BULLOUS SLE IS A RARE CUTANEOUS MANIFESTATION OF SYSTEMIC LUPUS

BULLOUS SLE IS A RARE CUTANEOUS MANIFESTATION OF SYSTEMIC LUPUS

BONFIM, LARA RIBEIRO TEIXEIRA; BELLINI, PAMELA PAULA; OGAWA, GABRIELLA MIYUKI; ANDRADE, SAMUEL OLIVEIRA; PUGLIESI, ALISSON ALIEL VIGANO; FRANÇA, ANDREA FERNANDES ELOY DE COSTA; APPENZELLER, SIMONE; COSTALLAT, LILIAN TERESA LAVRAS

Pôster:

Bullous systemic lupus erythematosus (LESB) is a rare subgroup of lupus and accounts for less than 5% of cutaneous manifestations. LESB is associated with autoimmunity to type VII collagen. The lesions predominate in black women aged 20-30 years. LESB is a generalized vesiculobullous rash that occurs in patients with SLE and makes a differential diagnosis with other bullous dermatoses such as: bullous pemphigoid, epidermolysis bullosa acquired, herpetic dermatitis and pharmacoderma. The lesions may appear anywhere, although there is predilection for the upper trunk, neck, supraclavicular region, as well as axillary folds and oral and vulvar mucosa. Diagnosis is necessary: 1) a diagnosis of SLE, 2) a systemic vesiculobullous eruption 3) histopathology with subepidermal blisters and predominant neutrophilic infiltrate and leukocytoclastic vasculitis, (4) negative or positive direct immunofluorescence for autoantibodies IGG, IGA, IGM and complement in the basement membrane (5) positive indirect immunofluorescence in the basal membrane.There are two types of LESB, depending on the presence of antibodies to type VII collagen that may be Type 1 with presence and type 2 with absence. Treatment is done with dapsone ,azathioprine or colchicine and the response tends to be rapid.

Case report

Patient woman 26y, with diagnosis of SLE (arthritis, serositis, photosensitivity, lymphopenia and fan +) for 7 months, using prednisone 60 mg. Three months ago, he had an episode of infection of upper areas that was treated with benzathine penicillin. After some bullous lesions appeared in the trunk, which regressed exponentially. She was instructed to discontinue antibiotics and prednisone for suspected pharmacodermia. The lesions progressed despite the suspension of the medications and after 2 months they became disseminated in the trunk, back and underarms, and oral mucosa. In the investigation of lesions, biopsy and direct immunofluorescence (DIF) showed: subepidermal bubbles, neutrophilic inflammatory infiltrate with eosinophils, IgG, IgM, IgA, C1q, +3 linear positive along the basement membrane of the epidermis, compatible with SLE bullous. First, we started intravenous corticoid, without improvement. After introduction of dapsone 100mg/day, there was a significant improvement of the lesions, but has presenteed allergic reaction to medication. Colchicine was started (1,5 mg/day) with complete improvement of skins lesions in a few weeks.

Conclusion

The diagnosis of bullous lupus is difficult to make since several pathologies with blisters have been reported in association with LE. These conditions may be differentiated from bullous lupus through a complete history, clinical presentation, characteristic histopathological findings and immunofluorescence.

Bullous systemic lupus erythematosus (LESB) is a rare subgroup of lupus and accounts for less than 5% of cutaneous manifestations. LESB is associated with autoimmunity to type VII collagen. The lesions predominate in black women aged 20-30 years. LESB is a generalized vesiculobullous rash that occurs in patients with SLE and makes a differential diagnosis with other bullous dermatoses such as: bullous pemphigoid, epidermolysis bullosa acquired, herpetic dermatitis and pharmacoderma. The lesions may appear anywhere, although there is predilection for the upper trunk, neck, supraclavicular region, as well as axillary folds and oral and vulvar mucosa. Diagnosis is necessary: 1) a diagnosis of SLE, 2) a systemic vesiculobullous eruption 3) histopathology with subepidermal blisters and predominant neutrophilic infiltrate and leukocytoclastic vasculitis, (4) negative or positive direct immunofluorescence for autoantibodies IGG, IGA, IGM and complement in the basement membrane (5) positive indirect immunofluorescence in the basal membrane.There are two types of LESB, depending on the presence of antibodies to type VII collagen that may be Type 1 with presence and type 2 with absence. Treatment is done with dapsone ,azathioprine or colchicine and the response tends to be rapid.

Case report

Patient woman 26y, with diagnosis of SLE (arthritis, serositis, photosensitivity, lymphopenia and fan +) for 7 months, using prednisone 60 mg. Three months ago, he had an episode of infection of upper areas that was treated with benzathine penicillin. After some bullous lesions appeared in the trunk, which regressed exponentially. She was instructed to discontinue antibiotics and prednisone for suspected pharmacodermia. The lesions progressed despite the suspension of the medications and after 2 months they became disseminated in the trunk, back and underarms, and oral mucosa. In the investigation of lesions, biopsy and direct immunofluorescence (DIF) showed: subepidermal bubbles, neutrophilic inflammatory infiltrate with eosinophils, IgG, IgM, IgA, C1q, +3 linear positive along the basement membrane of the epidermis, compatible with SLE bullous. First, we started intravenous corticoid, without improvement. After introduction of dapsone 100mg/day, there was a significant improvement of the lesions, but has presenteed allergic reaction to medication. Colchicine was started (1,5 mg/day) with complete improvement of skins lesions in a few weeks.

Conclusion

The diagnosis of bullous lupus is difficult to make since several pathologies with blisters have been reported in association with LE. These conditions may be differentiated from bullous lupus through a complete history, clinical presentation, characteristic histopathological findings and immunofluorescence.

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DOI: 10.5151/sbr2019-042

Referências bibliográficas

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Como citar:

BONFIM, LARA RIBEIRO TEIXEIRA; BELLINI, PAMELA PAULA; OGAWA, GABRIELLA MIYUKI; ANDRADE, SAMUEL OLIVEIRA; PUGLIESI, ALISSON ALIEL VIGANO; FRANÇA, ANDREA FERNANDES ELOY DE COSTA; APPENZELLER, SIMONE; COSTALLAT, LILIAN TERESA LAVRAS; "BULLOUS SLE IS A RARE CUTANEOUS MANIFESTATION OF SYSTEMIC LUPUS", p-42-42. In: Anais do 36º Congresso Brasileiro de Reumatologia. [ISBN 978-85-212-1892-0]. São Paulo: Blucher, 2019.
ISSN 23577282, DOI 10.5151/sbr2019-042