Lupus bullous: a true diagnostic and therapeutic challenge
DOI:
https://doi.org/10.18041/2390-0512/bioc..1.2243Keywords:
Cutaneous lupus, blistering lupus, belimumabAbstract
Systemic lupus erythematosus (SLE) is an autoimmune disease in which multiple
factors induce an uncontrolled immune response in an individual who is
genetically predisposed, presenting a variety of clinical manifestations that often
become a real diagnostic challenge and Therapeutic as in the case of their skin
presentations. We will now describe a case of a male patient with a SLE
diagnosis with severe extensive skin lesions with clinical and histopathological
diagnosis suggestive of blistering lupus refractory to standard treatment with
corticosteroid therapy and immunomodulators with a morphologic evolution in
their course, which then requires the initiation of biological therapy with
Belimumab observed a significant clinical remission of the lesions and also
contributing to decrease the doses of corticosteroids used since their entry. We
will review aspects related to SLE in men in terms of epidemiology, cutaneous
manifestations, histopathological findings, differential diagnosis and current
therapeutic options.
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References
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Rosana Quintana, Romina Nieto , Bernardo A. Pons- Estel. Lupus eritematoso sistémico. En: Rafael Alba Feriz, Roberto Muñoz Lowis ,et al, editores. Enfermedades inflamatorias reumatológicas. Primera edición. Producciones científicas Ltda. 2016. P 301-340.
L.G. Okon, V.P. Werth 392 . Cutaneous lupus erythematosus: Diagnosis and treatment Best Practice & Research Clinical Rheumatology 27 . 2013. 391–404.
Luis Alonso González, Mauricio Restrepo, Gloria Vásquez. Lupus Eritematoso Sistémico Ampolloso: dramática respuesta a la terapia con dapsona. Presentación de caso y revisión de la literatura . VOL. 16 No. 4, Diciembre 2009, pp. 352-360.
M. Llamas-Velascoa,y B.E. Paredes. La biopsia cutánea: bases fundamentales. Parte II. Actas Dermosifiliogr. 2012;103(2):100-110
Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol1995;131:48-52.
Garcia MA, Marcos JC, Marcos AI, et al. Male systemic lupus erythematosus in a latin-American inception cohort of 1214 patients. Lupus.2005;14(12): 938-46.
Bertoli AM, Alarcon GS, Calvo-Alen J, et al; LUMINA study Group. Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical, features, course, and outcome in patients with lateonset disease. Artritis Rheum. 2006; 54(5): 1580-7.
O’Neill S, Cervera R. Systemic lupus erythematosus best pract res clin Rheumatol.2010; 24(6):841-55.
Pons-Estel GJ, Alarcon GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum.2010; 39(4): 257-68.
D´Cruz DP, khamashta MA, Hughes GR. Systemic lupus erythematosus.lancet.2007; 369(9561): 587-96.
Borchers AT, Naguwa SM, Shoenfeld Y, Gershwin ME. The geoepidemiology of systemic lupus erythematosus. Autoimmum Rev.2010; 9 (5): A 277-87.
Garcia MA, Marcos JC, Marcos AI, et al. Male systemic lupus erythematosus in a latin-American inception cohort of 1214 patients. Lupus.2005;14(12): 938-46.
Bertoli AM, Alarcon GS, Calvo-Alen J, et al; LUMINA study Group. Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical, features, course, and outcome in patients with lateonset disease. Artritis Rheum. 2006; 54(5): 1580-7.
Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol 2009; 10:365.
Battagliotti CA, Gentiletti AA, Pons-Estel BA, Berbotto GA, pons-Estel GJ En: lupus Eritematoso sistémico, aspectos clínicos y terapéuticos. Primera edición. 2013.Ed.Arcangel Maggio,Industria Grafica, Buenos Aires, Argentina.
Tuffanelli DL. Cutaneous immunopathology: recent observations. J Invest Dermatol 1975; 65:143.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol 1993; 100:28S.
Gandhi K, Chen M, Aasi S, et al. Autoantibodies to type VII collagen have heterogeneous subclass and light chain compositions and their complement-activating capacities do not correlate with the inflammatory clinical phenotype. J Clin Immunol 2000; 20:416.
Chen M, Chan LS, Cai X, et al. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol 1997;108:68-72.
Chan LS, Lapiere JC, Chen M, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen. Arch Dermatol 1999;135:569-573.
Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997;6:112-121.
Peter H Schur, MDSamuel L Moschella, MD, Mucocutaneous manifestations of systemic lupus erythematosus, updated: Apr 16, 2014.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol. 1993;100:28S-34S.
Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997;6:112-121.
Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol. 2004;22:129-138.
Chan LS, Lapiere JC, Chen M, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin- 6,and type VII collagen. Arch Dermatol 1999;135:569-573.
Dotson AD, Raimer SS, Pursley TV, et al. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Arch Dermatol 1981;117:422-426.
Eckman JA, Mutasim DF. Bullous systemic lupus erythematosus with milia and calcinosis. Cutis 2002;70:31-34.
Rappersberger K, Tschachler E, Tani M, Wolff K. Bullous disease in systemic lupus erythematosus. J Am Acad Dermatol.1989;21:745-752.
Miyagawa S, Shiomi Y, Fukumoto T, et al. Bullous eruption of systemic lupus erythematosus. J Dermatol 1994;21:421-425.
Malcangi G, Brandozzi G, Giangiacomi M, Zampetti M, Danieli Mg. Bullous SLE: response to methotrexate and relationship with disease activity. Lupus 2003;12:63-66.
Camisa C. Vesiculobullous systemic lupus erythematosus. A report of four cases. J Am Acad Dermatol.1988;18:93-100.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol. 1993;100:28S-34S.
Lalova A, Pramatarov K, Vassileva S. Facial bullous systemic lupus erythematosus. Int J Dermatol 1997;36:356-373.
Tsuchida T, Furue M, Kashiwado T, et al. Bullous systemic lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis. J Am Acad Dermatol 1994;31:387-390.
Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol. 2004;22:129-138.
Campos Domínguez M, Suárez Fernández R, Lázaro Ochaita P. Diagnostic methods in autoimmune subepidermal bullous diseases. Actas Dermosifiliogr. 2006;97:485-502.
Mutasim DF, Adams BB. Immunofluorescence in dermatology. J Am Acad Dermatol. 2001;45:803-22; quiz 822-4. Review.
Estelle Hau, MD et al. Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases. Medicine . 2014. Volume 93 ( 29) : 1-13.
Lihua Duan et al. Treatment of Bullous Systemic Lupus Erythematosus. Journal of Immunology Research. 2015: 1-6.
L. Durcan , M. Petri. Immunomodulators in SLE: Clinical evidence and immunologic actions. Journal of Autoimmunity. 2016; 1-12.
Navarra SV et al ; BLISS-52 Study Group. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet. 2011;377(9767):721-731.
Andreia Vilas-Boas, Sandra A Morais, David A Isenberg. Belimumab in systemic lupus erythematosus. BMJ - Rheumatic and musculoskeletal diseases. 2015; 1-6.
William Stohl et al. Belimumab Reduces Autoantibodies, Normalizes Low Complement Levels, and Reduces Select B Cell Populations in Patients With Systemic Lupus Erythematosus. Arthritis and Rheumatism. 2012 ; Vol 64 : 2328-2337.
Susan Manzi et al. Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: combined results from two phase III trials. Ann Rheum Dis. 2012;71:1833–1838.
Vibeke Strand et al. Improvements in health-related quality of life with belimumab, a B-lymphocyte stimulator-specific inhibitor, in patients with autoantibody-positive systemic lupus erythematosus from the randomized controlled BLISS trials. Ann Rheum Dis 2014;73:838–844.
Rosana Quintana, Romina Nieto , Bernardo A. Pons- Estel. Lupus eritematoso sistémico. En: Rafael Alba Feriz, Roberto Muñoz Lowis ,et al, editores. Enfermedades inflamatorias reumatológicas. Primera edición. Producciones científicas Ltda. 2016. P 301-340.
L.G. Okon, V.P. Werth 392 . Cutaneous lupus erythematosus: Diagnosis and treatment Best Practice & Research Clinical Rheumatology 27 . 2013. 391–404.
Luis Alonso González, Mauricio Restrepo, Gloria Vásquez. Lupus Eritematoso Sistémico Ampolloso: dramática respuesta a la terapia con dapsona. Presentación de caso y revisión de la literatura . VOL. 16 No. 4, Diciembre 2009, pp. 352-360.
M. Llamas-Velascoa,y B.E. Paredes. La biopsia cutánea: bases fundamentales. Parte II. Actas Dermosifiliogr. 2012;103(2):100-110
Bernard P, Vaillant L, Labeille B, et al. Incidence and distribution of subepidermal autoimmune bullous skin diseases in three French regions. Arch Dermatol1995;131:48-52.
Garcia MA, Marcos JC, Marcos AI, et al. Male systemic lupus erythematosus in a latin-American inception cohort of 1214 patients. Lupus.2005;14(12): 938-46.
Bertoli AM, Alarcon GS, Calvo-Alen J, et al; LUMINA study Group. Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical, features, course, and outcome in patients with lateonset disease. Artritis Rheum. 2006; 54(5): 1580-7.
O’Neill S, Cervera R. Systemic lupus erythematosus best pract res clin Rheumatol.2010; 24(6):841-55.
Pons-Estel GJ, Alarcon GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic lupus erythematosus. Semin Arthritis Rheum.2010; 39(4): 257-68.
D´Cruz DP, khamashta MA, Hughes GR. Systemic lupus erythematosus.lancet.2007; 369(9561): 587-96.
Borchers AT, Naguwa SM, Shoenfeld Y, Gershwin ME. The geoepidemiology of systemic lupus erythematosus. Autoimmum Rev.2010; 9 (5): A 277-87.
Garcia MA, Marcos JC, Marcos AI, et al. Male systemic lupus erythematosus in a latin-American inception cohort of 1214 patients. Lupus.2005;14(12): 938-46.
Bertoli AM, Alarcon GS, Calvo-Alen J, et al; LUMINA study Group. Systemic lupus erythematosus in a multiethnic US cohort. XXXIII. Clinical, features, course, and outcome in patients with lateonset disease. Artritis Rheum. 2006; 54(5): 1580-7.
Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol 2009; 10:365.
Battagliotti CA, Gentiletti AA, Pons-Estel BA, Berbotto GA, pons-Estel GJ En: lupus Eritematoso sistémico, aspectos clínicos y terapéuticos. Primera edición. 2013.Ed.Arcangel Maggio,Industria Grafica, Buenos Aires, Argentina.
Tuffanelli DL. Cutaneous immunopathology: recent observations. J Invest Dermatol 1975; 65:143.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol 1993; 100:28S.
Gandhi K, Chen M, Aasi S, et al. Autoantibodies to type VII collagen have heterogeneous subclass and light chain compositions and their complement-activating capacities do not correlate with the inflammatory clinical phenotype. J Clin Immunol 2000; 20:416.
Chen M, Chan LS, Cai X, et al. Development of an ELISA for rapid detection of anti-type VII collagen autoantibodies in epidermolysis bullosa acquisita. J Invest Dermatol 1997;108:68-72.
Chan LS, Lapiere JC, Chen M, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin-6, and type VII collagen. Arch Dermatol 1999;135:569-573.
Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997;6:112-121.
Peter H Schur, MDSamuel L Moschella, MD, Mucocutaneous manifestations of systemic lupus erythematosus, updated: Apr 16, 2014.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol. 1993;100:28S-34S.
Yell JA, Wojnarowska F. Bullous skin disease in lupus erythematosus. Lupus 1997;6:112-121.
Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol. 2004;22:129-138.
Chan LS, Lapiere JC, Chen M, et al. Bullous systemic lupus erythematosus with autoantibodies recognizing multiple skin basement membrane components, bullous pemphigoid antigen 1, laminin-5, laminin- 6,and type VII collagen. Arch Dermatol 1999;135:569-573.
