Colorectal Adenocarcinoma ◽  
Preoperative Staging ◽  
Rapid Progression ◽  
Lung Function Tests ◽  
Clinical Pattern ◽  
Significant Acceleration

Introduction Raltitrexed is a folate analogue, which selectively inhibits thymidylate synthase, used in the treatment of colorectal carcinoma. Common side effects include asthenia and gastrointestinal and haematological toxicities. Case report We present the case of a 74-year-old gentleman with incidental mild interstitial lung disease on preoperative staging CT Chest who developed acute breathlessness whilst undergoing adjuvant raltitrexed treatment for a completely excised colorectal adenocarcinoma. Management and outcome Discontinuation of raltitrexed and a course of steroid therapy resulted in resolution of symptoms, mirrored by an improvement in lung function tests. Discussion The clinical pattern of rapid progression with steroid response highlights the potential for significant acceleration of interstitial lung disease by raltitrexed.

Corneal cross-linking for treatment of progressive keratoconus in a patient with Alport syndrome: A case report

2021 ◽  
pp. 112067212199767
Author(s):  
Iva Krolo ◽  
Aida Kasumović ◽  
Ivana Radman ◽  
Pavao Pavić
Keyword(s):  
Case Report ◽  
Alport Syndrome ◽  
Contact Lenses ◽  
Corneal Dystrophy ◽  
Clinical Findings ◽  
Cross Linking ◽  
Posterior Polymorphous Corneal Dystrophy ◽  
One Year ◽  
Corneal Tomography

Purpose: Ocular features of Alport syndrome include anterior lenticonus, posterior polymorphous corneal dystrophy, and fleck-and-dot retinopathy in most cases. Keratoconus in such patients has been rarely mentioned in previous studies. To our knowledge, this is the first report of corneal cross-linking for halting the progression of keratoconus in a patient with Alport syndrome. Case report: A 22-year-old male was referred for his initial corneal topography, after he was already prescribed with rigid gas-permeable contact lenses. Alport syndrome was diagnosed in his infancy and gene COL4A5 mutation was confirmed. Ophthalmological evaluation confirmed keratoconus. One-year follow-up showed a progression on his right eye and standard corneal cross-linking was performed. Stabilization of the disease marked by normalization in visual function and corneal tomography values was noticed 1 year after the procedure. Conclusions: When diagnosing ocular clinical findings of Alport syndrome, keratoconus should be considered. Standard corneal cross-linking protocol can halt its progression.

scholarly journals A Case Report on Frontal Osteoma

Pulse ◽  
2017 ◽  
Vol 9 (1) ◽  
pp. 45-48
Author(s):  
MR Molla ◽  
F Ferdousi ◽  
DR Shankar ◽  
AKMB Karim
Keyword(s):  
Case Report ◽  
Frontal Lobe ◽  
Frontal Sinus ◽  
Clinical Findings ◽  
Complete Excision ◽  
Tumor Mass ◽  
The Right ◽  
Orbital Region

A 13 years old boy admitted with the complaint of progressive exophthalmos and gradually decreasing vision on right eye, also occasional headache and deformity on the right fronto-orbital region. Radiological & clinical findings revealed a case of frontal osteoma in the right frontal sinus extending up to right frontal lobe, eroding right roof of the orbit. Complete excision of the tumor mass was possible surgically. Biopsy confirmed a case of osteoma. Below is a discussion on diagnosis & management of frontal osteomaPulse Vol.9 January-December 2016 p.45-48

scholarly journals An incidental case of biliary fascioliasis with subtle clinical findings: US and MRCP findings

2013 ◽  
Vol 47 (2) ◽  
pp. 125-127
Author(s):  
Hakan Önder ◽  
Faysal Ekici ◽  
Emin Adin ◽  
Suzan Kuday ◽  
Hatice Gümüş ◽  
...  
Keyword(s):  
Case Report ◽  
Bile Duct ◽  
Magnetic Resonance ◽  
Biliary Obstruction ◽  
Fasciola Hepatica ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Clinical Findings ◽  
Imaging Studies ◽  
Laboratory Findings ◽  
Acoustic Shadow

