Papulonodular mucinosis, Guillain-Barre syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report.
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SUBMITTER: Su X
PROVIDER: S-EPMC5286654 | biostudies-other | 2017 Feb
REPOSITORIES: biostudies-other
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Project description:INTRODUCTION: Bullous systemic lupus erythematosus (BSLE) is an autoantibody-mediated disease with subepidermal blisters. It is a rare form of presentation of SLE that occurs in less than 5% of cases of lupus. CASE REPORT: A 27-year-old, female, FRS patient reported the appearance of painful bullous lesions in the left nasal wing and left buccal mucosa that displayed sudden and rapid growth. She sought advice from emergency dermatology staff 15 days after onset and was hospitalized with suspected bullous disease. Intravenous antibiotics and steroids were administered initially, but the patient showed no improvement during hospitalization. She displayed further extensive injuries to the trunk, axillae, and vulva as well as disruption of the bullous lesions, which remained as hyperemic scars. Incisional biopsy of a lesion in the left buccal mucosa was performed, and pathological results indicated mucositis with extensive erosion and the presence of a predominantly neutrophilic infiltrate with degeneration of basal cells and apoptotic keratinocytes. Under direct immunofluorescence, the skin showed anti-IgA, anti-IgM, and anti-IgG linear fluorescence on the continuous dermal side of the cleavage. Indirect immunofluorescence of the skin showed conjugated anti-IgA, was anti-IgM negative, and displayed pemphigus in conjunction with anti-IgG fluorescence in the nucleus of keratinocytes, consistent with a diagnosis of bullous lupus erythematosus. DISCUSSION: BSLE is an acquired autoimmune bullous disease caused by autoantibodies against type VII collagen or other components of the junctional zone, epidermis, and dermis. It must be differentiated from the secondary bubbles and vacuolar degeneration of the basement membrane that may occur in acute and subacute cutaneous lupus erythematosus.
| S-EPMC4423270 | biostudies-other
Project description:Guillain-Barre syndrome (GBS) is a severe acute paralytic neuropathy with rapid progression usually occurring post infections. Inspite of the active medications it is associated with severe weakness, incomplete recovery and pain. Long disease course can cause autonomic dysfunction or deterioration in general health and life threatening complications like respiratory failures. Current case was diagnosed as GBS with motor, sensory & sphincter disturbance. Ayurveda diagnosis of Sarvangavata was made and customized treatment strategy was planned. First part of Kapha pitta samrushtavata (Vatadosha associated with Kapha and Pitta dosha) and then vatahara chikitsa were followed. Treatments were Koshta shodhana (gut cleansing), Abhyanga (massage of whole body with medicated oil), Ksheera parisheka (dripping of medicated milk over body), Shastikashali panda sweda (Rubbing of medicated rice poultice over body), Anna lepa (application of medicated rice over the body), Shirotalam (trans cranial drug administration by applying medicines over scalp), Basti (trans rectal administration of medicines) and Oral medicaments. Panchakarma treatments were for 14 days followed by oral medications for next 151 days. Intervention period of 165 days showed complete recovery of all the motor, sensory & sphincter deficits however follow up of the patient was maintained for 437 days looking in to the sustainability of the outcomes.
| S-EPMC7125361 | biostudies-literature
Project description:Novel outbreak with coronavirus 2019 began since 31 December 2019. Coronaviruses can cause multiple systemic infections that respiratory complications are the most obvious symptoms. In this report, we describe the symptoms of Guillain Barre syndrome (GBS) in one infected patient with COVID-19, for the first time. We reported a 65-years- old male patient with complaints of acute progressive symmetric ascending quadriparesis. Two weeks prior to hospitalization, the patient suffered from cough, fever, and RT-PCR was reported positive for COVID-19 infection. The electrodiagnostic test showed that the patient is an AMSAN variant of GBS. COVID-19 stimulates inflammatory cells and produces various inflammatory cytokines and as a result, it creates immune-mediated processes. GBS is an immune-mediated disorder and molecular mimicry as a mechanism of autoimmune disorder plays an important role in creating it. It is unclear whether COVID-19 induces the production of antibodies against specific gangliosides. Further investigations should be conducted about the mechanism of GBS in patients with COVID-19, in the future.
