Article information
Resumen
La urticaria vasculítica (UV) es una entidad clinicopatológica caracterizada por episodios recurrentes de urticaria y vasculitis leucocitoclástica en la histopatología. Una minoría de pacientes con urticaria crónica tiene urticaria vasculítica (aproximadamente un 5%). Aunque la definición de vasculitis ha cambiado de un estudio a otro, en este artículo nos adherimos a los autores que consideran que la leucocitoclasia y los depósitos de fibrina son indispensables para definir la entidad. Clásicamente la UV se manifiesta con habones eritematosos recurrentes por más de cuatro a seis semanas, que duran más de 24 horas y desaparecen dejando hiperpigmentación residual. Como las características de la urticaria vasculítica pueden sobreponerse con las de la urticaria común, su diagnóstico siempre debe apoyarse en un estudio histopatológico. Según los niveles de complemento esta entidad puede subdividirse en urticaria vasculítica normocomplementémica e hipocomplementémica. Una minoría de pacientes con urticaria vasculítica hipocomplementémica cumple con criterios diagnósticos de síndrome de urticaria vasculítica hipocomplementémica. Aquellos pacientes con hipocomplementemia tienen mayor riesgo de desarrollar compromiso multiorgánico y frecuentemente desarrollan lupus eritematoso sistémico (LES) durante el seguimiento, especialmente los que cursan con anticuerpos anti-C1q.
Palabras clave:
urticaria
vasculitis
lupus eritematoso sistémico
Summary
Urticarial vasculitis is a clinic-pathologic entity typified by recurrent episodes of urticaria that have the histopathologic features of leukocytoclastic vasculitis. Only a minority of patients with chronic urticarial lesions have urticarial vasculitis (approximately 5%). Even though the definition of vasculitis has varied, in this article, we adhere to the concept that leukocytoclasis and fibrinoid deposits are the most important features to define this entity. Classically, urticarial vasculitis manifest with recurrent erythematosus wheals that last for more than 4–6 weeks, the individual lesions persist more than 24 hours and leave residual hyperpigmentation. Because clinical characteristics of urticarial vasculitis may overlap with those of common urticaria, confirmation of the diagnosis requires a lesional skin biopsy. Urticarial vasculitis can be classified as normocomplementemic or hypocomplementemic depending on seric complement levels. Only a minority of patients with hypocomplementemic urticarial vasculitis fulfill diagnostic criteria for hypocomplementemic urticarial vasculitis syndrome. Hypocomplementemic patients have the propensity to have more severe multi-organ involvement and frequently develop systemic lupus erythematous when they are followed in time, in special when they have anti-C1q antibodies.
Key words:
urticaria
vasculitis
lupus erythematosus systemic
Full text is only aviable in PDF
Referencias
[1.]
F.C. McDuffie, W.M. Sams Jr., J.E. Maldonado, et al.
Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome.
Mayo Clin Proc, 48 (1973), pp. 340-348
[2.]
J. Venzor, W.L. Lee, D.P. Huston.
Urticarial vasculitis.
Clin Rev Allergy Immunol, 23 (2002), pp. 201-216
[5.]
D.R. Mehregan, M.J. Hall, L.E. Gibson.
Urticarial vasculitis: a histopathologic and clinical review of 72 cases.
J Am Acad Dermatol, 26 (1992), pp. 441-448
[6.]
M.D.P. Davis, M.S. Daoud, B. Kirby, L.E. Gibson, R.S. Rogers.
Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis.
J Am Acad Dermatol, 38 (1998), pp. 899-905
[7.]
N.A. Soter, K.F. Austen, I. Gigli.
Urticaria and arthralgias as manifestations of necrotizing angiitis (vasculitis).
J Invest Dermatol, 63 (1974), pp. 485-490
[8.]
C.R. Zeiss, F.X. Burch, R.J. Marder, et al.
A hypocomplementemic vasculitic urticarial syndrome: A report of four new cases and definition of the disease.
Am J Med, 68 (1980), pp. 867-875
[9.]
V. Agnello, S. Ruddy, R.J. Winchester, et al.
Hereditary C2 deficiency in systemic lupus erythematosus and acquired complement abnormalities in an unusual SLErelated syndrome.
Birth Defects, 11 (1975), pp. 312-317
[10.]
M.D. Davis, J.D. Brewer.
Urticarial vasculitis and hypocomplementemic urticarial vasculitis syndrome.
Immunol Allergy Clin North Am, 24 (2004), pp. 183-213
[11.]
