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Bullous Systemic Lupus Erythematosus

BSLE


Presentation

  • The present study presents a case of bullous systemic lupus erythematosus (BSLE) in a pregnant woman.[ncbi.nlm.nih.gov]
Fatigue
  • She also complained of arthralgia about 2 months ago, fatigue and weight loss of 10 kg. Physical examination revealed a malar rash and blisters affecting the abdomen and upper limbs ( Figure 1 , Figure 2 ).[em-consulte.com]
  • Clinical features Relatively common autoimmune disease of women, due to anti-DNA antibodies Fatigue, fever, arthritis, erythematous, bilateral butterfly (malar) rash of the face, renal disease, lymphadenopathy, and serositis Associated with altered immunity[pathologyoutlines.com]
  • Continued Sometimes, changes in blood counts (low red cell count, or anemia ), may cause fatigue , serious infections (low white cell count), or easy bruising or bleeding (low platelet count).[webmd.com]
  • "Treatment and management of chronic fatigue syndrome/myalgic encephalomyelitis: All roads lead to Rome" . British Journal of Pharmacology . 174 (5): 345–369. doi : 10.1111/bph.13702 . PMC 5301046 . PMID 28052319 .[en.wikipedia.org]
Rapidly Progressive Glomerulonephritis
  • Because of the appearance of marked proteinuria, a subsequent renal biopsy, and serological tests, the patient was diagnosed with rapidly progressive glomerulonephritis and systemic lupus erythematosus.[ncbi.nlm.nih.gov]
Melena
  • She denied arthralgias, photosensitivity, fevers or chills, cough, sore throat, hemoptysis, diarrhea or vomiting, epistaxis, hematemesis, or melena.[karger.com]
Photophobia
  • There is also associated complain of itching and photophobia but no complaint of fever or joint pain or history suggestive of Raynaud's phenomenon, or butterfly rash was revealed. Birth history and family history were nonsignificant.[ijpd.in]
Hemorrhagic Bullae
  • Examination of the skin was remarkable for numerous flaccid hemorrhagic bullae on a normal-appearing nonerythematous skin involving both the upper and lower extremities.[karger.com]
Arthralgia
  • A 31-yr-old woman presented with generalized erythematous patches, multiple bullae, arthralgia, fever, conjunctivitis, and hemolytic anemia.[ncbi.nlm.nih.gov]
  • She also complained of arthralgia about 2 months ago, fatigue and weight loss of 10 kg. Physical examination revealed a malar rash and blisters affecting the abdomen and upper limbs ( Figure 1 , Figure 2 ).[em-consulte.com]
  • The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease.[researchgate.net]
  • She denied arthralgias, photosensitivity, fevers or chills, cough, sore throat, hemoptysis, diarrhea or vomiting, epistaxis, hematemesis, or melena.[karger.com]
Joint Tenderness
  • Extracutaneous findings of systemic lupus erythematosus may include joint tenderness and swelling, pallor or petechiae (reflecting hematologic abnormalities), and auscultation evidence of a pulmonary or pericardial effusion.[emedicine.medscape.com]
Withdrawn
  • However, the cause of death in the latter case could be related to an APS relapse when the patient developed what has been called "catastrophic APS" after low-dose aspirin was inadvertently withdrawn.[ped-rheum.biomedcentral.com]
  • Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year. The patient has remained in remission without any systemic manifestations as well, over the last 1 year.[ncbi.nlm.nih.gov]
Denial
  • However, the renal biopsy could not be performed because of the denial from the patient's family. Skin biopsy from these bullous lesions revealed subepidermal cleft with a few neutrophil and eosinophils [Figure 3] and [Figure 4].[ijpd.in]
Renal Impairment
  • We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.[ncbi.nlm.nih.gov]
  • We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus. 1. Contestable, JJ, Edhegard, KD, Meyerle, JH.[journals.sagepub.com]
Uremia
  • The blood tests revealed neutropenia (leukocytes 3300/mm 3 and neutrophils 2000/mm 3 ), Uremia at 3.17 g/L, creatinine at 89 mg/L, serum potassium to 7.03 meq/L and albuminuria at 3.4 g/L.[em-consulte.com]

Workup

  • Our patient was initiated on empiric pulsed steroids, while diagnostic workup was in progress, and showed a good clinical response to treatment (fig 2 ).[karger.com]
  • Extensive serological workup revealed antinuclear antibody positivity of 1:640 (using hep-2 cells); positive anti-dsDNA, anti-Ro, and anti-La antibodies; low levels of C3 and C4 with a normal CH50 component; negative antiphospholipid antibody, rheumatoid[ncbi.nlm.nih.gov]
Pyuria
  • We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP.[ncbi.nlm.nih.gov]
Albuminuria
  • The blood tests revealed neutropenia (leukocytes 3300/mm 3 and neutrophils 2000/mm 3 ), Uremia at 3.17 g/L, creatinine at 89 mg/L, serum potassium to 7.03 meq/L and albuminuria at 3.4 g/L.[em-consulte.com]
Heinz Bodies
  • Cessation of dapsone therapy due to hemolysis with Heinz-body formation did not aggravate the bullous disease. Our case illustrates that a generalized vesiculobullous eruption can be the sole presenting manifestation of SLE.[ncbi.nlm.nih.gov]
Viral Inclusion Body
  • No mucin or viral inclusion body is seen. Direct immunofluorescence showed granular IgA and IgG deposition along dermo-epidermal junction with IgG IgA. Also, perivascular IgG deposition was seen in the dermis. She was diagnosed to have BSLE.[ijpd.in]

