scholarly journals IgG/IgA-pemphigus — extremely rare blistering autoimmune diseases

2020 ◽  
Vol 96 (1) ◽  
pp. 45-51
Author(s):  
Arfenya E. Karamova ◽  
Lyudmila F. Znamenskaya ◽  
Vadim V. Chikin ◽  
Viktoria A. Mihina ◽  
Maria A. Nefedova
Keyword(s):  
Skin Biopsy ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Biopsy Sample ◽  
Cytological Examination ◽  
Iga Pemphigus ◽  
Laboratory Examinations ◽  
Skin Biopsy Sample ◽  
Unaffected Skin ◽  
Effective Medication

Purpose. To present a clinical case of IgG/IgA pemphigus in a 32-year-old woman. Material and methods. Clinical and laboratory examinations were performed to diagnose the disease: cytological examination of an impression smear from the bottom of the erosion, histological examination of a skin biopsy sample from the lesion, immunofluorescent examination of a biopsy sample of visually accessible unaffected skin. Results. Clinical manifestations of the disease were similar to Duhring's dermatitis herpetiformis, morphological ones to pemphigus vulgaris, but immunofluorescent examination of the skin biopsy sample in equal measure revealed deposits of both IgG and IgA in the epidermis, which allowed to determine a diagnosis of IgG/IgA pemphigus. Dapsone therapy at a dose of 100 mg per day led to a significant improvement in the patient's condition. Conclusion. Diagnosis of bullous dermatoses requires immunofluorescent examination of a biopsy sample of visually accessible unaffected skin. Dapsone was an effective medication for the patient with IgG/IgA pemphigus.

Analysis of clinical and diagnostic features and criteria for the effectiveness of treatment of autoimmune bullous dermatoses pemphigus group

2021 ◽  
pp. 51-57
Author(s):  
М.Е. Zapolsky ◽  
М.М. Lebediuk ◽  
N.B. Prokofyeva ◽  
V.V. Goncharenko ◽  
S.V. Nerubashchenko
Keyword(s):  
Immunofluorescence Microscopy ◽  
Clinical Manifestations ◽  
Diagnostic Algorithm ◽  
Pemphigus Vulgaris ◽  
Diagnostic Features ◽  
Iga Pemphigus ◽  
Modern Classification ◽  
Pemphigus Erythematosus ◽  
Pemphigus Vegetans

The modern classification of autoimmune bullous dermatoses is represented by three groups of diseases that have not only clinical, but also histomorphological, histochemical and immunogenetic similarities. Autoimmune bullous dermatoses are a group of heterogeneous diseases accompanied by the formation of blisters and erosions on the skin and/or mucous membranes under the influence of specific autoimmune complexes. The main clinical manifestations and diagnostic markers of autoimmune bullous dermatoses (ABD) belonging to the pemphigus group are considered.Objective — to analyze the prevalence of clinical forms of pemphigus in the southern region of Ukraine for the period from 2010 to 2020.Materials and methods. The observation group consisted of 88 patients aged 21 to 75 years. The features of clinical manifestations of typical and atypical forms of pemphigus have been studied, a diagnostic algorithm and criteria for the effectiveness of treatment in the early stages of the disease have been developed.Results and discussion. Pemphigus vulgaris was found in 61 (69.3 %), pemphigus vegetans — in 7 (7.9 %), pemphigus erythematosus — in 6 (6.8 %), herpetiform pemphigus — in 4 (4.5 %), IgA pemphigus — in 3 (3.4 %), paraneoplastic pemphigus — in 3 (3.4 %) patients.Conclusions. Modern clinical diagnostic features of ABD (pemphigus group) require the inclusion of immunofluorescence microscopy, direct and indirect enzyme immunoassay in laboratory screening. Not only positive clinical dynamics, but also the rate of disappearance of autoantibodies that induce the diseases (desmoglein, desmoplakin, periplakin, etc.) should be considered the criteria of the effectiveness of treatment of patients with ABD. At the same time, knowledge of the clinical features of ABD and the diseases that mimic them remains an important aspect.

scholarly journals Clinical Characteristics and CT Manifestations of 143 Patients With 2019 Novel Coronavirus Disease (COVID-19) in Taizhou City, Zhejiang, China

