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.
|Published (Last):||8 September 2014|
|PDF File Size:||14.42 Mb|
|ePub File Size:||8.13 Mb|
|Price:||Free* [*Free Regsitration Required]|
Inset shows a positive lupus band IgG at the basement membrane zone. Bullous systemic lupus erythematosus. Systemic lupus erythematosus presenting as a bullous eruption in a child. Wojnarowska F, Briggaman RA, editors. J Biol Chem,pp. J Cutan Pathol, 28pp. J Am Acad Dermatol, 38pp.
EBA is histopathologically and immunopathologically identical, as it is also mediated by antibodies to type VII collagen; however, a dramatic response to dapsone, as in our case, helps differentiate the two as EBA is notoriously resistant to treatment.
Clin Dermatol, 22pp. Int J Dermatol, 33pp.
Bullous Systemic Lupus Erythematosus
William Stohl et al. 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. On examination, there were multiple, tense, clear as well as hemorrhagic, vesicles and bullae, arising over an erythematous base and predominantly involving upper body [ Figure 1 ].
Though, it may be difficult to introduce the drug initially, as in our case; every attempt should be made to use it, as relatively low doses mg have also been shown to be efficacious. Ann Intern Med,pp. As seen in our case, BSLE developing with or prior to features of SLE can present a diagnostic dilemma; hence, other subepidermal vesicobullous disorders need to be ruled out.
Bullous systemic lupus erythematosus. Continuing navigation will be considered as acceptance of this use. The patient has remained in remission without any systemic manifestations as well, over the last 1 year. J Am Acad Dermatol, 7pp. Marked improvement seen 1 week after starting dapsone therapy.
Hematinics were also initiated. Spectrum of subepidermal immunobullous disorders seen at the National Skin Centre Singapore: Extensive serological workup revealed antinuclear antibody positivity of 1: The carboxyl terminus of type VII collagen mediates antiparallel dimer formation and constitutes a new antigenic epitope for epidermolysis bullosa acquisita autoantibodies.
Diagnostic methods in autoimmune subepidermal bullous diseases. Received Nov; Accepted Jan.
Indirect immunofluorescence in vesiculobullous eruption of systemic lupus erythematosus. Int J Dermatol, 36pp. Dapsonetype VII collagenimmunobullous diseases. Bullous gulloso lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin- 6,and type VII collagen.
Immunodominant autoepitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous NC1 domain.
The geoepidemiology of systemic lupus erythematosus. This article also discusses the epidemiology, clinical, histopathologic and immunopathologic features, differential diagnosis and the treatment of BSLE.
Male systemic lupus erythematosus in a latin-American inception cohort of patients.
Lupus ampolloso: un verdadero reto diagnóstico y terapéutico
Even lupue 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. Bullous dermatosis and systemic lupus erythematosus in a year-old boy. Hermann Pinkus memorial Lecture Our patient showed only modest improvement on steroids and antimalarials, complete response being seen only with dapsone.
Detection of anti-basement membrane zone antibodies in bullous systemic lupus erythematosus.
There was only partial improvement with new lesions developing even after 4 bulloao wherein, she was started on dapsone.
Bullous eruption of systemic lupus erythematosus.
Biopsy taken from vesicular lesion revealed a subepidermal split, containing numerous red blood cells. J Cutan Pathol, 20pp.
Localization of basement membrane components after dermal-epidermal junction separation. Type I fulfilling all six criteria, while Type II undetermined antigen llupus dermal antigen other than type VII collagen satisfies criteria only. Bullous systemic lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis.