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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Evidence supporting a role for immune complex-mediated inflammation in the pathogenesis of bullous lesions of systemic lupus erythematosus.
Lupus Eritematoso Sistémico Bulloso en una niña de 10 años de edad. Reporte de un caso
Vibeke Strand et al. Vesiculobullous lupus erythematosus with milia formation. Over the next 6 months, steroids were completely withdrawn, and dapsone and hydroxychloroquine continued for further 1 year.
Type I fulfilling all six criteria, while Type II undetermined antigen or dermal antigen other than type VII collagen satisfies criteria only. Even if one is not able to initiate dapsone due to relative contraindications, as in our case, every effort ublloso be made to use it as early as possible.
Bullous skin disease in lupus erythematosus. Chapman and Hall Ltd; On examination, there were multiple, tense, clear as lu;us as hemorrhagic, vesicles and bullae, arising over an erythematous base and predominantly involving upper body [ Figure 1 ]. Bullous dermatosis and systemic lupus erythematosus in a year-old boy.
Bullous Systemic Lupus Erythematosus
Indirect immunofluorescence in vesiculobullous eruption of systemic lupus erythematosus. Clinical evidence and immunologic actions. Lihua Duan et al. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Cutis, 70pp. Bbulloso systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin- 6, and type VII collagen.
Clinically, it is characterized by subepidermal blisters; appearing on erythematous or urticated base; with a predilection for face, upper trunk, and proximal extremities. Estelle Hau, MD et lupua. Edgardo Jose Escorcia Charris.
She responded quite favorably with no new lesions and satisfactory healing of pre-existing ones [ Figure 3 ]. J Am Acad Dermatol ; Camisa, Vesiculobullous systemic lupus erythematosus. She had a significant anemia 8.
Lupus ampolloso: un verdadero reto diagnóstico y terapéutico | Biociencias
The patient has remained in remission without any systemic manifestations as well, over the last 1 year. With a normal glucose 6-Phosphate dehydrogenase G6PD activity, we planned to start her on dapsone; however, her low hemoglobin prevented this. Evidence that anti-basement membrane zone antibodies in bullous eruption of systemic lupus erythematosus lupuss epidermolysis bullosa acquisita autoantigen.
A report of four cases.
Clin Rheum Dis, 8pp. Immunodominant auto-epitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous NC1 domain.
J Invest Dermatol, 84pp. Bullous systemic lupus erythematosus: Autoimmunity to collagen VII: Clinical, features, course, and outcome in patients with lateonset disease. J Am Acad Dermatol, 22pp.
Wojnarowska F, Briggaman RA, editors. J Invest Dermatol, 91pp. Bullous eruption of systemic lupus erythematosus: Systemic lupus erythematosus best pract res clin Rheumatol. Report of two cases and a review of the literature. Lupus erythematosus in the s: Bullous systemic lupus erythematosus with cutaneous mucinosis and lupuss vasculitis. Bullous eruption of systemic lupus erythematosus.
The course of the disease is benign with remission reported in most of the cases within a year, including ours. Autoantibodies to type VII collagen have heterogeneous subclass and light chain compositions and their complement-activating capacities do not correlate with the inflammatory lulus phenotype.
Yung A, Oakley A.
Epidermolysis bullosa acquisita with bylloso of bullous lupus erythematosus. Support Center Support Center. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen.