Clinical case of effective treatment of gestational pemphigoid with intravenous immunoglobulins

Gestational pemphigus — is a rare autoimmune condition that manifest during pregnancy. This pathology was described for the first time in 1827 by the Karl Martius, but until now remains insufficiently studied and as a result, difficulties arise in the differential diagnosis and treatment. Clinical manifestations of the disease characterized by the appearance of a polymorphic rash on the skin and mucous membranes. In patients with impaired immune tolerance pemphigus can be complicated by hyperactivation of the immune system. To date, pregnancy is not considered as a factor that can aggravate the course of pemphigus, since during pregnancy the disease can worsen, enter into complete remission, or remain unchanged. In the literature, there is information about the manifestation of pemphigus in puerperium after rapid decrease of corticosteroid hormones in serum. The clinical course of gestational pemphigus is variable and can lead to diagnostic failures. The diagnosis usually made according to the clinic, histopathology and laboratory findings. In this case report we describe a case of diagnosis and treatment of gestational pemphigoid, refractory to corticosteroid therapy. Clinical improvement and recovery achieved after a course of high doses of intravenous immunoglobulins. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: pemphigoid, pregnancy, autoimmune disease, intravenous immunoglobulin.

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Legionnaire's disease in postoperative neurosurgical patients

Journal of Neurosurgery ◽

10.3171/jns.1983.59.4.0596 ◽

1983 ◽

Vol 59 (4) ◽

pp. 596-600 ◽

Cited By ~ 7

Author(s):

Mark C. Glazier ◽

Richard B. Kohler ◽

Robert L. Campbell

Keyword(s):

Legionella Pneumophila ◽

Clinical Course ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Surgical Patients ◽

Content Type ◽

Neurosurgical Patients ◽

Legionnaires Disease ◽

High Doses ◽

Fine Print

✓ Legionella pneumophila postoperative pneumonia may be an important cause of morbidity and mortality in selected surgical patients. This report presents five postoperative neurosurgical patients in whom the diagnosis of Legionnaires' disease was made. Their clinical course and treatment are presented. Clinical manifestations, methods of diagnosis, and treatment of L. pneumophila pneumonia are discussed. It is pointed out that neurosurgical patients who have received high doses of corticosteroids and who develop nosocomial postoperative pneumonias should be suspected of having Legionnaires' disease.

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Scleroderma and Related Disorders

10.2310/fm.1211 ◽

2017 ◽

Author(s):

Kristine Phillips

Keyword(s):

Differential Diagnosis ◽

Antinuclear Antibodies ◽

Clinical Course ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Localized Scleroderma ◽

Eosinophilic Fasciitis ◽

Tomographic Images ◽

Computed Tomographic

Scleroderma spectrum diseases are a heterogeneous group of disorders that are distinguished by abnormalities of the connective tissue in the skin and, in some cases, other organs. Each disorder may be characterized by the extent of cutaneous and internal involvement, as well as histopathologic features of skin biopsy. Scleroderma spectrum diseases include systemic scleroderma, localized scleroderma, and eosinophilic fasciitis. This chapter reviews the classification, epidemiology, etiology, pathophysiology and pathogenesis, diagnosis, differential diagnosis, treatment, outcome measures, management, and clinical course of scleroderma as well as the definition and classification, etiology/genetics, differential diagnosis, and treatment of localized scleroderma. Also discussed are the definition and classification, epidemiology, etiology/genetics/pathogenesis, diagnosis, differential diagnosis, and treatment of eosinophilic fasciitis. Tables review the classification of—and antinuclear antibodies in—scleroderma as well as the key assessments and interventions in scleroderma management. Figures illustrate the disease's presentation and clinical manifestations, including several images of scleroderma of the hands; face, palmar, and buccal telangiectasias in a patient with scleroderma; a radiograph demonstrating calcinosis of the elbow; Raynaud’s phenomenon; high-resolution computed tomographic images of diffuse cutaneous scleroderma, scleroderma and severe pulmonary hypertension, and limited cutaneous scleroderma; plus an esophagram demonstrating hypomotility. This review contains 11 highly rendered figures, 3 tables, and 72 references.

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On the clinical course of multiforme exudative erythema of the oral cavity (literature review)

Periodontology ◽

10.33925/1683-3759-2020-25-1-71-74 ◽

2020 ◽

Vol 25 (1) ◽

pp. 71-74

Author(s):

A. I. Bulgakova ◽

Z. R. Hismatullina ◽

M. V. Zatsepina ◽

J. A. Kudryavtseva

Keyword(s):

Literature Review ◽

Oral Cavity ◽

Mucous Membrane ◽

Oral Mucosa ◽

Erythema Multiforme ◽

Clinical Picture ◽

Clinical Course ◽

Clinical Manifestations ◽

Timely Diagnosis ◽

Mucous Membranes

Relevance. Multiform exudative erythema is a polyetiological disease, which is characterized by damage not only to the skin, but also to the mucous membranes, mainly the mucous membrane of the oral cavity. The article reviews the literature on the clinical course of multiform exudative erythema of the oral cavity.Purpose. Study the clinical course of erythema multiforme.Materials and methods. We conducted a review of studies of the etiology, pathogenesis, prevalence of erythema multiforme according to domestic and foreign authors, and also reflected the identification of various forms and clinical manifestations of this disease.Results. The analysis of clinical manifestations of erythema multiforme exudative in the oral cavity, the most common symptoms and manifestations on the oral mucosa.Conclusion. The clinical picture of lesions of the oral mucosa in multiform exudative erythema is very diverse, which is important for the timely diagnosis of the disease.

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Scleroderma and Related Disorders

10.2310/im.1211 ◽

2017 ◽

Author(s):

Kristine Phillips

Keyword(s):

Differential Diagnosis ◽

Antinuclear Antibodies ◽

Clinical Course ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Localized Scleroderma ◽

Eosinophilic Fasciitis ◽

Tomographic Images ◽

Computed Tomographic

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Rhabdomyolysis in a case of free-base cocaine ("crack") overdose.

Clinical Chemistry ◽

10.1093/clinchem/35.7.1547 ◽

1989 ◽

Vol 35 (7) ◽

pp. 1547-1549 ◽

Cited By ~ 12

Author(s):

M A Jandreski ◽

E W Bermes ◽

R Leischner ◽

S E Kahn

Keyword(s):

Renal Failure ◽

Acute Renal Failure ◽

Crack Cocaine ◽

Clinical Course ◽

Medical Center ◽

Clinical Manifestations ◽

Blood Chemistry ◽

Free Base ◽

Laboratory Findings ◽

Remarkable Recovery

Abstract This is the case of a 27-year-old black man who was admitted to Loyola University Medical Center after a one-time experience of smoking free-base ("crack") cocaine. Clinical manifestations of the resulting cocaine intoxication were rhabdomyolysis, acute renal failure, and transient liver failure. This patient came to our attention because of the striking alterations in his blood-chemistry values, which indicated acute tissue damage, and his remarkable recovery within 96 h. We discuss the dramatic changes in the laboratory findings and the clinical course of this patient.

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Vogt-Koyanagi-Harada disease in Spain

European Journal of Ophthalmology ◽

10.1177/11206721211033477 ◽

2021 ◽

pp. 112067212110334

Author(s):

Pedro Arriola-Villalobos ◽

Aina Moll-Udina ◽

Mario Carrasco-López-Brea ◽

Cristina Sacristan ◽

Maria Jose Capella ◽

...

Keyword(s):

Clinical Course ◽

Clinical Manifestations ◽

Acute Stage ◽

Tertiary Referral ◽

Stage Disease ◽

Maintenance Stage ◽

Multicenter Analysis ◽

Epidemiological Characteristics ◽

First Time

Purpose: To describe the clinical and epidemiological characteristics of patients with Vogt-Koyanagi-Harada (VKH) disease in Spain. Methods: This was a retrospective multicenter analysis of data from VKH patients followed for at least 6 months. The data collected were related to demographics, clinical manifestations, treatments, and complications. Results: Participants were 112 patients (224 eyes), from 13 tertiary referral centers, of mean age 37.5 ± 14.7 years; 83.9% were women. Ethnicities were 61.6% Caucasian and 30.4% Hispanic. The disease was classified as complete in 16.1%, incomplete in 55.4%, and probable in 28.6%. When seen for the first time, the clinical course was acute in 69.6%, recurrent chronic in 15.2%, and chronic in 14.3%. The most frequent treatment was corticosteroids (acute stage 42.2%, maintenance stage 55.6%). The most common complications were cataract (41.1%) and ocular hypertension (16.1%). In most eyes, visual acuity was improved (96.7%) or remained stable at the end of follow up. Conclusion: VKH in Spain mostly affects women and presents as incomplete acute stage disease. Visual prognosis is good. Cataract and glaucoma are the two most frequent complications.