2020 ◽  
Author(s):  
Yani Kuang ◽  
Susu He ◽  
Shuangxiang Lin ◽  
Rui Zhu ◽  
Rongzhen Zhou ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Protein A ◽  
Clinical Manifestations ◽  
Imaging Features ◽  
First Case ◽  
Severe Group ◽  
Laboratory Examinations ◽  
The Difference ◽  
Severity Of The Disease ◽  
Novel Coronavirus

Abstract Background: In December 2019, the first case of pneumonia associated with the SARS-CoV-2 was found in Wuhan and rapidly spread throughout China, so data are needed on the affected patients. The purpose of our study was to find the clinical manifestations and CT features of COVID-19.Methods: All patients with COVID-19 in Taizhou city were retrospectively included and divided into non-severe group and severe group according to the severity of the disease. The clinical manifestations, laboratory examinations and imaging features of COVID-19 patients were analyzed, and the differences between the two groups were compared.Results: A total of 143 laboratory-confirmed cases were included in the study, including 110 non-severe patients and 33 severe patients. The median age of patients was 47 (range 4–86 years). Fever (73.4%) and cough (63.6%) were the most common initial clinical symptoms. Between two groups of cases, the results of aspartate transaminase, creatine kinase and lactate dehydrogenase, serum albumin, CR, glomerular filtration rate, amyloid protein A, fibrinogen, calcitonin level and oxygen partial pressure, IL – 10, absolute value of CD3, CD4, CD8 were different, and the difference was statistically significant (P < 0.05). Therefore, these quantitative indicators can be used to help assess the severity. On admission, the CT showed that the lesions were mostly distributed in the periphery of the lung or subpleural (135 cases (98%)), and most of lesions presented as patchy (81%), mixed density (63%) shadow. Consolidation (68% vs 41%), bronchial inflation signs (59% vs 41%), and bronchiectasis (71% vs 39%) were more common in the severe group.Conclusions: Most of the cases of COVID-19 in Taizhou have mild symptoms and no death. In addition to clinical symptoms, some laboratory tests (such as absolute values of CD4 and CD8) and CT findings can be used to assess the severity of the disease.

scholarly journals The Significance of Scalp Involvement in Pemphigus: A Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-8 ◽  
Author(s):  
Marta Sar-Pomian ◽  
Lidia Rudnicka ◽  
Malgorzata Olszewska
Keyword(s):  
Web Of Science ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Hair Follicles ◽  
Response To Treatment ◽  
Direct Immunofluorescence ◽  
Pemphigus Foliaceus ◽  
Medical Databases ◽  
Scalp Lesions ◽  
Reliable Diagnostic Method

Scalp is a unique location for pemphigus because of the abundance of desmogleins localized in hair follicles. Scalp involvement is observed in up to 60% of patients in the course of pemphigus. The lesions may occasionally lead to alopecia. Unforced removal of anagen hairs in a pull test is a sign of high disease activity. Direct immunofluorescence of plucked hair bulbs is considered a reliable diagnostic method in patients with pemphigus. Follicular acantholysis is a characteristic histopathological feature of pemphigus lesions localized on the scalp. Trichoscopy may serve as a supplementary method in the diagnosis of pemphigus. This review summarizes the most recent data concerning scalp involvement in pemphigus vulgaris and pemphigus foliaceus. A systematic literature search was conducted in three medical databases: PubMed, Embase, and Web of Science. The analysis included literature data about desmoglein distribution in hair follicles, as well as information about clinical manifestations, histopathology, immunopathology, and trichoscopy of scalp lesions in pemphigus and their response to treatment.

Gitelman Syndrome in a Pregnant Woman

2021 ◽  
Author(s):  
Feifei Huo ◽  
Nan Li ◽  
Dong Zhang
Keyword(s):  
Treatment Guidelines ◽  
Case Reports ◽  
Serum Magnesium ◽  
Clinical Manifestations ◽  
Fetal Outcome ◽  
Gitelman Syndrome ◽  
Renal Tubules ◽  
Loss Of Function ◽  
Sodium Chloride Cotransporter ◽  
Laboratory Examinations

