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2.1
Bullous Systemic Lupus Erythematosus
BSLE

Presentation

The patient later presented with pulmonary hemorrhage and death. [ped-rheum.biomedcentral.com]

Lesions are present on covered as well as exposed areas A provisional diagnosis of BSLE was considered and she was thoroughly investigated. [ncbi.nlm.nih.gov]

Bullous lupus: An unusual initial presentation of systemic lupus erythematosus in an adolescent girl. [jhu.pure.elsevier.com]

No history of seizure, psychosis or history of trauma at the site of blisters was present. [ijpd.in]

Entire Body System

  • 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. [en.wikipedia.org]

Musculoskeletal

  • 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 also complained of diffuse hair loss and arthralgias. Her past medical history was unremarkable but her mother had systemic lupus erythematosus. [ispub.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]

Psychiatrical

  • 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]

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]

Urine

  • 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]

Serum

  • 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]

Other Pathologies

  • 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

Therefore, a comprehensive review of the treatment of BSLE would be beneficial to cure the disease. 1. [hindawi.com]

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]

Treatment of Bullous Systemic Lupus Erythematosus. J Immunol Res 2015; 2015: 167064–167064. [journals.sagepub.com]

treatment is associated with adverse metabolic effects and bone demineralization. [mdedge.com]

Prognosis

The prognosis in patients with SLE and bullous lesions is determined largely by the visceral manifestations of the SLE,5 yet the activity of the systemic and skin disease may not be linked.3,5 Our patient presented with immunobullous disease and serologic [mdedge.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]

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]

Prognosis Survival: [4] ~90% @ 10 years. Mortality (leading causes): [4] Active SLE - esp. kidney involvement. Infections. Thrombosis. Microscopic Lymph node pathology Skin See: Lupus erythematosus. [librepathology.org]

Etiology

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]

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]

It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. [fpnotebook.com]

(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]

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]

Pathophysiology

Key words: Systemic lupus erythematosus, bullous, immunosuppressant, dapsone Advertisement Journal of Molecular Pathophysiology SUBMIT YOUR ARTICLE NOW [scopemed.org]

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]

Zorkun, M.D., 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]

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]

Prevention

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]

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]

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