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Acute episode or chronic problem?

JOE R. MONROE, PA-C, MPAS

Joe Monroe practices in the dermatology department of the Warren Clinic, Tulsa, Oklahoma, is the department editor for Dermatology Digest, and is the founder and past president of the Society of Dermatology Physician Assistants. He has indicated no relationships to disclose relating to the content of this article.

CASE

A 59-year-old man presented with acute swelling of his lips and face. The swelling began about 2 hours before he arrived and was slowly resolving. Although the patient had experienced this swelling before, this instance was the first time his lips were affected. The patient denied any shortness of breath but admitted to a lifelong history of atopy marked by asthma, hives, seasonal allergies, and eczema. In the past, his primary-care physician treated his condition by prescribing antibiotics and a Medrol dosepak. This time the patient’s condition was more severe, so the physician referred the patient to an allergist, who, in turn, sent him to our dermatology department.

On physical examination, marked, painless swelling of the patient’s lips and surrounding tissue was noted (see Figure 1). The patient was not in acute distress. Despite the swollen lips, he was able to speak, albeit with some difficulty. No focal breaks in the skin were seen, and there were no palpable nodes in the area. Palpation of the lips revealed no tenderness, increased warmth, or redness.

THE MOST LIKELY DIAGNOSIS IS

  • Cellulitis
  • Angioedema
  • Contact dermatitis
  • A food allergy

DISCUSSION

The most likely diagnosis is angioedema, a deeper form of urticaria that causes painless, focal swelling of acute onset that typically lasts from a few minutes to 24 hours. While the lips are among the most commonly affected areas, angioedema can be seen on the eyelids, tongue, genitalia, and hands. It is most often idiopathic, but like ordinary urticaria it also can be stress-induced, related to ingestants, or caused by exposure to cold, heat, vibration, or ultraviolet light.

The fact that angioedema appears and resolves relatively quickly rules out a number of diagnostic possibilities. Moreover, the lack of pain or change in the skin surface is extremely helpful in eliminating diagnoses such as cellulitis and contact dermatitis from consideration.

Sensitivity to certain foods could lead to angioedema. However, given the patient’s history, a food allergy is not a likely diagnosis.

TREATMENT The list of conditions that exacerbate and resolve with such relative rapidity is a short one. In addition, this patient’s history of atopy and focal, painless swelling almost certainly make angioedema the diagnosis. But when this occurs without the usual wheals of common urticaria, the clinician needs to rule out hereditary angioedema, a rare autosomal-dominant condition.

Hereditary angioedema is a deficiency of C1-esterase inhibitor that allows for activation of the complement cascade. This results in the formation of several anaphylatoxins, which, in turn, can lead to death from laryngeal edema. This patient had no such family history, and his blood C1-esterase inhibitor level was normal.

Most, if not all, types of urticaria are caused by activation of mast cells by immune and non-immune mechanisms. While it is certainly worthwhile to investigate possible triggers for angioedema, most cases turn out to be idiopathic, as this one did.

The patient’s condition was treated with 10 mg cetirizine in the morning and 50 mg hydroxyzine at bedtime. Fortunately, the patient responded well after 2 days of treatment.






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