| S-EPMC7158817 | biostudies-literature
Project description:IntroductionInvasive aspergillosis (IA) is a fungal infection caused by Aspergillus species (spp.). Aspergillosis is the most common source of opportunistic fungal infection in humans. IA can cause serious complications related to high morbidity and mortality in immunocompromised patients.Presentation of caseWe report a case of a 22-year-old female with a chief complaint of having a hole in the roof of her mouth. She was diagnosed with SLE in 2009. She had been consuming oral methylprednisolone ever since. In 2018, she experienced worsened symptoms and was hospitalized. She experienced swelling and bleeding of her gums and some of her teeth becoming loose and falling out, and then developing a hole in the roof of her mouth. Subsequently, she was treated with oral cyclophosphamide, oral mycophenolate sodium, and oral fluconazole. She was asked to stop taking oral methylprednisolone. In 2019, the palate biopsy was performed and showed Aspergillus spp. invading the palate. Afterward, the patient was referred to our clinic for defect closure. The patient was operated on for debridement and reconstruction of the defect. There was no recurrence of the defect or complications observed in the follow-up. The patient was satisfied with the surgical results.DiscussionIA is a destructive and potentially harmful opportunistic fungal infection and treatments with surgical interventions should be well-thought-out in immunocompromised patients.ConclusionThe management of IA are controlling any underlying diseases and surgical debridement or necrotomy. Generally, antifungal therapy and prompt surgical intervention are successful in managing invasive aspergillosis.
| S-EPMC7484496 | biostudies-literature
Project description:A 28-year-old female patient was hospitalized primarily because of "intermittent fever for 28 days aggravated by systemic rashes, oral ulcer, and edema in both eyelids for 5 days." During treatment, convulsions and loss of consciousness occurred. Magnetic resonance imaging (MRI) of the head revealed an abnormal signal with shadows in the bilateral frontal, parietal, temporal, and occipital lobes; cerebellar hemispheres; and basal nodes, with high signal intensity on T2 weighted imaging (T2WI), on fluid-attenuated inversion-recovery, and of the apparent diffusion coefficient and low signal intensity on T1WI and diffusion weighted imaging. Therefore, the patient was diagnosed with systemic lupus erythematosus (SLE) with reversible posterior encephalopathy syndrome (RPES). Intravenous high-dose methylprednisolone and cyclophosphamide were administered for blood pressure control, which effectively controlled the disease. Therefore, when patients with SLE and hypertension or renal insufficiency or those receiving high-dose methylprednisolone or immunosuppressants suddenly present with neurologic abnormalities, a diagnosis of RPES must be considered, and head MRI is the first choice for diagnosis of this disease. In terms of treatment, the blood pressure should be quickly controlled, and the primary disease should be aggressively treated.
| S-EPMC8326631 | biostudies-literature
Project description:In recent months, the new beta-coronavirus has caused a pandemic with symptoms affecting mainly the respiratory system. It is established that the virus may play a neurotropic role and in recent months several cases of Guillain-Barré-Strohl syndrome (GBS) have been reported in patients infected with COVID-19. We report the case of a 54-year-old patient with acute demyelinating polyneuropathy during infection by SARS-CoV-2 who progressed clinically to require assisted ventilation. After several weeks of specific symptomatic treatment, the patient had a favorable outcome. In conclusion, despite being a rare complication, we think it is important to consider the possibility of diffuse involvement of the peripheral nervous system in patients with COVID-19 to adjust clinical monitoring and treatment in these cases.
