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Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous . Lúpus eritematoso sistêmico bolhoso – diagnóstico diferencial com dermatite herpetiforme. O lúpus eritematoso sistêmico pode apresentar inúmeras lesões cutâneas. As lesões bolhosas específi cas do lúpus, apesar de raras, apresentam. Systemic lupus erythematosus (SLE) can cause numerous skin lesions. Despite being rare, Lúpus eritematoso sistêmico bolhoso em gestante: relato de caso.

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Bullous systemic lupus erythematosus BSLE is a rare autoimmune subepidermal blistering disease, with few cases described in childhood. It has bolhosl clinical-pathological features. We report a case of BSLE in a year-old child with systemic lupus erythematosus, treated with prednisone and hydroxychloroquine. There was complete remission with dapsone, with no recurrence of skin lesions throughout one year of follow-up.

We highlight the rarity and early age of occurrence. Bullous systemic lupus erythematosus BSLE is a rare cutaneous manifestation of systemic lupus erythematosus SLEand even more uncommon in the pediatric population 12 It is a heterogenous disease, caused by autoantibodies against the dermo-epidermal junction, particularly type VII collagen.

We highlight the rarity, due to the fact that it developed during childhood, in a patient under standard SLE treatment. She also had occasional fever and hepatomegaly.


Laboratory tests revealed mild anemia Hb.: Echocardiogram showed a minimal increase in pericardial fluid and chest radiography showed a mild, bilateral pleural effusion. Histology from skin biopsy revealed subepidermal blister filled with fibrin, lymphomononuclear cells and neutrophils, thickening of the basement membrane in ulpus dermal papillae and mild perivascular lymphoplasmacytic infiltrate in the papillary, superficial dermis Figure 4.

S alt-split was positive for IgG in the dermis and epidermis and for IgA in the epidermis. Subepidermal blister filled with fibrin, lymphomononuclear and neutrophils. Clinical and laboratory abnormalities in this patient confirmed the suspicion of BSLE. Bolhozo patient remained stable during 1 year of follow-up.

Clinically, BSLE is characterized by a generalized vesiculobullous eruption, non-cicatricial, restricted to photo-exposed areas, or not. On histology, a subepidermal blister with neutrophilic inflammatory infiltrate is seen. In our case, the patient being treated for SLE presented with mostly tense bullae, mainly on the face, mucosa and flexures.

The activity of the bullous disease can occur isolated from or concomitantly with other systemic manifestations of SLE, nephritis and serositis in particular. The main differential diagnoses of BSLE are acquired epidermolysis bullosa, dermatitis herpetiformis, bullous pemphigoid and linear IgA bullous dermatosis. The treatment of choice is dapsone; the therapeutic response is usually satisfactory.

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Received May 22; Accepted Sep This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited.

Abstract Bullous systemic lupus erythematosus BSLE is a rare autoimmune subepidermal blistering disease, with few cases described in childhood.

Lúpus eritematoso sistêmico bolhoso: diagnóstico diferencial com dermatite herpetiforme

Autoimmunity, Skin diseases, vesiculobullous, Lupus erythematosus, systemic. Open in a separate window. Direct immunofluorescence with linear deposits lipus C3 in the BMZ. Direct immunofluorescence with fluorescence of the keratinocyte nuclei. Tense bullae overlying an erythematous base on the right axilla. Footnotes Conflict of interest: Childhood-onset bullous systemic lupus erythematosus.

Autoimmune Diseases

Bullous systemic lupus erythematosus: Am J Clin Dermatol. Bullous Systemic Lupus Erythematosus. A phenotypically distinctive but immunologically heterogeneous bullous disorder.

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