I have a 74-year-old male patient who has peeling lips and recurrent painful aphthous
ulcers. When these symptoms occur, he also has painful adenitis at the angle of
the mandibles, pain in the eyes (along with a feeling that he has sand in them when
he closes them), and generalized achiness—and he feels so cold that he has to get
under a blanket to keep warm. In addition, the patient complains of joint pain, especially
in his knees. An MRI scan of the right knee reveals a torn meniscus. Antinuclear
antibody (ANA) titer is 1:1280, and results of tests for cytomegalovirus, parvovirus,
and Epstein-Barr virus are all positive. Lower-lip biopsies are inconclusive.
What is the likely diagnosis, and what treatments would be effective?
Clearly, this patient has an immunologic disorder, although it is difficult to explain all his different symptoms with a single diagnosis.
Sorting through the differential. In general, when one sees a high ANA titer, one thinks of lupus. However, it would be rather unusual for lupus to develop at 74 years of age instead of in the second or third decade. Moreover, lupus is more common in women than in men. Drug-induced lupus does occur at this age, but it is not known whether the patient takes any medications that might precipitate it.
Peeling of the lips is an uncommon symptom and suggests an allergic reaction, such as that seen in Stevens-Johnson syndrome. This raises the possibility of a drug reaction; however—apart from drug-induced lupus—it would be unusual to see such a high ANA titer in this setting.
The patient's recurrent aphthous ulcers are painful. Typically, in a reactive arthropathy, such as Reiter disease, the ulcers tend to be painless. They can also be painless in lupus. Behçet syndrome is associated with painful ulcers—but not with high ANA titers.
The patient's adenitis, eye symptoms, joint symptoms, and general achiness are all seen in Sjögren syndrome, which could also account for his abnormal immunologic test result. Sicca syndrome would explain the sandlike feeling in his eyes, the adenitis, and the joint problems. The sensation of cold fits in with any of the immunologic disorders. That the lower lip biopsy was inconclusive is rather typical; we see this in Sjögren syndrome and in other settings.
Given the MRI finding of a torn meniscus, the knee pain sounds as though it were coincidental— although torn menisci are found in patients with no knee symptoms.