REPORT OF A CASE
A 38-year-old woman was seen with a 12-year history of sudden and recurrent swelling of the lips and perioral skin. The edema lasted from days to weeks, generally worsening during the course of the day and sometimes impeding speech.
The patient was unaware of any allergies or provoking agents. She was otherwise healthy and was taking no medications. No family member had a similar condition.
Physical examination revealed indurated erythematous edema of the lips and face (Fig 1). The tongue was fissured. Results of a neurologic examination and an examination of the gums, teeth, and mucous membranes were normal.
The chest roentgenogram was normal, as were the results of laboratory studies, including the complete blood cell count, ESR, liver function tests, and serum electrolytes.
A labial biopsy specimen showed a patchy perivascular lymphohistiocytic and plasma cell infiltrate throughout the submucosa (Fig 2) and an occasional epithelioid
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