Case 1: A 19-year-old patient with bullous systemic lupus erythematosus

A 19-year-old patient presented with single, disseminated, erythematous papules and sharply demarcated, partly urticarial plaques on his face. Over the past few weeks, the skin manifestations had spread to the décolleté and blisters had developed on his forearms after sun exposure. A skin biopsy of one of the blisters showed a subepidermal vesicle containing neutrophils and scattered eosinophils. A perivascular and interstitial infiltrate of lymphocytes and neutrophils was seen in the upper and mid dermis, as well as formation of neutrophil microabscesses. In the direct immunofluorescence test (lupus band test), linear immunofluorescence was shown along the basement membrane zone with anti-IgA and anti-IgG antibodies. In addition, the patient showed a moderately diffuse alopecia, Raynaud’s phenomenon, polyarthritis, synovitis, tendovaginitis splenomegaly, lymphadenopathy, fatigue, and night sweats. Laboratory analysis demonstrated anemia, leukopenia, hypocomplementemia, and autoantibodies (ANA and anti-dsDNA antibodies).

Bullous systemic lupus erythematosus (BSLE) is a rare disease associated with subepidermal blistering and, in most cases, severe systemic manifestations. The vesiculobullous skin changes can occur after sun exposure and can be associated with activation of SLE. Dapsone is the mainstay of systemic treatment in this disease and systemic corticosteroids and antimalarials have shown minimal improvement.^1–3

Discussion Points: Different forms of skin lesions and treatment options in patients with cutaneous lupus erythematosus.

References

1. Bacman D, et al. Bullöser systemischer lupus erythematodes [Bullous systemic lupus erythematosus]. Hautarzt 2004 Apr;55(4):392–395.

2. Chanprapaph K, et al. A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients. Lupus 2017 Oct;26(12):1278–1284.

3. Worm M, et al. S2k guideline: Diagnosis and management of cutaneous lupus erythematosus - Part 1: Classification, diagnosis, prevention, activity scores. J Dtsch Dermatol Ges 2021 Aug;19(8):1236–1247.

Learning Objectives

• Recognise the specific (i.e. ACLE, SCLE, CCLE, ICLE) and non-specific skin manifestations in cutaneous lupus

• Explain the RCLASI as validated activity and damage score of cutaneous lupus

• Discuss the therapeutic guidelines of cutaneous lupus

• Describe the preventive strategies in cutaneous lupus, including photoprotection

• Discuss the topical and systemic treatment options in cutaneous lupus