A painful ear may herald serious disease

A case of otitis externa came with an unusual history, leading to an unusual diagnosis, explains Dr Keith Barnard.

Otoscopy examination was unable to detect relapsing polychondritis
Otoscopy examination was unable to detect relapsing polychondritis

Mrs Jenkins was a busy 47-year-old woman, and had been putting up with her painful right ear for a couple of weeks. She said it wasn't discharging, but it felt swollen. I asked her about dizziness or hearing loss but she had neither of these.

Examination showed the entrance to the external meatus to be swollen with no blistering, but some redness. When I examined the tympanic membrane it was normal.

This was the third similar ear problem I had seen that day, and I was about to talk about otitis externa when Mrs Jenkins volunteered that she had suffered this before, and then her doctor had prescribed antibiotic ear drops, but they had not worked.

Mrs Jenkins said, 'Can I tell you about what's really worrying me?' I tried an encouraging smile and nodded.

Joint pains
'I've been experiencing joint pains for the past few months, first in one place, then another, and I'm a bit worried because I've lost nearly a stone and I can't say I've been trying,' she said.

She really had my attention now, and further questioning determined that she had had episodes where she thought she was running a temperature, that she tired easily, and was puffing for breath when she went up the stairs.

A number of thoughts flashed through my mind ranging from rheumatoid arthritis (RA) to Hodgkin's to leukaemia.

I examined her in more detail, but could find no lymphandenopathy. Without trying to worry her too much, I referred her urgently to the physicians, expressing my concerns in the letter of a possible malignancy.

I heard nothing more from Mrs Jenkins for several weeks, but when I eventually received a letter from the hospital it transpired that the woman had received several series of investigations by a number of different puzzled specialists.

Eventually a biopsy of the persistent inflammatory area in her ear was performed and showed perichondrial lymphatic T-cell infiltration.

Heads were put together and the diagnosis was relapsing polychondritis.

Destructive inflammation
I had to go straight to the books to learn more.

Relapsing polychondritis is an episodic and progressive multi-system rheumatic disease involving cartilage and connective tissue. It can affect cartilage in the ears, nose and joints.

Histological evidence
Diagnosis is complex. The condition requires the presence of three out of six specific clinical criteria plus histological evidence to be sure of the diagnosis. Treatment involves steroids and other immunosuppressants.

The list of differential diagnoses is a long one, with some of the main ones being Wegener's granulomatosis, RA, Reiter's syndrome, syphilis, TB, histoplasmosis and sarcoidosis.

When I next saw Mrs Jenkins some two months later she was remarkably cheerful, despite developing some mooning of her face from the high-dose steroids she was taking.

Dr Barnard is a former GP in Fareham, Hampshire.

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