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Clinical research of primary liver cancer mimicking liver abscess

International Surgery Journal ◽

10.18203/2349-2902.isj20170857 ◽

2017 ◽

Vol 4 (3) ◽

pp. 1033

Author(s):

Kong Jun ◽

Leng Tongmin ◽

Gong Jianping ◽

Tang Maoming

Keyword(s):

Liver Cancer ◽

Liver Abscess ◽

Early Stage ◽

Primary Liver Cancer ◽

Clinical Manifestations ◽

Pyogenic Liver Abscess ◽

Diagnosis And Treatment ◽

Hepatic Arteriography ◽

Laboratory Findings ◽

Post Operation

Background: Aiming at diagnosing at an early stage, minimizing the misdiagnosis rate and improving the prognosis, the author has investigated the clinical characteristics, diagnosis and treatment of primary liver cancer mimicking liver abscess with a summary and discussion.Methods: All of the 11 cases of primary liver cancer mimicking liver abscess, diagnosed during January 2009 to December 2015, were retrospectively viewed in terms of clinical manifestations, laboratory tests, radiological feature, diagnosis and treatment. And statistic analysis was made in all aspects mentioned above with that of pyogenic liver abscess and other types of liver cancer diagnosed in the corresponding period. Results: The clinical manifestations of the 11 cases were mostly characterized by fever, abdominal pain and weight loss. There was no significantly statistic difference between the study group and any of the three matched groups in underlying disease and lab results. All of the 11 patients were treated with enhanced antibiotics as basic therapy. Furthermore, eight cases accepted surgical operation, among them, one object underwent puncture and drainage of the liver abscess by ultrasound (PDLA) twice pre-operation, one object underwent PDLA and hepatic arteriography pre-operation and death in hospital post-operation, one object suffered myocardial infarction post-operation. In addition, three cases received conservative treatment only, in which, one object died in hospital.Conclusions: It is difficult to distinguish primary liver cancer mimicking liver abscess from pyogenic liver abscess as there are no specific clinical manifestations and laboratory findings which is prone to leading to misdiagnosis. What’s worse, the prognosis is so poor that high alert and close follow-up are required as well as early diagnosis and treatment.

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SKIN LESIONS IN CHILDREN WITH COVID-19-ASSOCIATED MULTISYSTEM INFLAMMATORY SYNDROME

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2020-99-6-259-265 ◽

2020 ◽

Vol 99 (6) ◽

pp. 259-265

Author(s):

O.B. Tamrazova ◽

◽

I.M. Osmanov ◽

A.S. Stadnikova ◽

L.N. Mazankova ◽

...

Keyword(s):

Clinical Observation ◽

Clinical Manifestations ◽

Chinese American ◽

Skin Lesions ◽

Disease Course ◽

Skin Manifestations ◽

Mucous Membranes ◽

Coronavirus Infection ◽

Inflammatory Syndrome ◽

First Time

The pandemic of the new coronavirus infection COVID-19, caused by SARS-CoV-2 coronavirus, continues to develop. The first data indicated that children tolerate this infection much easier than adults, the disease course is generally asymptomatic and mild. Chinese, American and European scientists described a variety of skin manifestations in children with COVID-19 coronavirus infection. The article presents literature data on skin manifestations in children, characteristic for COVID-19. In April 2020, the description of a new severe inflammatory syndrome in children associated with SARS-CoV-2, called multisystem inflammation syndrome (MIS), appeared for the first time. The article presents literature data on MIS clinical manifestations and its incidence. The authors describe clinical observation of MIS development in a 7-year-old child with COVID-19 and with lesions of the skin and mucous membranes.

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Novel heterozygous PIK3CD mutation presenting with only laboratory markers of combined immunodeficiency

LymphoSign Journal ◽

10.14785/lymphosign-2020-0003 ◽

2020 ◽

Vol 7 (2) ◽

pp. 49-55

Author(s):

Amarilla B. Mandola ◽

Harjit Dadi ◽

Brenda Reid ◽

Chaim M. Roifman

Keyword(s):

