Epidermolysis Bullosa Acquisita Occuring In A Patient With Systemic Lupus Erythematosus

Lita setyowatie, Yustian Devika Rahmawati, Arif Widiatmoko, Diah Prabawati Retnani


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.

 


Keywords


epidermolysis bullosa acquisita, systemic lupus erythematosu.

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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.




DOI: https://doi.org/10.22219/sm.Vol15.SMUMM2.9884 | Abstract views : 104 | PDF views : 104 |

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