Edema
Peter J. Lynch
Libby Edwards
Edema consists of increased subcutaneous interstitial tissue fluid. Acute edema is generally related to plasma and termed angioedema, whereas chronic edema is associated with lymph and termed lymphedema. When lymphedema is extraordinary so that hardening and disfigurement occurs, the result is termed elephantiasis. This secondary change is sometimes accompanied by firm nodules and even hyperkeratosis, resulting in elephantiasis nostras verrucosa. At times, angioedema can transition into lymphedema, especially when inflammation is operative, so that the distinction is not always straightforward.
Although both forms of edema are characterized by swelling, acute edema is generally softer and usually exhibits pitting (Fig. 12-1). Chronic lymphedema, however, is usually firm, more stable, and nonpitting (Fig. 12-2). Edema of both types are often asymptomatic, although acute edema associated with an allergic reaction tend to itch, and there are often symptoms associated with the underlying cause of the edema, such as infection or skin disease.
 Fig. 12-1. This woman experienced immediate, soft, pruritic angioedema of the labia minora during sexual activity, with no systemic symptoms. |
Acute Genital Edema (Angioedema)
Genital edema is most often associated with an allergic reaction, either to a topically applied agent or to a systemic allergen in a sensitized patient. Genitalia affected with angioedema related to an allergic reaction normally begins quickly and then resolves over hours to days, returning to normal between each episode.
Immunoglobulin E—Mediated Acute Edema
Type I hypersensitivity reactions are the most dangerous and important cause of acute genital edema. Patients with these immunoglobulin E (IgE)-mediated reactions risk the possibility of a systemic response and anaphylaxis. Latex and semen are the most common causes of this reaction in the genitalia.
 Fig. 12-2. Firm, nonpitting, stable, asymptomatic lymphedema is often associated with hidradenitis suppurativa. |
Latex Allergy
Latex has become a well-known cause of important allergic reactions, fortunately leading to near elimination of latex gloves and other latex sources in most health care environments. Up to 7.6% of the general population has a latex allergy, depending upon frequency of exposure and the presence of atopy.1
Although contact dermatitis with scaling and eczematous change is the most common skin reaction to latex in a sensitized patient, contact urticaria with edema and itching can also occur, and is relevant to this discussion. Occasionally, these patients can develop systemic reactions including anaphylaxis. Often, patients with type I hypersensitivity reaction to latex also report sensitivities to fruit, including banana and avocado.
The diagnosis is made on the basis of prick skin tests and serologic measurement of specific IgE. These patients should be managed by an allergist. Patient education and avoidance of latex is paramount; these patients should be aware of the possibility of latex in condoms, diaphragms, and other genital devices. Immunotherapy has been used, and the biologic omalizumab has shown promise.2
Semen Allergy
IgE-mediated hypersensitivity to semen (seminal plasma) is probably an underdiagnosed condition, affecting as many as 40 000 women in the United States.3 Hypersensitivity to the prostate-associated antigen has been reported as the culprit.4
Women can experience either localized or systemic reactions, with systemic reactions due to IgE hypersensitivity and localized due to a different immunologic phenomena.5 Symptoms begin with vaginal discomfort and, in the case of a systemic reaction, are followed by wheezing, urticaria, shortness of breath, angioedema, gastrointestinal symptoms, and sometimes anaphylaxis.4 Interestingly, about half of cases of localized reactions occur at first coitus. One study has noted cross-reactivity of an antigen found in male dog dander with the prostateassociated antigen, perhaps explaining this.6
The diagnosis is made clinically, when symptoms occur soon after intercourse, and are prevented with use of a condom. Edema following sexual activity can also occur in a setting of candidiasis, even very mild subclinical candidiasis, and following prolonged or traumatic intercourse causing an irritant reaction.
Other Allergic Reactions
Genital edema that occurs as a result of most other IgE-mediated allergic reactions is accompanied by systemic signs, including, at times, anaphylaxis. Other than antihistamines and epinephrine, these require input and management by an allergist.
Genital edema sometimes is related to topically applied agents as a result of a type IV cell-mediated immune response. Although this usually produces a contact dermatitis with eczematous morphology, rare cases of anaphylaxis have been reported with some topical medications including neomycin and bacitracin. Unlike immediate IgE-mediated reactions, these are delayed by many hours following exposure, show more redness, and are accompanied by eczematous change. Contact dermatitis and its causes are discussed primarily in Chapter 5.
