Background: Bullous systemic lupus erythematosus (BSLE) is a rare subtype of systemic lupus erythematosus, accounting for less than 1% of systemic lupus erythematosus (SLE) [1]. It is common in adults aged 20 to 40, with female predominace [2]. Its skin manifestations are mainly tensive blisters, especially involving oral and genital mucosa, Nissl’s sign is mostly negative, blisters break and heal to form pigmentation of varying degrees, leaving no scar. At present, the main treatment is systemic application of corticosteroids combined with immunosuppressants. Here, we report a BSLE who successfully treated with mycophenolate mofetil (MMF) combined with glucocorticoid.

Objectives: To be vigilant in the early identification of BSLE, and through the treatment experience of this case, it can provide a basis for the use of Mycophanol ester as the preferred drug for the treatment of BSLE in the future.

Methods: The clinical manifestation, laboratory test, treatment, and outcome were described.

Results: A 50-year-old female patient with systemic lupus erythematosus presented with wandering joint pain for 1 year and sporadic patchy erythema on eyelid, hands, and neck for 7 months. There are blisters on part of her erythema and normal skin, about 1mm-15mm in diameter, the blister fluid is cool, fusion is broken, and Nissl’s sign is negative. She suffered from anemia, albuminuria, polyserous effusion and other system damage. Skin histopathology showed mild hyperkeratosis of epidermis, formation of subepidermal blisters and accumulation of a large number of neutrophils; direct immunofluorescence showed that the positive expressions of IgA, IgG and C3 were found in the basement membrane of the skin, leading to a diagnosis of BSLE. In the initial stage, patients were treated with high-dose glucocorticoid (160 mg, qd) combined with immunosuppressants (thalidomide and hydroxychloroquine), but the number of blisters increased. After treatment with MMF (1 g, bid), the skin lesions disappeared, joint pain relieved, anemia was corrected, and polyserous effusion was absorbed. She was followed up for half a year and there was no recurrence.

Conclusion: Patients with BSLE are often accompanied by multiple system damage [2], so it is critical to correctly identify BSLE and provide appropriate treatment as soon as possible. For patients who do not respond to single glucocorticoid therapy, combined with MMF can be used as the preferred drug for the treatment of BSLE in the future.

REFERENCES:

[1] L. Duan et al., Treatment of Bullous Systemic Lupus Erythematosus. Journal of immunology research 2015, 167064 (2015).

[2] K. Chanprapaph, S. Sawatwarakul, V. Vachiramon, A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients. Lupus 26, 1278-1284 (2017).

Figure 1.

BSLE. Blisters can be seen on the face, neck, armpits and arms.

BSLE = bullous systemic lupus erythematous.

Disclosure of Interests: None declared