E.W. Monroe.
Urticarial vasculitis: an updated review.
J Am Acad Dermatol, 5 (1981), pp. 88-95
[12.]
N.A. Brown, J.D. Carter.
Urticarial vasculitis.
Curr Rheumatol Rep., 9 (2007), pp. 312-319
[13.]
J. Aboobaker, M.W. Greaves.
Urticarial vasculitis.
Clin Exp Dermatol, 11 (1986), pp. 436-444
[14.]
C.V. Dincy, R. George, M. Jacob, E. Mathai, S. Pulimood, E.P. Eapen.
Clinicopathologic profile of normocomplementemic and hypocomplementemic urticarial vasculitis: a study from South India.
J Eur Acad Dermatol Venereol, 22 (2008), pp. 789-794
[15.]
C. Tosoni, F. Lodi-Rizzini, M. Cinquini, G. Pasolini, M. Venturini, R.A. Sinico, P. Calzavara-Pinton.
A reassessment of diagnostic criteria and treatment of idiopathic urticarial vasculitis: a retrospective study of 47 patients.
Clin Exp Dermatol 2008, (2008 Aug 2),
[16.]
P.E. Koch, R. Lazova, J.R. Rosen, R.J. Antaya.
Urticarial vasculitis in an infant.
Cutis, 81 (2008), pp. 49-52
[17.]
S. Kaur, G.P. Thami.
Urticarial vasculitis in infancy.
Indian J Dermatol Venereol Leprol, 69 (2003), pp. 223-224
[18.]
A. Soylu, S. Kavukçu, N. Uzuner, N. Olgaç, O. Karaman, E. Ozer.
Systemic lupus erythematosus presenting with normocomplementemic urticarial vasculitis in a 4-year-old girl.
Pediatr Int, 43 (2001), pp. 420-422
[19.]
N.A. Soter.
Chronic urticaria as a manifestation of necrotizing venulitis.
N Engl J Med, 296 (1977), pp. 1440-1442
[20.]
D. Weedon.
Skin Pathology.
2nd, Churchill Livingstone, (2002),
[21.]
R. Russell Jones, B. Bhogal, A. Dash, et al.
Urticaria and vasculitis: A continuum of histopathological and immunopathological changes.
Br J Dermatol, 108 (1983), pp. 139-149
[22.]
R.E. Berg, G.R. Kantor, W.F. Bergfeld.
Urticarial vasculitis.
Int J Dermatol, 27 (1988), pp. 468-472
[23.]
J.S. Lee, T.H. Loh, S.C. Seow, S.H. Tan.
Prolonged urticaria with purpura: the spectrum of clinical and histopathologic features in a prospective series of 22 patients exhibiting the clinical features of urticarial vasculitis.
J Am Acad Dermatol, 56 (2007), pp. 994-1005
[24.]
N.L. Kao, H.J. Zeitz.
Urticarial skin lesions and polymyositis due to lymphocytic vasculitis.
West J Med, 162 (1995), pp. 156-158
[25.]
J. Guitart.
Lymphocytic vasculitis” is not urticarial vasculitis.
J Am Acad Dermatol, 59 (2008), pp. 353
[26.]
N.P. Sanchez, R.K. Winkelmann, A.L. Schroeter, C.H. Dicken.
The clinical and histopathologic spectrums of urticarial vasculitis: study of forty cases.
J Am Acad Dermatol, 7 (1982), pp. 599-605
[27.]
Y. Kano, M. Orihara, T. Shiohara.
Cellular and molecular dynamics in exercise-induced urticarial vasculitis lesions.
Arch Dermatol, 134 (1998), pp. 62-67
[28.]
K. Chen, M.R. Pittelkow, W.P. Su, et al.
Recurrent cutaneous necrotizing eosinophilic vasculitis.
Arch Dermatol, 130 (1994), pp. 1159-1166
[29.]
D.R. Mehregan, L.E. Gibson.
Pathophysiology of urticarial vasculitis.
Arch Dermatol, 134 (1998), pp. 88-89
[30.]
J.L. Dienstag, A.R. Rhodes, A.K. Bhan, A.M. Dvorak, M.C. Mihm Jr., J.R. Wands.
Urticaria associated with acute viral hepatitis type B: studies of pathogenesis.
Ann Intern Med, 89 (1978), pp. 34-40
[31.]
J.J. Wisnieski, S.M. Jones.
Comparison of autoantibody to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome and systemic lupus erythematosus.
J Immunol, 148 (1992), pp. 1396-1403
[32.]
J.J. Wisnieski, S.M. Jones.
IgG autoantibodies to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome, systemic lupus erythematosus, and 6 other musculoskeletal or rheumatic diseases.
J Rheumatol, 19 (1992), pp. 884-888
[33.]
J.J. Wisnieski, A.N. Baer, J. Christensen, T.R. Cupps, D.N. Flagg, J.V. Jones, P.L. Katzenstein, J.J. McMillen, E.R. McFadden, M.A. Pick, G.W. Richmond, S. Simon, H.R. Smith, R.D. Sontheimer, L.B. Trigg, D. Weldon, J.J. Zone.
Hypocomplementemic urticarial vasculitis syndrome: clinical and serologic findings in 18 patients.
Medicine, 74 (1995), pp. 24-41
[34.]
C.E. Siegert, M.R. Daha, M.L. Westedt, E. van der Voort, F. Breedveld.
IgG autoantibodies against C1q are correlated with nephritis, hypocomplementemia, and dsDNA antibodies in systemic lupus erythematosus.
J Rheumatol, 18 (1991), pp. 230-238
[35.]
I.E. Coremans, M.R. Daha, E.A. van der Voort, Y. Muizert, C. Halma, F.C. Breedveld.
Antibodies against C1q in anti-glomerular basement membrane nephritis.
Clin Exp Immunol, 87 (1992), pp. 256-260
[36.]
D.W. Lienesch, K.E. Sherman, A. Metzger, et al.
Anti-c1q antibodies in patients with chronic hepatitis C infection.
Clin Exp Rheumatol, 24 (2006), pp. 183-185
[37.]
A. Babjanians, M. Chung-Park, J.J. Wisnieski.
Recurrent pericarditis and cardiac tamponade in a patient with hypocomplementemic urticarial vasculitis syndrome.
J Rheumatol, 18 (1991), pp. 752-755
[38.]
D.P. Hunt, R. Weil, A.G. Nicholson, M.M. Burke, R.M. Du Bois, A.U. Wells.
Pulmonary capillaritis and its relationship to development of emphysema in hypocomplementemic urticarial vasculitis syndrome.
Sarcoidosis Vasc Diffuse Lung Dis, 23 (2006), pp. 70-72
[39.]
M. Mannik, M.H. Wener.
Deposition of antibodies to the collagen-like region of C1q in renal glomeruli of patients with proliferative lupus glomerulonephritis.
Arthritis Rheum, 40 (1997), pp. 1504-1511
[40.]
F. Vázquez-López, C. Maldonado-Seral, T. Soler-Sanchez, N. Perez-Oliva, A.A. Marghoob.
Surface microscopy for discriminating between common urticaria and urticarial vasculitis.
Rheumatology (Oxford), 42 (2003), pp. 1079-1082
[41.]
M.V. Dahl.
Clinical pearl: diascopy helps diagnose urticarial vasculitis.
J Am Acad Dermatol, 30 (1994), pp. 481-482
[42.]
A.S. Sturgess, G.O. Littlejohn.
Jaccoud's arthritis and panvasculitis in the hypocomplementemic urticarial vasculitis syndrome.
J Rheumatol, 15 (1988), pp. 858-861
[43.]
O. Ishikawa, Y. Miyachi, H. Watanabe.
Hypocomplementemic urticarial vasculitis associated with Jaccoud's syndrome.
Br J Dermatol., 137 (1997), pp. 804-807
[44.]
E. Palazzo, P. Bourgeois, O. Meyer, M. De Bandt, M. Kazatchkine, M. Kahn.
Hypocomplementemic urticarial vasculitis syndrome. Jaccoud's syndrome, valvulopathy: a new syndrome combination.
J Rheumatol, 20 (1993), pp. 1236-1240
[45.]
L. Hong, F. Wackers, M. Dewar, M. Kashgarian, P.W. Askenase.
Atypical fatal hypocomplementemic urticarial vasculitis with involvement of native and homograft aortic valves in an African American man.
J Allergy Clin Immunol, 106 (2000), pp. 1196-1198
[46.]
H.J. Chen, K.J. Bloch.
Hypocomplementemic urticarial vasculitis, Jaccoud's arthropathy, valvular heart disease, and reversible tracheal stenosis: a surfeit of syndromes.
J Rheumatol, 28 (2001), pp. 383-386
[47.]
H. Amano, N. Furuhata, N. Tamura, Y. Tokano, Y. Takasaki.
Hypocomplementemic urticarial vasculitis with Jaccoud's arthropathy and valvular heart disease: case report and review of the literature.
