Angioedema

Angioedema (also called angioneurotic edema)-swelling of the skin and mucous membranes, especially around and in the mouth and throat... 

Pemphigus, bullous dermatitis herpetiformis, severe erythema multiforme (Stevens-Johnson syndrome), exfoliative dermatitis, mycosis fungoides, severe psoriasis, severe seborrheic dermatitis, angioedema, urticaria, various skin disorders, such as lichen planus or keloids... 

Adverse reactions to immune globulins are rare. However, local tenderness and pain at the injection site may occur. The most common adverse reactions include urticaria, angioedema, erythema, malaise, nausea, diarrhea, headache, chills, and fever. Adverse reactions, if they occur, usually last for several hours. Systemic reactions are extremely rare... 

II pruritus flush urticaria angioedema (not mandatory) nausea cramping rhinorrhea hoarseness dyspnea tachycardia (A >20 beats/min) blood pressure change (A >20 mm Hg systolic) arrhythmia... 

III pruritus flush Urticaria angioedema (not mandatory) vomiting defecation diarrhea laryngeal edema bronchospasm cyanosis shock... 

Cl inhibitor deficiency causes angioedema as a result of excessive bradykinin production. Thus the pathways and control mechanisms for bradykinin formation and... 

HAE is transmitted as an autosomal dominant disorder due, in most instances, to alterations of the Cl INH gene. Its prevalence is 1/50,000 [60] however, there is a high incidence of de novo mutations accounting for close to 25% of cases. Thus there may not be a family history to guide evaluation of such patients and it is therefore reasonable to obtain a C4 and Cl INH determination in any patient presenting with recurrent angioedema in the absence of urticaria. 

Schreiber A, Zweiman B, Atkins P. Goldwein F, Pietra G, Atkinson B, et al Acquired angioedema with lymphoproliferafive disorder association of Cl inhibitor deficiency with cellular abnormality. Blood 1976 48 567-580. 

Malbran A, Hammer C, Frank M. Fries L Acquired angioedema observations on the mechanism of action of autoanfibodies directed against C1 esterase inhibitor. J Allergy Clin Immunol 1988 81 1199-1204. 

Ruddy S, Gigli I, Sheffer A, Austen K The laboratory diagnosis of hereditary angioedema in Rose N, Richter M, Sehon A (eds) Proceedings of the Sixth International Congress of Allergology. Amsterdam, Excerpta Medica, 1968, pp 351-359. 

Zuraw B, Sugimoto S, Curd J The value of rocket Immunoelectrophoresis for C4 activation in the evaluation of patients with angioedema or Cl-inhibitor deficiency. J Allergy Clin Immunol 1986 78 1115-1120. 

Fields T, Ghebrehiwet B, Kaplan AP Kinin formation in hereditary angioedema plasma evidence against kinin derivation from C2 and in support of spontaneous formation of bradykinin. J Allergy 82 Chn Immunol 1983 72 54-60. 

Curd J, Yelvington M, Burridge N Generation of bradykinin during incubation of hereditary angioedema plasma. Mol Immunol 1983 19 1365. 83... 

Curd J, et al PrekaUikrein activation and high-molec-ular-weight kininogen consumption in hereditary angioedema. N Engl J Med 1983 308 1050-1053. 

Local bradykinin generation in hereditary angioedema. J Allergy Clin Immunol 1999 104 1321-1322. 

Zahedi R, Bissler J, Davis AR. Andreadis C. Wisnieski J Unique Cl inhibitor dysfunction in a kindred without angioedema. II. Identification of an Ala443 Val substitution and functional analysis 92 of the recombinant mutant protein. J Clin Invest 1995 95 1299-1305. 

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