Idiopathic Recurrent Angioedema

      Angioedema is a constellation of syndromes characterized by swelling of the deeper layers of the skin or submucosa (or both). Although any part of the body may be affected by angioedema, the face and extremities are most commonly involved. The swellings develop from extravasation of plasma in the affected areas. The extravasation is due to transient increase in endothelial permeability caused by vasoactive substances produced locally. Episodic abdominal pain arising from swelling of the submucosa of any portion of the gastrointestinal tract may occur without swelling of the skin; therefore, angioedema should be included in the differential diagnosis of intermittent, unexplained abdominal pain.
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      References

        • Cugno M.
        • Nussberger J.
        • Cicardi M.
        • et al.
        Bradykinin and the pathophysiology of angioedema.
        Int Immunopharmacol. 2003; 3: 311-317
        • Kaplan A.P.
        • Greaves M.W.
        Angioedema.
        J Am Acad Dermatol. 2005; 53: 373-388
        • Frigas E.
        • Nzeako U.C.
        Angioedema. Pathogenesis, differential diagnosis, and treatment.
        Clin Rev Allergy Immunol. 2002; 23: 217-231
        • Mathews K.P.
        Urticaria and angioedema.
        J Allergy Clin Immunol. 1983; 72: 1-14
        • Beltrani V.S.
        Angioedema: some “new” thoughts regarding idiopathic angioedema.
        in: Greaves M.W. Kaplan A.P. Urticaria and angioedema. Marcel Dekker, New York2004: 421-439
        • Champion R.H.
        • Greaves M.W.
        • Black A.K.
        • et al.
        The urticarias.
        Churchill Livingstone, Edinburgh1985
        • Champion R.H.
        Urticaria: then and now.
        Br J Dermatol. 1988; 119: 427-436
        • Krause K.H.
        • Rentrop U.
        • Mehregan U.
        Cerebral manifestations in angioneurotic edema.
        J Neurol Sci. 1979; 42 ([in German]): 429-435
        • Pellacani A.
        • Brunner H.R.
        • Nussberger J.
        Plasma kinins increase after angiotensin-converting enzyme inhibition in human subjects.
        Clin Sci (Lond). 1994; 87: 567-574
        • Nussberger J.
        • Cugno M.
        • Cicardi M.
        Bradykinin-mediated angioedema.
        N Engl J Med. 2002; 347: 621-622
        • Casale T.B.
        • Sampson H.A.
        • Hanifin J.
        • et al.
        Guide to physical urticarias.
        J Allergy Clin Immunol. 1988; 82: 758-763
        • Gorevic P.D.
        Cryopathies: cryoglobulins and cryofibrinogenemia.
        in: Frank M.M. Austen K.F. Claman H.N. Samter's immunologic diseases. 5th edition vol 2. Little, Brown and Company, Boston1995: 951-974
        • Nzeako U.C.
        • Frigas E.
        • Tremaine W.J.
        Hereditary angioedema: a broad review for clinicians.
        Arch Intern Med. 2001; 161: 2417-2429
        • Leznoff A.
        • Josse R.G.
        • Denburg J.
        • et al.
        Association of chronic urticaria and angioedema with thyroid autoimmunity.
        Arch Dermatol. 1983; 119: 636-640
        • Leznoff A.
        • Sussman G.L.
        Syndrome of idiopathic chronic urticaria and angioedema with thyroid autoimmunity: a study of 90 patients.
        J Allergy Clin Immunol. 1989; 84: 66-71
        • Gruber B.L.
        • Baeza M.L.
        • Marchese M.J.
        • et al.
        Prevalence and functional role of anti-IgE autoantibodies in urticarial syndromes.
        J Invest Dermatol. 1988; 90: 213-217
        • Grattan C.E.
        • Hamon C.G.
        • Cowan M.A.
        • et al.
        Preliminary identification of a low molecular weight serological mediator in chronic idiopathic urticaria.
        Br J Dermatol. 1988; 119: 179-183
        • Hide M.
        • Francis D.M.
        • Grattan C.E.
        • et al.
        Autoantibodies against the high-affinity IgE receptor as a cause of histamine release in chronic urticaria.
        N Engl J Med. 1993; 328: 1599-1604
        • Fiebiger E.
        • Maurer D.
        • Holub H.
        • et al.
        Serum IgG autoantibodies directed against the α chain of FcɛRI: a selective marker and pathogenetic factor for a distinct subset of chronic urticaria patients?.
        J Clin Invest. 1995; 96: 2606-2612
        • Fiebiger E.
        • Hammerschmid F.
        • Stingl G.
        • et al.
        Anti-FcɛRIα autoantibodies in autoimmune-mediated disorders: identification of a structure-function relationship.
        J Clin Invest. 1998; 101: 243-251
        • Niimi N.
        • Francis D.M.
        • Kermani F.
        • et al.
        Dermal mast cell activation by autoantibodies against the high affinity IgE receptor in chronic urticaria.
        J Invest Dermatol. 1996; 106: 1001-1006
        • Ferrer M.
        • Kinet J.P.
        • Kaplan A.P.
        Comparative studies of functional and binding assays for IgG anti-FcɛRIα (α-subunit) in chronic urticaria.
        J Allergy Clin Immunol. 1998; 101 (Erratum in. J Allergy Clin Immunol 1998;102(1):156): 672-676
        • Sabroe R.A.
        • Fiebiger E.
        • Francis D.M.
        • et al.
        Classification of anti-FcɛRI and anti-IgE autoantibodies in chronic idiopathic urticaria and correlation with disease severity.
