Dermatol Clin. Jul;11(3) Epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. Diseases of autoimmunity to type VII collagen. Bullous Systemic Lupus Erythematosus (BSLE) is a rare manifestation of . (BP), inflammatory variant of epidermolysis bullosa acquisita (EBA), and linear IgA. However, unlike epidermolysis bullosa acquisita, bullous systemic lupus erythematosus tends to respond dramatically to treatment with.
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Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Diagnostic immunohistochemistry of the skin, pp.
Biociencias13 1. Efficacy and safety of belimumab lupue patients with active systemic lupus erythematosus: Improvements in health-related quality of life with belimumab, a B-lymphocyte stimulator-specific inhibitor, in patients with autoantibody-positive systemic lupus erythematosus from the randomized controlled BLISS trials. Bullous systemic lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis.
Bullous eruption of systemic lupus erythematosus Dramatic response to dapsone therapy. J Biol Chem,pp.
Our patient showed only modest improvement on steroids and antimalarials, complete response being seen only with dapsone. You can change the settings or obtain more information by clicking here. This article has been cited by other articles in PMC.
There was only partial improvement with new lesions developing even after 4 weeks; wherein, she was started on dapsone. Superficial erosions luups also noted over the buccal mucosa, hard palate, and lips.
Direct immunofluorescence both perilesional and distant-covered skin revealed IgM and C3 deposition along the basement membrane zone BMZ as well as perivascular location. J Am Acad Dermatol. Clin Dermatol, 22pp. BMJ – Rheumatic and musculoskeletal diseases.
Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Management of Blistering Diseases.
Other biochemical investigations were essentially normal, with no evidence of renal derangement. Nil Lupud of Interest: Nephrol Dial Transplant, 14pp.
A diagnosis of bullous SLE was made. The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysis bullosa acquisita autoantibodies.
Lupus Eritematoso Sistémico Bulloso en una niña de 10 años de edad. Reporte de un caso
J Am Acad Dermatol. J Dermatol, 21pp. Subepidermal blister formation in the course of severe systemic lupus erythematosus SLE can occur due to extensive interface inflammation and basal cell vacuolation, presenting as polycyclic erosions with advancing blistering border predominantly on sun-exposed areas. Int J Dermatol, 33pp. Bullous skin lesions in systemic lupus erythematosus.
NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via a unique subdomain within the fibronectin-like repeats. Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year. With a normal glucose 6-Phosphate dehydrogenase G6PD activity, we bullso to start her on dapsone; however, her low hemoglobin prevented this. J Invest Dermatol, 81pp. Male systemic lupus erythematosus in a latin-American inception cohort of patients.
Our understanding of the pathology of this interesting and unique dermatologic condition has evolved with improved diagnostic criteria and clinicohistologic profiling. J Invest Dermatol ; X-rays of chest and involved joints were normal. Whether or not BSLE is associated with systemic activity is a bullozo debated topic.
Immunodominant auto-epitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous NC1 domain. La respuesta al tratamiento con dapsona fue notable a las 48 horas. Often associated is a heterogeneous profile of autoimmunity to components of type VII collagen. Autoimmunity to collagen VII: J Am Acad Dermatol, 7pp.
Bullous Systemic Lupus Erythematosus
Evidence supporting a role for immune lups inflammation in the pathogenesis of bullous lesions of systemic lupus erythematosus.
A year-old lady, a homemaker and mother of three, presented with largely asymptomatic vesicobullous lesions 2 months durationinvolving head and neck, upper limbs, and trunk over both photo-exposed and unexposed areas. Vibeke Strand et al. Bullous systemic lupus erythematosus. Epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus.
The disease is very responsive to dapsone therapy. Systemic lupus erythematosus presenting as a bullous eruption in a child. Introduction What was known?
J Am Acad Dermatol, 38pp. Autoimmunity to collagen VII: Lupus, 12pp. Clin Exp Dermatol,pp.