A Tricky Case of Arm Pain and a Swollen Ear

Stephen Paget, MD

Disclosures

July 27, 2016

Case Diagnosis

The main clinical challenge with this patient was to first determine whether we were dealing with two or three separate illnesses that seemingly occurred at the same time or a single illness with multiple components.

The patient seems to have relapsing polychondritis, with the characteristic relapsing and remitting episodes of inflammation in the region of the ear cartilage, but with no inflammation in the lobule, which contains no cartilage. Relapsing polychondritis can be a severe, episodic, and progressive inflammatory condition that involves cartilaginous structures, predominantly those of the ears, nose, and laryngotracheobronchial tree. The eyes, cardiovascular system, peripheral joints, skin, middle and inner ear, and central nervous system can also be affected. The condition can occur as a local or systemic vasculitic illness unto itself, or can be the manifestation of other autoimmune disorders, such as systemic lupus erythematosus. In some situations, it is a paraneoplastic syndrome.

The array of possible presenting symptoms and the episodic nature of relapsing polychondritis can result in a significant delay in its diagnosis. In addition, no laboratory findings are specific for this condition. A laboratory evaluation commensurate with the spectrum of reported symptoms is indicated to ascertain the presence of complicating conditions. Usually, the clinical presentation is sufficiently compelling that a painful ear cartilage biopsy is not necessary. The mainstay of treatment for more severe disease is systemic corticosteroid therapy, but other medications have been tried, including anti-malarial drugs, immunosuppressive agents, and dapsone.

It seems clear that her parvovirus B19 infection was transmitted from her son, whose entire school was dealing with a mini epidemic. She developed the classic slapped cheek rash and lacy erythematous skin changes on the arms and legs. Also present was a transient symmetrical polyarthritis that resembled rheumatoid arthritis but, as in most cases, was self-limited. Immunoglobulin M titers remained elevated for 3 months and helped support the diagnosis.

But what is the cause of the patient's severe neck, shoulder, and arm pain, and how does it fit into the entire clinical picture? Parsonage-Turner syndrome is an uncommon neurologic disorder characterized by the rapid onset of severe pain in the shoulder and arm. The syndrome is also known as Parsonage-Aldren-Turner syndrome, acute brachial neuropathy, acute brachial radiculitis, neuralgic amyotrophy, brachial neuritis, brachial plexus neuropathy, and brachial plexitis.

Parsonage-Turner syndrome involves neuropathy of the suprascapular nerve in 97% of cases, and can involve the axillary and subscapular nerves. As such, the supraspinatus and infraspinatus muscles are usually involved, both of which are innervated by the suprascapular nerve. The acute phase can last a few hours to a few weeks, and is followed by wasting and weakness of the muscles (amyotrophy) in the affected areas. The syndrome is idiopathic, although an autoimmune etiology has been hypothesized and many specific risk factors have been identified, such as a patient's state after an operation, infection, trauma, or vaccination. The severity of the disorder can vary widely from one person to another, in part because of the specific nerves involved. Some affected people recover without treatment—strength returns to the affected muscles and the pain goes away—but episodes can recur. Other affected people experience residual pain and potentially significant disability. Treatment is usually conservative, with pain medications and physical therapy, but, at times, steroids are used.

Final Verdict

When "things fall from the sky at the same time," they are likely connected in some way. One hypothesis is that all of these problems are in some way derived from the patient's relapsing polychondritis, so treatment should be aimed at controlling the underlying systemic disorder that can present as a vasculitic problem. Another very reasonable hypothesis is that the patient developed a parvovirus-triggered brachial neuritis after becoming infected with parvovirus B19. I favor the latter, mainly because the patient seems to be getting better on her own and, if this was a vasculitic problem, steroids would have been necessary.

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