        J Allergy Clin Immunol. 2002; 110: 492-499
        • Ferrer M.
        • Nakazawa K.
        • Kaplan A.P.
        Complement dependence of histamine release in chronic urticaria.
        J Allergy Clin Immunol. 1999; 104 (Erratum in. J Allergy Clin Immunol 2000;105(2):392): 169-172
        • Kikuchi Y.
        • Kaplan A.P.
        A role for C5a in augmenting IgG-dependent histamine release from basophils in chronic urticaria.
        J Allergy Clin Immunol. 2002; 109: 114-118
        • Horn M.P.
        • Pachlopnik J.M.
        • Vogel M.
        • et al.
        Conditional autoimmunity mediated by human natural anti-FcɛRIα autoantibodies?.
        FASEB J. 2001; 15: 2268-2274
        • Pachlopnik J.M.
        • Horn M.P.
        • Fux M.
        • et al.
        Natural anti-FcɛRIα autoantibodies may interfere with diagnostic tests for autoimmune urticaria.
        J Autoimmun. 2004; 22: 43-51
        • Confino-Cohen R.
        • Aharoni D.
        • Goldberg A.
        • et al.
        Evidence for aberrant regulation of the p21Ras pathway in PBMCs of patients with chronic idiopathic urticaria.
        J Allergy Clin Immunol. 2002; 109: 349-356
        • Grattan C.E.
        • Francis D.M.
        • Slater N.G.
        • et al.
        Plasmapheresis for severe, unremitting, chronic urticaria.
        Lancet. 1992; 339: 1078-1080
        • O'Donnell B.F.
        • Barr R.M.
        • Black A.K.
        • et al.
        Intravenous immunoglobulin in autoimmune chronic urticaria.
        Br J Dermatol. 1998; 138: 101-106
        • Grattan C.E.
        • O'Donnell B.F.
        • Francis D.M.
        • et al.
        Randomized double-blind study of cyclosporin in chronic ‘idiopathic’ urticaria.
        Br J Dermatol. 2000; 143: 365-372
        • Gach J.E.
        • Sabroe R.A.
        • Greaves M.W.
        • et al.
        Methotrexate-responsive chronic idiopathic urticaria: a report of two cases.
        Br J Dermatol. 2001; 145: 340-343
        • Kozel M.M.
        • Mekkes J.R.
        • Bossuyt P.M.
        • et al.
        The effectiveness of a history-based diagnostic approach in chronic urticaria and angioedema.
        Arch Dermatol. 1998; 134: 1575-1580
        • Agostoni A.
        • Aygoren-Pursun E.
        • Binkley K.E.
        • et al.
        Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.
        J Allergy Clin Immunol. 2004; 114: S51-S131
        • Bork K.
        • Fischer B.
        • Dewald G.
        Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy.
        Am J Med. 2003; 114: 294-298
        • Greaves M.W.
        Chronic urticaria as new autoimmune disease?.
        in: Hertl M. Autoimmune diseases of the skin: pathogenesis, diagnosis, management. Springer, Wien2001: 283-302
        • Rumbyrt J.S.
        • Schocket A.L.
        Chronic urticaria and thyroid disease.
        Immunol Allergy Clin North Am. 2004; 24: 215-223
        • Winkelmann R.K.
        • Frigas E.
        Eosinophilic panniculitis: a clinicopathologic study.
        J Cutan Pathol. 1986; 13: 1-12
        • Gleich G.J.
        • Schroeter A.L.
        • Marcoux J.P.
        • et al.
        Episodic angioedema associated with eosinophilia.
        N Engl J Med. 1984; 310: 1621-1626
        • Bressler R.B.
        • Sowell K.
        • Huston D.P.
        Therapy of chronic idiopathic urticaria with nifedipine: demonstration of beneficial effect in a double-blinded, placebo-controlled, crossover trial.
        J Allergy Clin Immunol. 1989; 83: 756-763
        • Weiner M.J.
        Methotrexate in corticosteroid-resistant urticaria.
        Ann Intern Med. 1989; 110: 848
        • Wong E.
        • Eftekhari N.
        • Greaves M.W.
        • et al.
        Beneficial effects of danazol on symptoms and laboratory changes in cholinergic urticaria.
        Br J Dermatol. 1987; 116: 553-556
        • Parsad D.
        • Pandhi R.
        • Juneja A.
        Stanozolol in chronic urticaria: a double blind, placebo controlled trial.
        J Dermatol. 2001; 28: 299-302
        • Parslew R.
        • Pryce D.
        • Ashworth J.
        • et al.
        Warfarin treatment of chronic idiopathic urticaria and angio-oedema.
        Clin Exp Allergy. 2000; 30: 1161-1165
        • Wiles J.C.
        • Hansen R.C.
        • Lynch P.J.
        Urticarial vasculitis treated with colchicine.
        Arch Dermatol. 1985; 121: 802-805
        • Nurnberg W.
        • Grabbe J.
        • Czarnetzki B.M.
        Urticarial vasculitis syndrome effectively treated with dapsone and pentoxifylline.
        Acta Derm Venereol. 1995; 75: 54-56
        • Jaffer A.M.
        Sulfasalazine in the treatment of corticosteroid-dependent chronic idiopathic urticaria.
        J Allergy Clin Immunol. 1991; 88: 964-965
        • Engler R.J.
        • Squire E.
        • Benson P.
        Chronic sulfasalazine therapy in the treatment of delayed pressure urticaria and angioedema.
        Ann Allergy Asthma Immunol. 1995; 74: 155-159