| S-EPMC7420397 | biostudies-literature
Project description:INTRODUCTION: Systemic lupus erythematosus is a multi-system connective tissue disorder. Peripheral neuropathy is a known and underestimated complication in systemic lupus erythematosus. Ganglionopathy manifests when neuronal cell bodies in the dorsal root ganglion are involved. Autoimmune disorders are a known etiology, with systemic lupus erythematosus being a rare cause. CASE PRESENTATION: A 32-year-old South Asian woman presented with oral ulceration involving her lips following initiation of treatment for a febrile illness associated with dysuria. She had a history of progressively worsening numbness over a period of 4 months involving both the upper and lower limbs symmetrically while sparing the trunk. Her vibration sense was impaired, and her reflexes were diminished. For the past 4 years, she had had a bilateral, symmetrical, non-deforming arthritis involving the upper and lower limbs. Her anti-nuclear antibody and anti-double-stranded deoxyribonucleic acid status were positive. Although her anti-Ro antibodies were positive, she did not have clinical features suggestive of Sjögren syndrome. Nerve conduction studies revealed sensory neuronopathy. A diagnosis of systemic lupus erythematosus complicated by sensory neuronopathy was made. Treatment with intravenous immunoglobulin resulted in clinical and electrophysiological improvement. CONCLUSION: Peripheral neuropathy in systemic lupus erythematosus can, by itself, be a disabling feature. Nerve conduction studies should be considered when relevant. Neuropathy in systemic lupus erythematosus should be given greater recognition, and rarer forms of presentation should be entertained in the differential diagnosis when the clinical picture is atypical. Intravenous immunoglobulin may have role in treatment of sensory neuronopathy in systemic lupus erythematosus.
| S-EPMC4229803 | biostudies-other
Project description:Introduction: Lichen planus/lupus erythematosus overlap syndrome is rarely seen in the clinic and has the characteristic clinical manifestations, histopathology, and immunopathology of lichen planus (LP) and lupus erythematosus (LE). This is the first reported case of bullous lichen planus (BLP)/systemic lupus erythematosus (SLE) overlap syndrome with hair loss as the first symptom. Case Presentation: A 48-year-old female presented with alopecia for half a year, and skin lesions accompanied by itching on her face, trunk, and limbs for 3 months. She had a history suggestive of photosensitivity. Laboratory tests and histopathology were performed for diagnosis. Histopathologic features of the upper arm and back of the hand were consistent with BLP, whereas the scalp lesion indicated LE. Laboratory examination indicated positive for antinuclear antibody (ANA) (1:160), leukopenia, increased urinary protein, decreased C3/C4, and normal BP180. The patient was given glucocorticoid combined with acitretin and immunosuppressive therapy after a definite diagnosis of BLP/SLE overlap syndrome. The lesions of the patient disappeared and some hair had regrown during the two years of follow-up. Conclusion: This is the first reported case of BLP/SLE overlap syndrome which responded well to glucocorticoids, retinoids, and immunosuppressive drugs. Multiple biopsies from characteristic lesions will guide doctors to avoid misdiagnoses and delayed treatment.
| S-EPMC8599928 | biostudies-literature
Project description:During the recent coronavirus disease 2019 (COVID-19) outbreak in Northern Italy, we observed a 57-year-old man developing acute motor-sensory axonal neuropathy, a variant of Guillain-Barré syndrome (GBS), 12 days after severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2) infection. Similarly to other bacterial and viral infections, dysregulation of the immune system due to post-infectious mechanisms, such as the molecular mimicry, could lead to an indirect damage of the peripheral nervous system related to SARS-CoV-2. GBS causes motor dysfunctions that are not easily recognizable in non-neurological settings or in patients requiring ventilatory assistance. Several reports also suggested that GBS and Miller Fisher syndrome (MFS) could be neurological complications of COVID-19. Therefore, we performed a review of the 29 articles so far published, describing 33 GBS cases and five MFS cases associated with SARS-CoV-2 infection. We recommend awareness of this rare, but treatable, neurological syndrome, which may also determine a sudden and otherwise unexplained respiratory deterioration in COVID-19 patients.
| S-EPMC7471770 | biostudies-literature
Project description:BackgroundHepatitis E infection is a global disorder that causes substantial morbidity. Numerous neurologic illnesses, including Guillain-Barre syndrome (GBS), have occurred in patients with hepatitis E virus (HEV) infection.Case presentationWe report a 58 year-old non-immunocompromised man who presented with progressive muscle weakness in all extremities during an episode of acute HEV infection, which was confirmed by measuring the anti-HEV IgM antibodies in the serum. Both cerebrospinal fluid examination and electrophysiological study were in agreement with the diagnosis of HEV-associated GBS. Following the treatment with intravenous immunoglobulin, the patient's neurological condition improved rapidly.ConclusionsHEV infection should be strongly considered in patients with neurological symptoms, especially those with elevated levels of liver enzymes.
| S-EPMC5778630 | biostudies-literature