Disseminated Verruconis gallopava infection in a patient with systemic lupus erythematosus in Japan: A case report, literature review, and autopsy case.
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SUBMITTER: Murata K
PROVIDER: S-EPMC8783064 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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:Immune-mediated inflammatory diseases are a group of diseases characterized by generalized inflammation that results from immune dysregulation, especially involving the mechanisms of acquired immunity. These diseases may be familial, showing that genetic factors play an important role in their development. Additionally, the occurrence of one disease makes a patient prone to other diseases. However, the coexistence of systemic lupus erythematosus (SLE) and psoriasis (Ps) is very rare due to their distinct genetic determinants and mechanisms of pathogenesis. Treatment is also challenging, as medications used to treat one condition exacerbate or even trigger the symptoms of the other. This paper presents the case of a Ps patient with a family history of autoimmune diseases, who developed systemic lupus erythematosus during puberty, as well as a discussion on the coexistence of SLE and Ps in developmental age based on available literature searching for PubMed database and American College of Rheumatology and European League Against Rheumatism abstracts particularly in this subject.
| S-EPMC7174791 | 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:BackgroundSystemic erythematous lupus (SLE) is an autoimmune disease associated with significant cardiovascular morbidity and mortality, even in young patients.Case summaryA young female with recently diagnosed SLE under corticotherapy developed pulmonary oedema and respiratory failure. The diagnostic workup revealed mildly elevated cardiac troponin, significantly elevated NT-proB-type natriuretic peptide (NT-proBNP) and mild pericardial effusion without other echocardiographic abnormalities. Systemic erythematous lupus-associated myocarditis was presumed, and her clinical status improved after corticotherapy intensification. However, transthoracic echocardiogram repeated days later revealed a large pericardial effusion with findings suggestive of a contained myocardial rupture originating in the inferolateral basal left ventricular (LV) segment, which was confirmed by computed tomography scan. Cardiac catheterization exhibited normal coronary arteries. The patient was submitted to cardiac surgery and the LV pseudoaneurysm was successfully repaired.DiscussionMyocardial rupture with LV pseudoaneurysm formation usually occurs in the setting of acute myocardial infarction, but also in other rare contexts. Cardiac rupture is associated with an extremely high mortality unless early diagnosis and urgent surgical intervention are provided.
| S-EPMC7850616 | biostudies-literature
Project description:We report a rare case of dengue fever triggering systemic lupus erythematosus and lupus nephritis. The patient presented herself during a large outbreak of dengue fever in December 2012 in Maharashtra, India. The diagnosis of dengue fever was confirmed by the presence of NS-1 antigen during the first few days of febrile illness. Eight weeks later, kidney tissue biopsy studies revealed evidence of lupus nephritis on microscopic examination and immunofluorescence. The report interpreted it as focal proliferative glomerulonephritis and segmental sclerosis (Stage IIIC). The case was also found positive for perinuclear antineutrophil cytoplasmic antibodies by indirect immunofluorescence assay. An active and effective management of a case essentially calls for clear perception of differentiating dengue-induced lupus flare, antineutrophil cytoplasmic antibody-related nephropathy, and/or dengue-induced de-novo lupus disease. Dengue viremia may be the trigger for immune complex formation in patients who are predisposed to developing autoimmune diseases. The present case explains the importance of considering the diagnosis of dengue-related lupus nephritis as an atypical occurrence in appropriate situations, as in this case. It would not be improper to regard this escalating disease as an expanded feature of dengue.
| S-EPMC3818025 | biostudies-literature
Project description:There are hundreds of twin adult patients with systemic lupus erythematosus (SLE), but male children with SLE are rarely affected. Two monozygotic twin brothers developed SLE at the age of 11 years during 1 month. The index brother manifested with Henoch-Shonlein purpura, accompanied by ANA positivity, and later developed critical left femoral arterial stenosis with high levels of anti-dsDNA, antiphospholipid antibodies, hypocomplementemia, and Coombs-positive hemolytic anemia. At that time his twin brother had only identical autoimmune findings and developed clinical manifestation (myositis and fasciitis) a month later. Both twins had increased IFN-score and shared a heterozygous variant in the RNASEL gene. Index patients developed scalp rash and nephritis 6 months after their parents refused the treatment which has been lasted for 1 year after disease diagnostics.ConclusionThe simultaneous onset of the pediatric SLE in the male twin is a very rare situation suspected monogenic origin of the disease. Further functional studies are required to confirm the causative role of the mutation.
| S-EPMC9243501 | 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: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:Lysinuric protein intolerance (LPI) is an inborn error of metabolism caused by defective transport of cationic amino acids in epithelial cells of intestines, kidneys and other tissues as well as non-epithelial cells including macrophages. LPI is caused by biallelic, pathogenic variants in SLC7A7. The clinical phenotype of LPI includes failure to thrive and multi-system disease including hematologic, neurologic, pulmonary and renal manifestations. Individual presentations are extremely variable, often leading to misdiagnosis or delayed diagnosis. Here we describe a patient that clinically presented with immune dysregulation in the setting of early-onset systemic lupus erythematosus (SLE), including renal involvement, in whom an LPI diagnosis was suspected post-mortem based on exome sequencing analysis. A review of the literature was performed to provide an overview of the clinical spectrum and immune mechanisms involved in this disease. The precise mechanism by which ineffective amino acid transport triggers systemic inflammatory features is not yet understood. However, LPI should be considered in the differential diagnosis of early-onset SLE, particularly in the absence of response to immunosuppressive therapy.
| S-EPMC8172984 | biostudies-literature
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