Sleuthing for the Cause of an Odd Symptom Complex

I have a 74-year-old male patient who has peeling lips and recurrent painful aphthousulcers. When these symptoms occur, he also has painful adenitis at the angle ofthe mandibles, pain in the eyes (along with a feeling that he has sand in them whenhe closes them), and generalized achiness--and he feels so cold that he has to getunder a blanket to keep warm. In addition, the patient complains of joint pain, especiallyin his knees. An MRI scan of the right knee reveals a torn meniscus. Antinuclearantibody (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?
–MD

Clearly, this patient has an immunologic disorder, although it is difficult to explain allhis different symptoms with a single diagnosis.

Sorting through the differential. In general, when one sees a high ANA titer, onethinks of lupus. However, it would be rather unusual for lupus to develop at 74 yearsof age instead of in the second or third decade. Moreover, lupus is more common inwomen than in men. Drug-induced lupus does occur at this age, but it is not known whether thepatient takes any medications that might precipitate it.

Peeling of the lips is an uncommon symptom and suggests an allergic reaction, such as thatseen in Stevens-Johnson syndrome. This raises the possibility of a drug reaction; however-apartfrom 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 asReiter disease, the ulcers tend to be painless. They can also be painless in lupus. Behçet syndrome isassociated with painful ulcers-but not with high ANA titers.

The patient's adenitis, eye symptoms, joint symptoms, and general achiness are all seen in Sjgrensyndrome, which could also account for his abnormal immunologic test result. Sicca syndrome wouldexplain the sandlike feeling in his eyes, the adenitis, and the joint problems. The sensation of cold fits inwith any of the immunologic disorders. That the lower lip biopsy was inconclusive is rather typical; wesee 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.

Thus, the differential diagnosis has to include an overlap condition, such as "mixed connectivetissue disorder," or 2 separate disorders.

Treatment considerations. Treatment recommendations depend on laboratory results thatare not given (helpful test results in this setting would include those from a complete blood cellcount, measurements of erythrocyte sedimentation rate and C-reactive protein level, and a plateletcount) and on the answers to such questions as:

• Does the patient have any renal dysfunction?
• Is he short of breath?
• Are radiographs normal?

To determine whether this is a corticosteroid-sensitive phenomenon, try a short trial of entericcoatedprednisolone, starting with 10 mg each morning and tapering the dose to zero over a 4-weekperiod.

Appropriate eyedrops may alleviate the patient's ophthalmic symptoms.
- Andrei Calin, MD
    Consultant Rheumatologist
    Royal National Hospital for Rheumatic Diseases
    Upper Borough Walls
    Bath, England