Lúpus eritematoso sistêmico bolhoso: diagnóstico diferencial com dermatite herpetiforme

Abstract

O lúpus eritematoso sistêmico bolhoso é um subtipo raro do lúpus eritematoso sistêmico, que ocorre ainda de forma mais incomum nos pacientes pediátricos. Relatamos o caso de uma adolescente de 12 anos, apresentando lesões vésico-bolhosas em face, pescoço, tronco, mucosas oral e genital, anemia, leucocitúria estéril, FAN: 1/1280 padrão nuclear pontilhado grosso, Anti-Sm e Anti-RNP positivos. O estudo anatomopatológico sugere dermatite herpetiforme e a imunofluorescência direta revela IgG, IgA e fibrina ao longo da zona de membrana basal. Apresentamos um caso típico de lúpus eritematoso sistêmico bolhoso e enfatizamos a importância do diagnóstico diferencial com a dermatite herpetiforme<br>Bullous systemic lupus erythematosus is a rare subset of systemic lupus erythematosus that is even rarer in pediatric patients. 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. Pathological examination suggested dermatitis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemic lupus erythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformi

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

“…Usually, papillary neutrophilic microabscesses progress to subepidermal vacuolization and vesicle formation. In DIF microscopy granular IgA deposits in dermal papillae of perilesional skin is found [48]. The clinical and histopathological features of DH can be the same as those of BSLE.…”

“…There are many case reports showing that BSLE can be the initial presentation of SLE in both adults and children [11,12,21,[48][49][50][51]. Moreover, some authors estimated that BSLE may be associated with increased activity of SLE such as renal involvement, serositis such as pericarditis or pleuritis, pulmonary hemorrhage, cutaneous vasculitis, etc.…”

Bullous systemic lupus erythematosus in a 10-year-old child

“…BSLE is a rare bullous variant, 1 , 3 - 6 affecting less than 5% of SLE patients. 6 It is even more uncommon in the pediatric population and presents rarely as the initial manifestation of lupus erythematosus.…”

“…Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of systemic lupus erythematosus (SLE), and even more uncommon in the pediatric population 1 , 2 It is a heterogenous disease, caused by autoantibodies against the dermo-epidermal junction, particularly type VII collagen. 3 Occasionally, other autoantigens, such as laminin 5, laminin 6 and BP230 are described.…”

Efeitos do uso da colchicina em melhora de alterações miocárdicas correlacionadas com prevenção e complicação do infarto agudo do miocárdio: revisão sistemática / Effects of the colchicine to improve myocardial changes correlated with prevention and complication of acute myocardial infarction: systematic review

“…Esse fuso é essencial para organização das cadeias de cromossomos no equador celular antes de finalizar a divisão deste. Quando essa ação é feita em células granulocíticas, diminui a atividade sobre a região inflamada por reduzir a quimiotaxia e a liberação de enzimas lisossomais (8) .…”