Three cases of bullous lupus erythematosus

• Published in: Annales de dermatologie et de vénéréologie Vol. 140; no. 12; pp. 778 - 783
• Main Authors: Jira, M, Elqatni, M, Sekkach, Y, Elomri, N, Mekouar, F, Ghafir, D
• Format: Journal Article
• Language: French
• Published: France 01-12-2013
• Subjects: Adult; Arthralgia - etiology; Autoantibodies - analysis; Blister - diagnosis; Blister - drug therapy; Blister - etiology; Blister - immunology; Blister - pathology; Collagen - immunology; Diagnosis, Differential; Disease Progression; Drug Eruptions - diagnosis; Female; Humans; Immunosuppressive Agents - therapeutic use; Lupus Erythematosus, Cutaneous - diagnosis; Lupus Erythematosus, Cutaneous - drug therapy; Lupus Erythematosus, Cutaneous - immunology; Lupus Erythematosus, Systemic - complications; Lupus Erythematosus, Systemic - diagnosis; Lupus Erythematosus, Systemic - drug therapy; Lupus Erythematosus, Systemic - immunology; Lupus Nephritis - etiology; Male; Neutrophil Infiltration; Oral Ulcer - etiology; Prednisone - therapeutic use; Skin - immunology; Skin - pathology; Young Adult; Sub-epidermal blistering; Lupus érythémateux bulleux; Anti-collagen VII antibody; Anticorps anti-collagène VII; Bullous lupus erythematosus; Bulle sous-épidermique
• ISSN: 0151-9638
• DOI: 10.1016/j.annder.2013.04.087

Bullous lupus is a subepidermal autoimmune bullous dermatosis, a rare entity that forms one of the cutaneous signs of systemic lupus erythematosus. We report on the clinical, immunopathological and progressive features of bullous lupus in three patients.
Our patients consisted of two women and one man aged 34, 22 and 30 years respectively. A diagnosis of bullous lupus erythematosus was evoked by blisters or vesicular blisters and confirmed, in addition to criteria for the diagnosis of systemic lupus erythematosus, by the presence of subepidermal blistering with infiltrate containing neutrophils and eosinophils as revealed by histological analysis, and of deposits of IgG and IgM (two cases) or of IgA (one case) at the dermo-epidermal junction observed under direct immunofluorescence. Indirect immunofluorescence showed anti-collagen VII antibodies. Lupus nephritis was present in two cases. Our patients were treated with corticosteroids and immunosuppressants.
Bullous lupus erythematosus may be the first sign of systemic lupus erythematosus with severe visceral involvement, especially renal involvement, suggesting that it may be a marker of activity and prognosis.