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In The Name Of GOD
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Bullous systemic lupus erythematosus
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Vesicles & bullae in patients with SLE
Classic lesions of discoid , systemic, or subcutaneous lupus erythematosus (rarely) Previous history of SLE Befor making the diagnosis in patients with bullous lesions
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Lesions characteristics:
Not symmetrical Not pruritic Not predilection for extensor surfaces of arms,elbows, or scalp Photodistributed or widespread Dramatic response to dapsone therapy
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Tense vesiculobullous lesions on the neck
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Bullous eruption of systemic lupus erythematosus
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Histopathology 3 histologic patterns:
1- Basal layer vacuolization + subsequent blister formation 2-Vasculitis + subepidermal blister & pustule formation 3-Dermatitis herpetiformis-like histologic pattern (most common)
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Vasculitis - Small vessel ,neutrophil –rich leukocytoclasstic vasculitis - Beneath the blister - 25% of cases
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Histopathology Supepidermal bulla with interface changes in intact areas Neutrophils along DEJ Perivascular and periadnexal infiltrate with increased mucin
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Bullous Systemic Lupus Erythematosus: Subepidermal blister ,neutrophils are predominant in the blister & superficial dermis
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Systemic lupus erythematosus (Bullous)
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IF Testing IgG & C3 deposition Epidermal basement membrane zone
(in all cases)
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IF Testing Linear pattern > 50% of cases
Granular bandlike : 25% of cases IgM deposition : 50% of cases IgA deposition : 60% of cases
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Granular patterns In general,deposition of
circulating immune complexes in situ or in situ binding of Ag and Ab
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Direct immunofluorescent studies will show homogeneous linear staining along the dermal-epidermal junction. Salt split skin studies show localization to the floor
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C3 linear basement membrane zone (40x)
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IIF study IIF study of serum
Circulating anti-squamous basement membrane zone antibodies (rarely) against type VII collagen
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IIF study Salt-split skin preparation more sensitive substrate
localization to the split floor (as EBA)
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Ultrastructural Study
Immunelectron microscopic examination: Electron-dense deposits of IgG lower edge of basal lamina & immediately adjacent dermis
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Differential Diagnoses
Bullous Pemphigoid Dermatitis Herpetiformis Epidermolysis Bullosa Acquisita (EBA) Drug-Induced Bullous Disorders Linear IgA Dermatosis Dermatologic Manifestations of Graft Versus Host Disease Epidermolysis Bullosa Erythema Multiforme Hydroa Vacciniforme Porphyria Cutanea Tarda Pseudoporphyria
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Differential Diagnoses
Dermatitis Herpetiformis - Subepidermal blister containing predominantly neutrophils with rare eosinophils Neutrophilic microabscesses in dermal papillae DIF : Granular IgA deposition in dermal papillae, with/without linear deposits of IgA at BMZ.
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Differential Diagnoses
Bullous Pemphigoid Subepidermal bulla containing eosinophils with admixed lymphocytes and neutrophils Superficial perivascular and interstitial lymphocytic infiltrate with eosinophils DIF : IgG and C3 at DEJ.
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Differential Diagnoses
Linear IgA Bullous Dermatosis -Supepidermal blister formation with papillary dermal edema -Linearly aligned neutrophils along DEJ -Superficial perivascular and interstitial neutrophilic infiltrate with admixed eosinophils and lymphocytes may be seen -DIF : Linear IgA at DEJ
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Differential Diagnoses
Bullous Drug Eruption Subepidermal blister with associated perivascular and interstitial mixed infiltrate Admixed eosinophils DIF : Negative
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Differential Diagnoses
Epidermolysis Bullosa Acquisita Supepidermal blister with minimal inflammation, or mimicking pemphigoid DIF : Linear IgG and C3 at DEJ Distinguishing from EBA with neutrophil-rich inflammatory cell infiltrate may be achieved by lupus serologies, and relatively quick response to dapsone or azathioprine in bullous SLE (EBA is usually resistant to therapy)
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