Epidermolysis Bullosa Acquisita Occuring In A Patient With Systemic Lupus Erythematosus
DOI:
https://doi.org/10.22219/sm.Vol15.SMUMM2.9884Keywords:
epidermolysis bullosa acquisita, systemic lupus erythematosu.Abstract
Epidermolysis Bullosa Acquisita (EBA) is a rare, chronic autoimmune subepidermal bullous disease and has been noted to be associated with systemic lupus erythematosus (SLE). The incidence of EBA and SLE in one patient within the period of 1980-1990 found only 7 published case reports. A 23 years old woman with exfoliate skin since 12 years ago. Initially itchy on her buttock then appeared small blister. Blister spread almost the entire body and rupture. This complaint got worsening in a year accompanied with hair loss, weight loss, and oral ulcer. Dermatological examination showed patch eritematosa, hyperpigmentation, hypopigmentation, erotion with erythematous base, yellow brownish crust. Also obtained sclerodactyli toes and nail fingers. Laboratory examination anemia gravis, hypoalbuminemia, Coombs test +2, ANA Test negatif, dsDNA IgG 32,80. Histopathology examination showed blister subepidermal, no vacuolar degeneration, no superficial and deep infiltrat, and minimal lymphocyte. Patient had diagnosed SLE from Internal Department based on MEX-SLEDAI score. The patient was treated with metylprednisolone intravenous pulse dose 500 mg on 3 days then tappering off and wound care. Epidermolysis Bullosa Acquisita immunogenetically related with MHC class II haplotype in particular HLA-DR2. This factor suggest playing role in the development of EBA to express more aggresive SLE.
Downloads
References
Badsha H, Edward CJ. (2003) Intravenous pulses of metylprednisolone for systemic lupus erythematosus. Semin Arthritis Rheum, 32(6), 370-77. Boh E, Roberts LJ, Lieu TS, Gammon WR, Sontheimer RD. (1990). Epidermolysis bullosa acquisita preceeding the development of systemic lupus erythematosus. J Am Acad Dermatol, 22: 587-93. Dotson AD, Raimer SS, Purslye TV, Tschen J. (1981). Systemic lupus erythematosus occuring in a patient with epidermolysis bullosa acquisita. Arch Dermatol, 117, 422-26. Gammon WR, Woodley DT, Dole KC, Briggaman RA. (1985). Evidence that anti-basement membrane zone antibodies in bullous systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigen. The Journal Of Investigate Dermatology, 84:472-76. Ganapathy S, Vedam V, Rajeev V, Arunachalam R. (2017). Autoimmune disorder-immunopathogenesis and potential therapies. J Young Pharm, 9(1) , 14-22. Gupta RBS, Woodley DT, Chen Mei. (2012). Epidermolysis bullosa acquisita. Clin Dermatol, 30(1), 60-69. Ishii N, Hamada T, Dainichi T, Karashima T, Nakama T, Yasumo S et al. (2010). Epidermolysis bullosa acquisita : what’s new?. Journal of Dermatology Japanese Dermatological Association, 37 , 220-30. Kim JH, Kim YH, Kim SC. (2011). Epidermolysis bullosa acquisita : a retrospective clinical analysis of 30 cases. Acta Derm Venereol, 91, 307-12. Kim JH, Kim SC. (2013). Epidermolysis bullosa acquisita. JEADV, 1-10. Ludwig RJ. (2013). Clinical presentation, pathogenesis, diagnosis, and treatment of epidermolysis bulosa acquisita. ISRN Dermatology, 1-26. Pope E, Corrales IL, Mellerio J, Martinez A, Schultz G, Burrell R, et al. (2012). Consensus approach to wound care in epidermolysis bullosa. J Am Acad Dermatol, 67(5) , 904-17. Woodley DT, Chen M. Epidermolysis Bullosa Acquisita. In Goldsmith LA, Katz SI, GilchrestBA, Paller AS, Leffel DJ, Wolff K, editor. (2012). Fitzpatrick’s dermatology in general medicine. 8th edition. New York : McGraw Hill Companies ; .p.634-41.
Downloads
Published
Issue
Section
License
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution-ShareAlike 4.0 International License that allows others to share the work with an acknowledgment of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgment of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).
This work is licensed under a Creative Commons Attribution-ShareAlike 4.0 International License.