Dotson AD, Raimer SS, Pursley TV, et al. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Arch Dermatol 1981;117:422-426.
Eckman JA, Mutasim DF. Bullous systemic lupus erythematosus with milia and calcinosis. Cutis 2002;70:31-34.
Rappersberger K, Tschachler E, Tani M, Wolff K. Bullous disease in systemic lupus erythematosus. J Am Acad Dermatol.1989;21:745-752.
Miyagawa S, Shiomi Y, Fukumoto T, et al. Bullous eruption of systemic lupus erythematosus. J Dermatol 1994;21:421-425.
Malcangi G, Brandozzi G, Giangiacomi M, Zampetti M, Danieli Mg. Bullous SLE: response to methotrexate and relationship with disease activity. Lupus 2003;12:63-66.
Camisa C. Vesiculobullous systemic lupus erythematosus. A report of four cases. J Am Acad Dermatol.1988;18:93-100.
Gammon WR, Briggaman RA. Bullous SLE: a phenotypically distinctive but immunologically heterogeneous bullous disorder. J Invest Dermatol. 1993;100:28S-34S.
Lalova A, Pramatarov K, Vassileva S. Facial bullous systemic lupus erythematosus. Int J Dermatol 1997;36:356-373.
Tsuchida T, Furue M, Kashiwado T, et al. Bullous systemic lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis. J Am Acad Dermatol 1994;31:387-390.
Vassileva S. Bullous systemic lupus erythematosus. Clin Dermatol. 2004;22:129-138.
Campos Domínguez M, Suárez Fernández R, Lázaro Ochaita P. Diagnostic methods in autoimmune subepidermal bullous diseases. Actas Dermosifiliogr. 2006;97:485-502.
Mutasim DF, Adams BB. Immunofluorescence in dermatology. J Am Acad Dermatol. 2001;45:803-22; quiz 822-4. Review.
Estelle Hau, MD et al. Neutrophilic Skin Lesions in Autoimmune Connective Tissue Diseases. Medicine . 2014. Volume 93 ( 29) : 1-13.
Lihua Duan et al. Treatment of Bullous Systemic Lupus Erythematosus. Journal of Immunology Research. 2015: 1-6.
L. Durcan , M. Petri. Immunomodulators in SLE: Clinical evidence and immunologic actions. Journal of Autoimmunity. 2016; 1-12.
Navarra SV et al ; BLISS-52 Study Group. Efficacy and safety of belimumab in patients with active systemic lupus erythematosus: a randomised, placebo-controlled, phase 3 trial. Lancet. 2011;377(9767):721-731.
Andreia Vilas-Boas, Sandra A Morais, David A Isenberg. Belimumab in systemic lupus erythematosus. BMJ - Rheumatic and musculoskeletal diseases. 2015; 1-6.
William Stohl et al. Belimumab Reduces Autoantibodies, Normalizes Low Complement Levels, and Reduces Select B Cell Populations in Patients With Systemic Lupus Erythematosus. Arthritis and Rheumatism. 2012 ; Vol 64 : 2328-2337.
Susan Manzi et al. Effects of belimumab, a B lymphocyte stimulator-specific inhibitor, on disease activity across multiple organ domains in patients with systemic lupus erythematosus: combined results from two phase III trials. Ann Rheum Dis. 2012;71:1833–1838.
Vibeke Strand et al. Improvements in health-related quality of life with belimumab, a B-lymphocyte stimulator-specific inhibitor, in patients with autoantibody-positive systemic lupus erythematosus from the randomized controlled BLISS trials. Ann Rheum Dis 2014;73:838–844.
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2018-01-27
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How to Cite
Escorcia Charris, E. J., Carbono Camargo, J., Salas Siado, J., & Coronell Buzon, L. (2018). Lupus bullous: a true diagnostic and therapeutic challenge. Biociencias, 13(1), 125-136. https://doi.org/10.18041/2390-0512/bioc..1.2243
