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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A year-old lady, a homemaker and bulloo 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.
Lesions are present on covered as well as exposed areas. J Clin Immunol ; Inset shows a positive lupus band IgG at the basement membrane zone. Most reported cases have dramatically responded to dapsone, with cessation of new lesions in days and healing within several days. Lupus, 12pp. Bullous dermatosis and systemic lupus erythematosus in a year-old boy. It needs to be differentiated from other subepidermal bullous diseases in view of potentially associated systemic manifestations of Lupys.
Open in a separate window. Australas J Dermatol, 41pp.
J Dermatol, 21pp. A report of four cases. As seen in our case, BSLE developing with or prior to features of SLE can present a diagnostic dilemma; luupus, other subepidermal vesicobullous disorders need to be ruled out. You can change the settings or obtain more information by clicking here.
Bullous Systemic Lupus Erythematosus
The disease is very responsive to dapsone therapy. Estelle Hau, MD et al. Bullous systemic lupus erythematosus: Bullous eruption of systemic lupus erythematosus Dramatic response to dapsone therapy.
Also, the dramatic response to dapsone is highlighted which remains the drug of choice in the management of this disorder.
Clinical, histopathological, and immunopathological overlap exists with BP as well. Bullous skin disease in lupus erythematosus. Author information Article notes Copyright and License information Disclaimer. Ann NY Acad Sci,pp. Other bullous disorders such as bullous pemphigoid, epirdermolysis bullosa acquisita, linear IgA bullous dermatosis and dermatitis herpetiformis has also been reported in association with SLE.
Dapsonetype VII collagenimmunobullous diseases. Systemic lupus erythematosus in a multiethnic US cohort. Wojnarowska F, Briggaman RA, editors.
Vesiculobullous lupus erythematosus with milia formation. Bullous disease in systemic lupus erythematosus. Arch Dermatol,pp. Lupux or not BSLE is associated with systemic activity is a hotly debated topic. Br J Dermatol,pp. A diagnosis of bullous SLE was made. This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.
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 bullloso.
Indirect immunofluorescence in vesiculobullous eruption of systemic lupus erythematosus. Autoantibody mediated pathomechanisms regulate clinical-pathological phenotypes of acquired epidermolysisbullosa and bullous SLE. Subepidermal blister formation in the course of severe systemic bullodo 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.
The carboxyl terminus of type VII collagen mediates antiparallel dimer kupus and constitutes a new antigenic epitope lupsu epidermolysis bullosa acquisita autoantibodies. J Am Acad Dermatol, 38pp.
Sem Cut Med Surg, 17pp. Bullous systemic lupus erythematosus SLE is a clinicopathologically distinctive immunobullous eruption occurring during the course of SLE. Male systemic lupus erythematosus in a latin-American inception cohort of patients. Hematinics were lupsu initiated. Journal of Immunology Research. We described a patient who developed severe bullous lesions 14 days after high-dose systemic glucocorticoids and cyclophosphide therapies were initiated for severe SLE manifestations nephritis and alveolar hemorrhage.
J Am Acad Dermatol. Due to resource constraints, indirect immunofluorescence or salt-split skin could not be performed. Camisa, Vesiculobullous systemic lupus erythematosus.
Vesiculobullous systemic luous erythematosus. Epidermolysis ulpus acquisita preceding the development of systemic lupus erythematosus. J Am Acad Dermatol, 15pp. Occasionally other antigens such as laminin 5, laminin 6, and BP bullous pemphigoid antigen have also been described.
Lupus ampolloso: un verdadero reto diagnóstico y terapéutico | Biociencias
J Am Acad Dermatol, 21pp. Dapsone should be considered the drug of choice for bullous systemic lupus erythematosus as treatment response to other options is suboptimal. There was only partial improvement with new lesions developing even after 4 weeks; wherein, she was started on dapsone. Diagnostic methods in autoimmune ljpus bullous diseases.