Background. Fascioliasis is a disease caused by the trematode Fasciola hepatica. Cholangitis is a common clinical manifestation. Although fascioliasis may show various radiological and clinical features, cases without biliary dilatation are rare. Case report. We present unique ultrasound (US) and magnetic resonance cholangiopancreatography (MRCP) findings of a biliary fascioliasis case which doesn’t have biliary obstruction or cholestasis. Radiologically, curvilinear parasites compatible with juvenile and mature Fasciola hepatica within the gallbladder and common bile duct were found. The parasites appear as bright echogenic structures with no acoustic shadow on US and hypo-intense curvilinear lesions on T2 weighted MRCP images. Conclusions. Imaging studies may significantly contribute to the diagnosis of patients with subtle clinical and laboratory findings, particularly in endemic regions.

scholarly journals A conundrum of West syndrome, behavioural problems and parental expressed emotions: a case report

2020 ◽  
Vol 33 (5) ◽  
pp. e100111
Author(s):  
Yogender Kumar Malik ◽  
Nidhi Chauhan ◽  
Akhilesh Sharma ◽  
Susanta Padhy
Keyword(s):  
Case Report ◽  
Early Recognition ◽  
West Syndrome ◽  
Behavioural Problems ◽  
Optimal Outcome ◽  
Optimal Care ◽  
Epileptic Spasms ◽  
Expressed Emotions ◽  
Neurodevelopmental Problems ◽  
The Ideal

West syndrome (WS) is the most common epileptic syndrome in infancy characterised by epileptic spasms, hypsarrhythmia and neurodevelopmental problems. Epileptic spasms remain in many ways a conundrum, and the ideal intervention, as well as how to screen patients to provide optimal care and certainly its genetic cause, remains puzzling. It is important to screen infants for early recognition and intervention to achieve the optimal outcome. We hereby discuss the approach to management of a boy aged 4½ years old with WS and behavioural problems and of parental expressed emotions.

scholarly journals The myositis clinical phenotype associated with anti-Zo autoantibodies: a case series of nine UK patients

Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1626-1631 ◽  
Author(s):  
Sarah L Tansley ◽  
Zoe Betteridge ◽  
Hui Lu ◽  
Emma Davies ◽  
Simon Rothwell ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Clinical Phenotype ◽  
Disease Onset ◽  
Usual Interstitial Pneumonia ◽  
Trna Synthetase ◽  
Muscle Disease ◽  
Ancestral Haplotype ◽  
Common Finding

Abstract Objectives It has been over 10 years since the first report of autoantibodies directed against phenylalanyl tRNA synthetase (anti-Zo) in a patient with features of the anti-synthetase syndrome. In that time no further cases have been published. Here we aim to characterize more fully the clinical phenotype of anti-Zo–associated myositis by describing the clinical features of nine patients. Methods Anti-Zo was identified by protein-immunoprecipitation in patients referred for extended spectrum myositis autoantibody testing at our laboratory. Results were confirmed by immunodepletion using a reference serum. Medical records were retrospectively reviewed to provide detailed information of the associated clinical phenotype for all identified patients. Where possible, HLA genotype was imputed using Illumina protocols. Results Nine patients with anti-Zo were identified. The median age at disease onset was 51 years, and six patients were female. Seven patients had evidence of inflammatory muscle disease, seven of interstitial lung disease and six of arthritis. The reported pattern of interstitial lung disease varied with usual interstitial pneumonia, non-specific interstitial pneumonia and organizing pneumonia all described. Other features of the anti-synthetase syndrome such as RP and mechanics hands were common. HLA data was available for three patients, all of whom had at least one copy of the HLA 8.1 ancestral haplotype. Conclusion Patients with anti-Zo presenting with features of the anti-synthetase syndrome and interstitial lung disease is a common finding. Like other myositis autoantibodies, there is likely to be a genetic association with the HLA 8.1 ancestral haplotype.

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