Abstract Background: Gitelman syndrome (GS) is an autosomal recessive inherited salt-losing tubulopathy resulted from a loss-of-function mutation in the gene SLC12A3 encoding the thiazide-sensitive sodium-chloride cotransporter (NCCT) protein located in the distal renal tubules. Investigations revealed hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria and increased activity of renin-angiotensin-aldosterone system. There have been very few case reports on Gitelman syndrome in pregnancy, and some cases showed adverse consequences of the fetus.Case presentation: We presented a case report of a pregnant female with hypokalemia, a large amount of intravenous potassium was required to maintain a relatively normal level of serum potassium.Therefore, further laboratory examinations and whole blood DNA sequencing were carried out. The patient was eventually diagnosed with Gitelman syndrome. In terms of treatment, the amount of potassium supplementation was gradually reduced, and magnesium supplementation was intermittently provided at the same time to maintain the patient's serum potassium at about 3.0mmol/L and serum magnesium at about 0.8mmol/L. Obstetric ultrasound during hospitalization indicated normal fetal development, and the patient was discharged from hospital after her condition improved.Conclusions: The clinical manifestations of GS are non-specific, and there is a lack of evidence-based treatment guidelines for pregnant GS patients, so multidisciplinary management of pregnant GS women is essential. Treatment should be cautious and individual, and the electrolytes should be closely monitored to avoid complications caused by electrolyte disturbance and strive to obtain a good maternal and fetal outcome.

scholarly journals Oral pemphigus vulgaris

2017 ◽  
Vol 4 (3) ◽  
pp. 100-103
Author(s):  
Mohit Sharma
Keyword(s):  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Oral Literature ◽  
Therapeutic Approaches ◽  
The Past ◽  
Mucosal Surfaces ◽  
Life Threatening ◽  
Delays In Diagnosis ◽  
Oral Involvement ◽  
Dental Professionals

Pemphigus is a group of potentially life-threatening autoimmune mucocutaneous diseases characterized by epithelial blistering affecting cutaneous and/ or mucosal surfaces. Pemphigus affects 0.1-0.5 patients per 100,000 population per year. Oral lesions of pemphigus are seen in up to 18% of patients at dermatology out-patient clinics, but despite the frequency of oral involvement, and novel therapeutic approaches, there are surprisingly few recent studies of either the oral manifestations of pemphigus or their management, and delays in diagnosis are still common. Most patients are initially misdiagnosed and improperly treated for many months or even years. Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment, since this in turn determines the prognosis and course of the disease. Pemphigus has been reviewed in the oral literature in the past decade, but several advances in the understanding of the etiopathogenesis, pemphigus variants, and management warrant an update. Here, we report a case of pemphigus vulgaris that was misdiagnosed in its earliest stage. Oral ulceration may arise from a variety of causes. This case illustrates that, although rare, pemphigus vulgaris may need to be included in differential diagnosis.

scholarly journals Prosthodontic Rehabilitation of an Oral Pemphigus Vulgaris Patient

2011 ◽  
Vol 1 (2) ◽  
pp. 128-131
Author(s):  
Ufuk Ates ◽  
Bulem Yuzugullu
Keyword(s):  
Early Diagnosis ◽  
Dental Care ◽  
Oral Hygiene ◽  
Systemic Treatment ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Oral Disease ◽  
Dental Management ◽  
Increased Risk ◽  
Hygiene Measures

ABSTRACT Pemphigus vulgaris is a rare autoimmune mucocutaneous disease with high mortality if untreated. Dental management is complicated due to involvement of oral mucosa, increased risk of oral disease and difficulty in rendering dental care. A male patient with exacerbated characteristics was rehabilitated with full mouth restorations following systemic treatment of pemphigus vulgaris. Owing to the nature of the disease and discomfort in providing adequate oral hygiene measures, dietary intake and masticatory function, it is imperative to rehabilitate the patient with well-designed prosthesis. Although these diseases are relatively uncommon, clinicians must be sufficiently familiar with clinical manifestations to ensure early diagnosis and treatment.

scholarly journals Clinical Study on Outcome of Treatment for Herpes Zoster

2021 ◽  
Author(s):  
Kishan Rasubhai Ninama ◽  
Rashmi Samir Mahajan ◽  
Atmakalyani Rashmi Shah ◽  
Apexa Prakash Jain
Keyword(s):  
Herpes Zoster ◽  
Lupus Erythematosus ◽  
De Novo ◽  
Clinical Manifestations ◽  
Pemphigus Vulgaris ◽  
Clinical History ◽  
Skin Lesions ◽  
Enzyme Linked Immunosorbent Assay ◽  
Oral Acyclovir ◽  
Varicella Zoster

