RCOM RADIOLOGICAL CASE OF THE MONTH

A 47-year-old woman presented to the rheumatology clinic for evaluation of her back pain. She described aching thoracic back pain that interfered with her sleep. She denied other musculoskeletal symptoms and had no gastrointestinal symptoms. Her medical history was significant for left-sided facial edema, left facial nerve paralysis, and 6 months of chronic headaches. A computed tomography (CT) scan of the face had been performed, which showed an abnormality in the soft tissues of the left cheek. A soft tissue biopsy of the cheek revealed nonspecific granulomatous inflammation. She was given the presumptive diagnosis of Melkersson-Rosenthal syndrome and was referred for further evaluation of the relationship of the syndrome with her musculoskeletal pain. She had been treated with glucocorticoids for 1 month with no obvious benefit. Physical examination was remarkable only for left-sided facial swelling and thoracic kyphosis. There was no evidence of peripheral inflammatory arthritis. A previous CT scan of the face was reviewed, and radiographs of the thoracic spine were ordered.

Radiographs of the thoracic spine revealed multiple mild wedge compression deformities of midthoracic vertebral bodies, resulting in kyphosis. Schmorl’s nodes, eburnation, osteophytes, and changes in disc space narrowing were visualized at multiple discovertebral junctions (Figure 1). A CT scan of the face showed abnormal soft tissue density in the subcutaneous tissues overlying the malar eminence of the left cheek but no other abnormality (Figure 2).

Scheuermann’s disease and Melkersson-Rosenthal syndrome

Scheuermann’s disease is a kyphotic deformity of the spine that develops in adolescence.¹ The disease is usually recognized by the postural deformity and the radiographic appearance of anterior wedging of ≥5 degrees in 3 consecutive thoracic vertebral bodies.² Additional radiographic findings include Schmorl’s nodes, end-plate narrowing, and irregular end plates that help confirm the diagnosis.² The etiology of Scheuermann’s disease remains unknown, but genetics³ and infection⁴ have been implicated as contributing factors. While Scheuermann’s disease is usually detected in adolescents, adults may present with back pain and may be diagnosed after age 40.⁵

Melkersson-Rosenthal syndrome, sometimes called cheilitis granulomatosa, is a rare condition of unknown etiology characterized by non-caseating granulomatous inflammation of the face with edema, facial nerve paralysis, and plication of the tongue.⁶ Not all patients with this syndrome have all of the clinical findings, as in this case in which the tongue findings were absent.⁷ There is a disputed association of the syndrome with Crohn’s disease.^8,9 Melkersson-Rosenthal syndrome is not known to be associated with the musculoskeletal findings of Scheuermann’s disease.

After discussing the radiographic findings with the radiologist, the patient recalled having back pain as a teenager. Since the symptoms of facial edema and 7th nerve palsy appeared 30 years after the first back symptoms, it is highly unlikely that the conditions are related. The patient has the unusual situation of having a common childhood problem undiagnosed until middle age; at the same time, she suffers from a very rare syndrome. The medical history was not as useful as radiographic diagnosis for evaluation of back pain in this case.