Medical Mystery: Treatment-resistant mouth sores

A 55-year-old man came to an oral health clinic after suffering from mouth sores for two months. The sores covered his tongue, top and bottom, as well as his lips and the insides of his cheeks, making it hard to eat much of anything. Not surprisingly, he was losing weight.

Soon after the mouth sores started, he said, he developed skin, genital, and eye sores. What's more, he was having a little trouble breathing.

He was evaluated by his dentist and an oral and maxillofacial surgeon. After a biopsy of his mouth sores, he was diagnosed with a dermatologic disease called lichen planus, a fairly common condition in which the immune system attacks cells of the skin or mucous membranes. Its cause is unknown.

But medications prescribed earlier for this condition didn't help this patient, whose mouth sores kept getting worse. He also had a raised lesion on the inside of his left eye, indicating his lichen planus had spread widely.

He was prescribed topical and systemic corticosteroids, anesthetic mouth rinse, and some changes to his diet and oral hygiene to ease his discomfort and promote healing.

On follow-up, his mouth sores appeared better, but they still were present and he still had trouble eating. His breathing issues had grown worse, and a pulmonology evaluation revealed very severe breathing obstruction.

Clearly, he had problems beyond lichen planus. The medical team ordered more tests to figure out what was going on.

Immunological blood tests suggested the patient's mouth sores and skin lesions were actually symptoms of an condition called paraneoplastic pemphigus, usually caused by an underlying cancer. Chest imaging showed scarring due to lung disease, accounting for his breathing problems.

His abdomen was full of enlarged lymph nodes, which on biopsy revealed lymphoma, a type of blood cancer. The patient responded somewhat to chemotherapy, but died of respiratory failure about a year after he first noticed the mouth sores.

Oral ulcers sometimes are the only outward sign of paraneoplastic pemphigus. Other times, as with this patient, there also are skin lesions.

It's a difficult condition to diagnose because the symptoms are similar to other, less serious problems.

However, this patient's case underscores the importance of regular dental exams and prompt evaluation of oral sores that don't heal quickly on their own. Far from simply annoying, these lesions may be the first signs of a possibly life-threatening condition.

Eric T. Stoopler, D.M.D., is an associate professor of oral medicine, and Thomas P. Sollecito, D.M.D., is a professor of oral medicine at the University of Pennsylvania School of Dental Medicine. A fuller version of this case appeared last year in the Special Care Dentistry Journal.

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