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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William Stohl et al.
A provisional lupjs of BSLE was considered and she was thoroughly investigated. Report of two cases and a review of the literature. She was also found to have photosensitivity, significant anemia, and high antibody titers confirming SLE.
J Am Acad Dermatol, 24pp. J Am Acad Dermatol ; Footnotes Source of Support: You can change the settings or obtain more information by clicking here. This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE.
La respuesta al tratamiento con dapsona fue notable a las 48 horas. A report of four cases.
Our patient showed only modest improvement on steroids and antimalarials, complete response being seen only with dapsone. Understanding the epidemiology and progression bullowo systemic lupus erythematosus.
Yung A, Oakley A. Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: Int J Dermatol, 33 bullloso, pp. BMJ – Rheumatic and musculoskeletal diseases. Presence of tense vesicles marked with an arrow filled with cleat fluid along with superficial erosions with polycyclic margins. National Center for Biotechnology InformationU.
Yell JA, Wojnarowska F. Other biochemical investigations were essentially normal, with no evidence of renal derangement. Camisa, Vesiculobullous systemic lupus erythematosus. Also, the dramatic response to dapsone is highlighted which remains the drug of choice in the management of this disorder.
Autoantibodies to type VII collagen have heterogeneous subclass and light chain compositions and their complement-activating capacities do not correlate with the inflammatory clinical phenotype.
J Am Acad Dermatol, 38pp. Inset shows a positive lupus band IgG at the basement membrane zone. Lupus, 6pp. Routine histopathology and immunoflorescence studies suggested BSLE and she responded favorably to dapsone. Vibeke Strand et al. Bullous Systemic Lupus Erythematosus: Marked improvement seen 1 week after starting dapsone therapy.
Bullous Systemic Lupus Erythematosus
Even if one is not able to initiate dapsone due to relative contraindications, as in our case, every effort should be made to use it as early as possible. Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year. Male systemic lupus erythematosus in a latin-American inception cohort of patients.
Clin Rheum Dis, 8pp. J Am Acad Dermatol, 18pp.
Lupus Eritematoso Sistémico Bulloso en una niña de 10 años de edad. Reporte de un caso
Bullous systemic lupus erythematosus. The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysis bullosa acquisita autoantibodies. There was only partial improvement with new lesions developing even after 4 weeks; wherein, she was started on dapsone. NC1 domain of type VII collagen binds to the beta3 chain of laminin 5 via lpuus unique subdomain within the fibronectin-like repeats. J Am Acad Dermatol, 22pp.
The patient has remained in remission without any systemic manifestations as well, buloso the last 1 year.