Hypogammaglobulinemia with Facial Edema

A 35-year-old man came to the offi ce with right-sided facial swelling, which he had noted over the last two years. The swelling was worse in the mornings and decreased through the day; however there were no symptom-free days. He had not experienced fevers, chills, or changes in his vision. He had experienced multiple episodes of lower extremity cellulitis, left more frequent than right leg, beginning in childhood. Although most instances were treated with oral antibiotics, he required hospitalization for intravenous antibiotics at least four times. Previous evaluation had confi rmed lymphedema by radionucleotide clearance. In addition, he had symptoms of allergic rhinitis and two episodes of otitis media as an adult, but he had no history of pneumonia or other signifi cant respiratory or gastrointestinal infections. On physical examination he had no evidence of wasting or malnutrition. He had normal tonsils and no cervical lymphadenopathy; there was mild right facial swelling with induration and trace erythema from the eye to mid-cheek. The right facial skin was slightly warmer than the left, but it was non-tender to palpation. Tympanic membranes and chest examination were normal. His legs were moderately swollen, left greater than right, with a " woody " or indurated texture to the left leg; there were no rashes or other skin lesions. He had no hepatosplenomegaly. Frequent infections raise the possibility of immunodefi ciency. A targeted immunologic evaluation should be guided by the clinical symptoms as well as the relative frequency of known immunodefi ciencies. Immune defects in the humoral system are most common and screening can be performed with tests for serum immunoglobulin levels and titers of specifi c antibody. A suggestion of immunoglobulin defi ciency arises if there is a low total protein on standard chemistry panels, as the immunoglobulins make up a considerable portion of serum proteins. Clinical symptoms of immunoglobulin defi ciency include increased frequency or severity of sino-pulmonary and other bacterial infections. Cellular immune defi ciencies are suggested by opportunistic and viral infections. An initial step in the evaluation of these is a complete blood count (a low lymphocyte number can be missed if only total white cells are counted) followed by a lymphocyte panel enumerating CD4 and CD8 T cells as well as B cells and natural killer cells. It is important to obtain the lymphocyte evaluation with a standard complete blood count to allow for the calculation of absolute numbers of …