Lupus, 17 (2008 Sep), pp. 837-841
[48.]
H.R. Schwartz, F.C. McDuffie, L.F. Black, A.L. Schroeter, D.L. Conn.
Hypocomplementemic urticarial vasculitis: association with chronic obstructive pulmonary disease.
Mayo Clin Proc, 57 (1982), pp. 231-238
[49.]
S. Kobayashi, M. Nagase, S. Hidaka, T. Arai, N. Ikegaya, A. Hishida, N. Honda.
Membranous nephropathy associated with hypocomplementemic urticarial vasculitis: report of two cases and a review of the literature.
Nephron, 66 (1994), pp. 1-7
[50.]
N.A. Soter.
Urticarial vasculitis.
The urticarias, pp. 141-148
[51.]
M.D. Jones, E. Tsou, E. Lack, T.R. Cupps.
Pulmonary disease in systemic urticarial vasculitis: the role of bronchoalveolar lavage.
Am J Med, 88 (1990), pp. 431-434
[52.]
C.L. Ludivico, A.R. Myers, K. Maurer.
Hypocomplementemic urticarial vasculitis with glomerulonephritis and pseudotumor cerebri.
Arthritis Rheum, 22 (1979), pp. 1024-1028
[53.]
H. Alachkar, F. Qasim, Y. Ahmad, M. Helbert.
Meningococcal meningitis in a patient with urticarial vasculitis: is there a link?.
J Clin Pathol, 60 (2007), pp. 1160-1161
[54.]
P.A. Koul, A. Wahid, S.U. Shah, A.N. Koul, S.M. Saleem.
Hypocomplementemic urticarial vasculitis and lower cranial nerve palsies.
J Assoc Physicians India, 48 (2000), pp. 536-537
[55.]
J.P. Callen, S. Kalbfleisch.
Urticarial vasculitis: a report of nine cases and review of the literature.
Br J Dermatol, 107 (1982), pp. 87-94
[56.]
G.Y. Chew, P.A. Gatenby.
Inflammatory myositis complicating hypocomplementemic urticarial vasculitis despite on-going immunosuppression.
Clin Rheumatol, 26 (2007), pp. 1370-1372
[57.]
G. Bolla, P. Disdier, D. Verrot, L. Swiader, L. Andrac, J.R. Harle, et al.
Acute transverse mielitis and primary urticarial vasculitis.
Clin Rheumatol, 17 (1998), pp. 250-252
[58.]
K. Aydogan, S.K. Karadogan, S.B. Adim, et al.
Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus.
Int J Dermatol, 45 (2006), pp. 1057-1061
[59.]
K. Saigal, I.C. Valencia, J. Cohen, F.A. Kerdel.
Hypocomplementemic urticarial vasculitis with angioedema, a rare presentation of systemic lupus erythematosus: rapid response to rituximab.
J Am Acad Dermatol, 49 (2003), pp. S283-S285
[60.]
S. O’Loughlin, A.L. Schroeter, R.E. Jordon.
Chronic urticaria-like lesions in systemic lupus erythematosus: a review of 12 cases.
Arch Dermatol, 114 (1978), pp. 879-883
[61.]
T.T. Provost, J.J. Zone, D. Synkowski, P.J. Maddison, M. Reichlin.
Unusual cutaneous manifestations of systemic lupus erythematosus: I. Urticaria-like lesions. Correlation with clinical and serological abnormalities.
J Invest Dermatol, 75 (1980), pp. 495-499
[62.]
S. Kuniyuki, H. Katoh.
Urticarial vasculitis with papular lesions in a patient with type C hepatitis and cryoglobulinemia.
J Dermatol, 123 (1996), pp. 279-283
[63.]
E.L. Alexander, T.T. Provost.
Cutaneous manifestations of primary Sjögren's syndrome; a reflection of vasculitis and association with anti-Ro (SSA) antibodies.
J Invest Dermatol, 80 (1983), pp. 386-391
[65.]
R. Werni, T. Schwarz, F. Gschnait.
Colchicine treatment of urticarial vasculitis.
Dermatologica, 172 (1986), pp. 36-40
[66.]
J.C. Wiles, R.C. Hansen, P.J. Lynch.
Urticarial vasculitis treated with colchicine.
Arch Dermatol, 121 (1985), pp. 802-805
[67.]
J.P. Callen.
Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis.
J Am Acad Dermatol, 13 (1985), pp. 193-200
[68.]