Introduction: Herpes Zoster (HZ) is caused by reactivation of Varicella Zoster Virus (VZV). It is characterised by occurrence of grouped vesicles on erythematous base which involves the entire dermatome innervated by a single spinal or cranial sensory ganglion and is associated with radicular pain. Antivirals (Acyclovir, Famciclovir and Valacyclovir) started within 72 hours of onset of lesions are the agents of choice. Aim: To study the clinical manifestations, comorbidities, efficacy and safety of Acyclovir, complications and sequelae associated with HZ. Materials and Methods: A 3-year longitudinal cohort study was conducted in 212 adult patients (>18 years of age) suffering with HZ in the Department of Dermatology, Dhiraj General Hospital, Pipariya, Gujarat, India. In this study 212 patients with HZ were prescribed oral Acyclovir in a dose of 800 mg 5 times a day for 7 days. All patients were analysed in terms of clinical manifestations, pre-existing co-morbidities and incidence of complications. The clinical history and findings were recorded in a prestructured proforma. All patients were subjected to cytological examination (Tzanck smear) and Human immunodeficiency viruses (HIV) testing Enzyme-Linked Immunosorbent Assay (ELISA). Diagnosis was made primarily on the basis of clinical findings and presence of multinucleated giant cells in Tzanck smear. All the patients were treated with Oral Acyclovir. Cases were followed-up fortnightly for six weeks and evaluated for relief of symptoms, treatment outcome and complications/sequelae. Results: Two hundred and twelve cases were studied. One hundred and forty-two cases were in the 4th and 5th decades of life. Sixty-three cases had comorbidities like diabetes mellitus in 31, autoimmune diseases like pemphigus vulgaris, systemic lupus erythematosus, rheumatoid arthritis and inflammatory bowel disease in 19 and AIDS in 8 cases. Five cases had malignancy/lymphomas and were receiving chemotherapy for the same. In the majority, HZ occurred de novo without any comorbidities. The most common dermatomes involved were cervical and thoracic. Out of 212 cases Oral Acyclovir 800 mg was well tolerated by 74. Most common complication was Postherpetic Neuralgia (PHN), seen in 80 cases. Conclusion: The treatment of HZ with Oral Acyclovir 800 mg 5 times a day for 7 days is efficacious for healing of skin lesions and also reduces the chances of PHN if instituted within 72 hours.

scholarly journals IgA nephropathy suspected to be combined with Fabry disease or Alport syndrome: a case report

2019 ◽  
Vol 48 (3) ◽  
pp. 030006051989129
Author(s):  
Wen Hao ◽  
Lina Ao ◽  
Chenli Zhang ◽  
Lei Zhu ◽  
Deqiong Xie
Keyword(s):  
Fabry Disease ◽  
Renal Biopsy ◽  
Iga Nephropathy ◽  
Alport Syndrome ◽  
Immunoglobulin A ◽  
Clinical Manifestations ◽  
Hereditary Diseases ◽  
Zebra Bodies ◽  
Gene Test ◽  
Laboratory Examinations

Immunoglobulin A (IgA) nephropathy is the most common glomerular disease, and it often manifests as persistent microscopic hematuria or gross hematuria. Fabry disease and Alport syndrome are hereditary diseases caused by mutation of genes, and these diseases are rare in China. At present, patients can be diagnosed with IgA nephropathy by clinical manifestations and laboratory examinations, but there is still controversy about the simultaneous diagnosis of Alport syndrome and Fabry disease in patients with IgA nephropathy. The present case was a 17-year-old girl with hematuria and proteinuria who underwent a renal biopsy. Light microscopy and immunofluorescence showed that IgA was deposited in the mesangium. Under electron microscopy, zebra bodies with a lamellated structure were detected. A gene test showed a COL4A3 gene mutation. The patient was administered prednisone 40 mg once a day and dispersible tablets of mycophenolate mofetil 0.75 g two times a day. The patient’s condition showed a trend of remission. The findings in our case emphasize the importance of renal biopsy and gene detection in hereditary kidney disease, especially for Fabry disease and its rare coexistence with Alport syndrome.

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