Newborn Screening ◽

Catalytic Domain ◽

Clinical Course ◽

Phenotypic Variability ◽

Severe Combined Immunodeficiency ◽

Clinical Manifestations ◽

Combined Immunodeficiency ◽

Laboratory Findings ◽

Number Of Patients ◽

Increased Risk

Introduction: Phosphatidylinositol-4,5-Bisphosphate 3-Kinase Catalytic Subunit Delta (PIK3CD) is one part of a heterodimer forming the enzyme phosphoinositide 3-kinase (PI3K), found primarily in leukocytes. PIK3CD generates phosphatidyl-inositol 3,4,5-trisphosphate (PIP3), and is involved in cell growth, survival, proliferation, motility, and morphology. An increasing number of patients have been described with heterozygous PIK3CD gain-of-function (GOF) mutations, leading to combined immunodeficiency with both B- and T-cell dysfunction. Patients suffer recurrent respiratory infections, often associated with bronchiectasis and ear and sinus damage, as well as severe recurrent or persistent infections by herpesviruses, including EBV-induced lymphoproliferation. Aim: To present the clinical phenotypic variability of a novel PI3KCD mutation within a family. Methods: Patient information was collected prospectively and retrospectively from medical records. Comprehensive immune work up, genetic, and signaling evaluation was performed. Results: We describe here 2 patients, daughter and mother, with heterozygous PIK3CD mutation identified by whole exome sequencing and Sanger confirmation. The child was screen-positive by newborn screening for severe combined immunodeficiency (SCID). Cellular assays revealed an increase in the baseline phosphorylation of T cells in the patient. Furthermore, both patients had hyper-activation of the catalytic domain, resulting in increased phosphorylation of AKT upon activation. Discussion: GOF mutations affecting the PIK3CD gene are associated with an increased risk for lymphoproliferation leading to Activated PIK3-delta syndrome (APDS). The clinical course of APDS is highly variable, ranging from combined immunodeficiency with recurrent infections, autoimmune complications, and requiring stem cell transplantation, through isolated antibody deficiency, to asymptomatic adults. Our patient is the first to be identified by newborn screening for SCID. Surprisingly, the clinical course has so far been unremarkable, as well, the mother appears to be completely asymptomatic. Nevertheless, the persistent lymphopenia indicates PIK3CD dysfunction. Because of the wide gap between laboratory findings and clinical manifestations, this kindred poses both a diagnostic as well treatment challenge. Statement of novelty: We report here a novel PIK3CD mutation diagnosed due to abnormal newborn screen for SCID.

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Clinical presentations and long-term prognosis of childhood-onset polyarteritis nodosa in a single centre: a retrospective study

10.21203/rs.3.rs-62870/v1 ◽

2020 ◽

Author(s):

Jeong-Seon Lee ◽

JoongGon Kim ◽

Soyoung Lee

Keyword(s):

Polyarteritis Nodosa ◽

Treatment Guidelines ◽

Clinical Manifestations ◽

Diagnosis And Treatment ◽

Antineutrophil Cytoplasmic Antibodies ◽

Childhood Onset ◽

Single Centre ◽

Laboratory Findings ◽

Paediatric Patients

Abstract Background: Polyarteritis nodosa (PAN) is a systematic necrotizing vasculitis involving medium-sized arteries. Childhood-onset PAN ranges from mild to severe systematic disease causing damage and early mortality. As it is a rare disease in children, there is less study on clinical features, diagnosis and treatment than adults. And diagnosis occurs relatively late in most paediatric patients with polyarteritis nodosa. Therefore, this study aimed to investigate clinical manifestations, laboratory findings, treatment strategies, and long-term outcomes among patients with childhood-onset polyarteritis nodosa treated at a single-centre in Korea. We further aimed to evaluate the usefulness of the Birmingham Vasculitis Activity Score (BVAS) and Five-Factor Score (FFS) in paediatric patients.Methods: We retrospectively analysed data collected from patients with childhood-onset PAN treated at our institution from March 2003 to February 2020. Results: Nine patients (6 male and 3 female) were included in the study. The median ages at symptom onset and diagnosis were 7.6 (3–17.5) and 7.7 (3.5–17.6) years, respectively. The median follow-up duration was 7.0 (1.6–16.3) years. Eight patients were diagnosed with systemic PAN, while one was diagnosed with cutaneous PAN. All patients exhibited skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. Constitutional symptoms such as fever, arthralgia, myalgia, and weight loss were also observed. Antineutrophil cytoplasmic antibodies were absent in all patients. The median BVAS at diagnosis was 8 (range: 2–29), and one patient had an FFS of 1. Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived despite some complications such as intracranial haemorrhage or digital necrosis requiring amputation.Conclusions: Early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. Further multicentre studies are required to clarify the unique characteristics of childhood-onset PAN and establish treatment guidelines, early detection strategies, and biomarkers. Trial registration: Retrospectively registered.

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