Lupus ampolloso: un verdadero reto diagnóstico y terapéutico
DOI:
https://doi.org/10.18041/2390-0512/bioc..1.2243Palabras clave:
Lupus cutáneo, lupus ampolloso, belimumabResumen
El lupus eritematoso sistémico (LES) es una enfermedad de tipo autoinmune en donde existen múltiples factores que inducen una respuesta inmunológica no controlada en un individuo que genéticamente está predispuesto, presentándose una variedad de manifestaciones clínicas que muchas veces se convierten en un verdadero reto diagnóstico y terapéutico como es el caso de sus presentaciones en piel. Describiremos a continuación un caso de un paciente género masculino con diagnostico LES con lesiones cutáneas extensas severas con diagnóstico clínico y histopatológico sugestivo de lupus ampolloso refractarias a tratamiento estándar con corticoterapia e inmunomoduladores con evolución tórpida en su curso, que requiere posteriormente inicio de terapia biológica con Belimumab observándose una remisión clínica significativa de las lesiones y contribuyendo además a disminuir las dosis de corticoides utilizadas desde su ingreso . Se revisarán aspectos en relación del LES en hombres en lo que refiere a epidemiologia, manifestaciones cutáneas, hallazgos histopatológicos, diagnóstico diferencial y opciones terapéuticas actuales.
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Referencias
M. G. Enríquez-Mejía. Fisiopatología del lupus eritematoso sistémico. Revista de medicina e investigación. 2013; 1(1):8-16.
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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Cómo citar
Lupus ampolloso: un verdadero reto diagnóstico y terapéutico. (2018). Biociencias, 13(1), 125-136. https://doi.org/10.18041/2390-0512/bioc..1.2243
