Bullous systemic lupus erythematosus a case report

Abstract

Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (30–40 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLE—a rare presentation of SLE—which may evolve with marked clinical presentation.

Vesiculobollous disease with Cutaneous Systemic Lupus Erythematosus Treated by Rituxima; a case report

“…Mucosal involvement is common in the laryngeal, pharyngeal, perioral, and genital areas. The lesions usually develop with no scarring, but hyper or hypochromia can occur (7). Here we believed that this case report will receive the most attention from Sudanese clinicians and rekindle knowledge and awareness in regard to vesiculobollous disease in patient who is known to be suffering from SLE.…”

“…The incidence of this disease with SLE is about 1%. As the knowledge about it is mainly based on case series or reports (7,9). Patients with the vesiculobullous disease usually present with clinical features of SLE, the blisters mainly occur on the oral mucosa, neck, face and upper extremities (10).…”

Prevalência do lúpus eritematoso cutâneo em centro dermatológico terciário do Brasil

“…14 Ocorre preferencialmente no sexo feminino, sendo rara na faixa etária pediátrica, e possuindo caracteristicamente a presença de vesículas ou bolhas sob máculas ou pápulas eritematosas. 15 Em nosso estudo, verificamos prevalência de 1,88% e relação entre sexos de 5:1, entretanto a idade média de acometimento observada foi baixa, ainda que na faixa adulta, quando comparada com outros estudos.…”

“…18 Existem teorias que tentam relacionar fisiopatologicamente desordens autoimunes, como o vitiligo, com alguns tipos específicos de lúpus cutâneo, especialmente o bolhoso, mas a literatura ainda carece de dados confirmatórios. 15,19 Algumas doenças encontradas neste estudo, como acne, que surgiram associadas ao LEC, podem ser mais concernentes à faixa etária e a fatores de risco de surgimento de outras afecções cutâneas.…”

Bullous systemic lupus erythematosus: exceptional and challenging presentation

“…Among these lesions, BSLE is observed only in less than 1% of cases [5,6]. The first well-documented case of BSLE was published by Hall RP et al, in 1982 [7]; since the majority of cases are reported as sporadic observation or small series not exceeding ten cases [4][5][6][7][8][9][10]. Indeed, the latest systemic review of the world literature by de Risi T et al, in 2017 found only 118 published cases of BSLE [8].…”