Treatment

  • It was approved by the US Food and Drug Administration for the treatment of relapsed or refractory low-grade or follicular non-Hodgkin lymphoma and for the treatment of moderate to severely active rheumatoid arthritis.[ncbi.nlm.nih.gov]

Prognosis

  • European Society of Cutaneous Lupus Erythematosus (EUSCLE) Cutaneous lupus erythematosus (CLE) is a disease with a wide spectrum of clinical manifestations and a variable prognosis.[bioportfolio.com]
  • As opposed to bullous SLE, SLE with blisters has not been associated with specific systemic lupus manifestations and therefore does not necessarily imply a worse prognosis.[ped-rheum.biomedcentral.com]
  • Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol 2004;22:121-4. [ PUBMED ] 3. Crowson AN, Magro C. The cutaneous pathology of lupus erythematosus: A review. J Cutan Pathol 2001;28:1-23. 4.[cjhr.org]
  • Clinical course and prognosis of cutaneous lupus erythematosus. Clin Dermatol 2004;22:121-4. 4. Hall RP, Lawley TJ, Smith HR, Katz SI. Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy.[ijpd.in]
  • Differentiating these two disorders is important to inform the physician on optimum management and prognosis.[karger.com]

Etiology

  • Her past medical history was significant for hypertension on thiazides, hyperlipidemia, rheumatoid arthritis (not on disease-modifying antirheumatoid drugs), and thrombocytopenia of unclear etiology.[karger.com]
  • Etiology and Epidemiology The etiology of EBA is unknown, but because the disease features IgG autoantibodies directed against type VII collagen, it is thought that EBA has an autoimmune pathogenesis (Woodley et al. 1984,1988).[rrnursingschool.biz]
  • (See Etiology below.) For patient education information, see Lupus (Systemic Lupus Erythematosus). Etiology The production of autoantibodies represents a central feature of systemic lupus erythematosus.[emedicine.medscape.com]
  • It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system.[fpnotebook.com]

Epidemiology

  • […] receptor biology and its clinical significance in neuropsychiatric SLE; platelet C4d and vascular complications; biomarkers in lupus nephritis; endothelial function and its implications for cardiovascular and renal disease in SLE; changing worldwide epidemiology[books.google.com]
  • This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE. Keywords : bullous systemic lupus erythematosus; dapsone; cutaneous lupus.[scielo.org.co]
  • "Epidemiological trends in skin mycoses worldwide". Mycoses . 51 (Supplement 4): 2–15. doi : 10.1111/j.1439-0507.2008.01606.x . PMID 18783559 . Rivera, ZS; Losada, L; Nierman, WC (October 2012).[en.wikipedia.org]
  • "Epidemiology and pathogenesis of bullous pemphigoid and mucous membrane pemphigoid." UpToDate.com. Apr. 26, 2016..[medicinenet.com]
Sex distribution
Age distribution

Pathophysiology

  • Key words: Systemic lupus erythematosus, bullous, immunosuppressant, dapsone Advertisement Journal of Molecular Pathophysiology SUBMIT YOUR ARTICLE NOW[scopemed.org]
  • Abstract Vesiculobullous eruptions in the elderly represent a diverse range of varying pathophysiologies and can present a significant clinical dilemma to the diagnostician.[karger.com]
  • Pathophysiology of SLE is not completely understood; however, the following have been implicated Apoptosis (programmed cell death) leading to loss of immune tolerance Interferon-alpha (IFN-α) and plasmacytoid dendritic cells (PDCs) Toll-like receptors[arupconsult.com]
  • ., Ph.D. [3], Raviteja Guddeti, M.B.B.S. [4] Synonyms and Keywords: SLE, Lupus erythematosus, Systemic lupus, Lupus, Disseminated lupus erythematosus, Discoid lupus, Cutaneous lupus erythematosus Overview Historical perspective Classification Pathophysiology[wikidoc.org]
  • The underlying pathophysiology of BSLE and EBA relates to the structure of the DEJ.[mdedge.com]

Prevention

  • The goals of the treatment plan are to Prevent flares Treat flares when they occur Reduce organ damage and other problems Treatments may include drugs to Reduce swelling and pain Prevent or reduce flares Help the immune system Reduce or prevent damage[icdlist.com]
  • [ edit ] There are several preventive foot hygiene measures that can prevent athlete's foot and reduce recurrence.[en.wikipedia.org]
  • Is it possible to prevent bullous pemphigoid? No. There is no prevention for bullous pemphigoid. From Bullous Pemphigoid Center References REFERENCE: Leiferman, Kristin M., et al.[medicinenet.com]
  • Potentially pregnant women must be enrolled in appropriate pregnancy prevention programs. Antimalarials may be associated with ocular toxicity. Regular (every 4-6 months) ophthalmologic evaluation is recommended.[dermquest.com]
  • Dapsone directly impairs the myeloperoxidase-hydrogen peroxide-halide system of polymorphonuclear neutrophils (PMNs), which prevents generation of proinflammatory oxygen intermediates caused by activation of neutrophils.[mdedge.com]

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