Bradykinin Pathway-Mediated Acute Edema
Bradykinin-mediated angioedema is a rare disease, due to vasodilation and increased vascular permeability resulting from bradykinin. There is no urticaria or skin rash, and neither antihistamines nor corticosteroids are useful. This most commonly affects the abdominal and upper airways, and genital involvement occurs in only a small number of patients. This condition occurs due to an excess of bradykinin, occurring most often in patients receiving angiotensin-converting enzyme inhibitors but also in patients with C1 inhibitor deficiency leading to hereditary angioedema.7 Management consists, obviously, of discontinuation of any causative medication, and perhaps icatibant, a bradykinin B2 receptor antagonist, in angiotensin-converting enzyme-associated disease. Recent literature does not support its use in hereditary disease.8
Acute Genital Edema Related to Infection
Infection can produce genital edema. The most common infections to do this include bacterial infection, especially cellulitis, candidiasis in women, and epididymitis in men. Cellulitis is characterized by edema, erythema, and pain, often accompanied by fever and an elevated white blood count (Fig. 12-3). Risk factors include immunosuppression,
skin diseases that produce fissures and erosions, diabetes, and liver/kidney disease. The diagnosis is made clinically and confirmed by blood cultures, wound cultures when there are appropriate sites to culture, and response to treatment. Patients who are ill with more severe constitutional should be evaluated for a necrotizing deep tissue infection (Fournier gangrene, necrotizing fasciitis). This necrosis prevents the delivery of antibiotics to the area of infection, and this is a life-threatening emergency requiring urgent surgical debridement (Fig. 12-4).
skin diseases that produce fissures and erosions, diabetes, and liver/kidney disease. The diagnosis is made clinically and confirmed by blood cultures, wound cultures when there are appropriate sites to culture, and response to treatment. Patients who are ill with more severe constitutional should be evaluated for a necrotizing deep tissue infection (Fournier gangrene, necrotizing fasciitis). This necrosis prevents the delivery of antibiotics to the area of infection, and this is a life-threatening emergency requiring urgent surgical debridement (Fig. 12-4).
 Fig. 12-3. This woman has developed cellulitis, manifested by the acute onset of edema, erythema, and pain. |
 Fig. 12-4. Fournier gangrene (necrotizing fasciitis) refers to bacterial infection that has progressed to deep tissue necrosis, obliterating blood vessels and interrupting the blood supply to the area; this causes edema and sloughing, generally resulting in a catastrophic outcome without early intervention. |
Candidiasis often produces puffiness of the modified mucous membranes of the vulva, but rarely, subclinical candidiasis is responsible for immediate and remarkable immediate edema with intercourse. Occasionally, even a culture is negative but ongoing suppression with once or twice weekly fluconazole prevents the edema.
Epididymitis is characterized by redness, pain, and edema of the scrotum, usually one-sided. This is most likely produced by gonorrhea or chlamydia in young men, or spread from a urinary tract infection or prostatitis in older men.
Acute Genital Edema Related to Trauma
Clearly, trauma can produce edema or deep contusions, but these are usually recognized. However, hair can rarely twist around the clitoris or glans penis and gradually produce strangulation and edema. Long-distance bicycle riding can produce unrecognized trauma and edema. Peritoneal dialysis and paracentesis are sometimes associated with acute edema, and vulvar edema is well known to occur during late pregnancy and following delivery.
Idiopathic Acute Scrotal Edema
This condition occurs primarily in boys and adolescents, with 60%-90% younger than 10 years of age.9 Children present with sudden painless edema and redness of the scrotum, bilateral or unilateral. The main diseases to be considered in the differential diagnosis are epididymitis and testicular torsion. The condition clears without treatment over 2 or 3 days. Diagnosis consists of the exclusion of more important diseases; Doppler shows equal blood supply to both testicles, and ultrasound shows homogeneity of the testicular parenchyma.9 Although rare, this can occur in adults as well, and ultrasound and a careful examination may avoid unnecessary surgery.10
Chronic Genital Edema (Elephantiasis)
Chronic edema occurs when there is disruption of lymphatic drainage; this can occur as a result of tumor, surgery, infection, radiation, infection, structural abnormalities, etc. Occasionally, there is no known cause. Although lymphedema of some areas of the body, such as the legs, can be somewhat managed with compression, chronic edema of the genital area is rarely successfully treated.
Chronic Edema Related to Congenital Abnormalities
Primary lymphangioma circumscriptum is a congenital vascular abnormality of lymphatic vessels manifested by grouped, sturdy vesicles that represent lymphatic vessels pushing up through the skin. These usually contain clear fluid but occasionally bloody fluid where the underlying lymphatic vessels connect with blood vessels. This malformation may or may not be associated with local edema. Treatment is difficult, since destruction or removal of the superficial vesicles is regularly followed by recurrence because the underlying malformation persists. There have been some recent case reports of benefit from topical sirolimus 0.1%, an inhibitor of angiogenesis.11
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