R.A. Asherson, N. Buchanan, S. Kenwright, C.M. Fletcher, G.R. Hughes.
The normocomplementemic urticarial vasculitis syndrome- report of a case and response to colchicine.
Clin Exp Dermatol, 16 (1991), pp. 424-427
[69.]
C. Muramatsu, E. Tanabe.
Urticarial vasculitis: response to dapsone and colchicine.
J Am Acad Dermatol, 13 (1985), pp. 1055
[70.]
C.N. Matthews, E.M. Saihan, R.P. Warin.
Urticaria-like lesions associated with systemic lupus erythematosus: response to dapsone.
Br J Dermatol, 99 (1978), pp. 455-457
[71.]
J.S. Forston, J.J. Zone, M.E. Hammond, G.C. Groggel.
Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone.
J Am Acad Dermatol, 15 (1986), pp. 1137-1142
[72.]
A.R. Eiser, P. Singh, H.M. Shanies.
Sustained dapsoneinduced remission of hypocomplementemic urticarial vasculitis–a case report.
Angiology, 48 (1997), pp. 1019-1022
[73.]
T. Ruzicka, G. Goerz.
Systemic lupus erythematosus and vasculitic urticaria. Effect of dapsone and complement levels.
Dermatologica, 162 (1981), pp. 203-205
[74.]
L.R. Lopez, K.C. Davis, P.F. Kohler, et al.
The hypocomplementemic urticarial-vasculitis syndrome: Therapeutic response to hydroxychloroquine.
J Allergy Clin Immunol, 73 (1984), pp. 600-603
[76.]
A. Borcea, M.W. Greaves.
Methotrexate-induced exacerbation of urticarial vasculitis: an unusual adverse reaction.
Br J Dermatol, 143 (2000), pp. 203-204
[77.]
M. Worm, M. Muche, P. Schulze, W. Sterry, G. Kolde.
Hypocomplementemic urticarial vasculitis: successful treatment with cyclophosphamide-dexamethasone pulse therapy.
Br J Dermatol, 139 (1998), pp. 704-707
[78.]
J. Soma, H. Sato, S. Ito, T. Saito.
Nephrotic syndrome associated with hypocomplementemic urticarial vasculitis syndrome: successful treatment with cyclosporin A.
Nephrol Dial Transplant, 14 (1999), pp. 1753-1757
[79.]
R. Enríquez, A.E. Sirvent, F. Amorós, M. Pérez, J. Matarredona, A. Reyes.
Crescentic membranoproliferative glomerulonephritis and hypocomplementemic urticarial vasculitis.
J Nephrol, 18 (2005), pp. 318-322
[80.]
M. Worm, W. Sterry, G. Kolde.
Mycophenolate mofetil is effective for maintenance therapy of hypocomplementaemic urticarial vasculitis.
Br J Dermatol, 143 (2000), pp. 1324
[81.]
S. Hamid, P.D. Cruz Jr., W.M. Lee.
Urticarial vasculitis caused by hepatitis C virus infection: response to interferon alpha therapy.
J Am Acad Dermatol, 39 (1998), pp. 278-280
[82.]
R. Misiani, P. Bellavita, D. Fenili, O. Vicari, D. Marchesi, P.L. Sironi, et al.
Interferon alfa-2a therapy in cryoglobulinemia associated with hepatitis C virus.
N Engl J Med, 330 (1994), pp. 751-756
[83.]
P. Staubach-Renz, E. von Stebut, W. Bräuninger, M. Maurer, K. Steinbrink.
Hypocomplementemic urticarial vasculitis syndrome. Successful therapy with intravenous immunoglobulin.
Hautarzt, 58 (2007), pp. 693-697
[84.]
D. Shah, A.W. Rowbottom, C.L. Thomas, P. Cumber, M.M. Chowdhury.
Hypocomplementaemic urticarial vasculitis associated with non-Hodgkin lymphoma and treatment with intravenous immunoglobulin.
Br J Dermatol, 157 (2007), pp. 392-393
[85.]
D.R. Carr, M.P. Heffernan.
Off-label uses of rituximab in dermatology.
Dermatol Ther, 20 (2007), pp. 277-287
[86.]
P.S. Kelkar, J.H. Butterfield, A.N. Kalaaji.
Urticarial vasculitis with asymptomatic chronic hepatitis C infection: response to doxepin, interferon-alpha, and ribavirin.
J Clin Gastroenterol, 35 (2002), pp. 281-282
Copyright © 2009. Asociación Colombiana de Reumatología
