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#52: Relapsing Polychondritis (RP)
It is not
surprising that Danny Dermorama has been called a gentle giant: he never raises
his voice, rarely appears annoyed and experiences almost no conflict in his
life, perhaps as a result of his size. Not surprisingly, he and his wife were
both mildly incredulous when he was diagnosed, at first, with “Boxer’s ear”
after showing your locum his swollen, painful pinna. He could recall no trauma
to the area, let alone multiple blows from someone’s fist, and although he did
“ease up on his diabetes” with a “Campari and soda or two” and a “couple of
sfogliatelles from Moio’s” during the World Cup, he has kept both his drinking
and his diabetes under excellent control for more than a year now. He chuckles
as he tells you about the all the diagnoses he was offered while you were away,
which included staphylococcal infection, pseudomonal infection, and bilateral
cellulitis. He has had no improvement despite parenteral cepazolin and
ciprofloxacin and the “infection” has now spread to both ears. Luckily,
refreshed from your time away, you immediately recognize the condition and your
treatment provides rapid relief.
eMedicine has a great article on relapsing
polychondritis that can be easily googled and from which the following excerpts
have been taken.
What is this?
Relapsing polychondritis (RP)
is a severe, episodic, and progressive inflammatory condition involving
cartilaginous structures, predominantly those of the ears, nose, and
laryngotracheobronchial tree. Other affected structures may include the eyes,
cardiovascular system, peripheral joints, skin, middle and inner ear, and CNS.
Signs and symptoms of RP include the following. Auricular chondritis: Of
patients with RP, 85-95% develop auricular chondritis, typically with the sudden
onset of unilateral or bilateral ear (auricle) pain, swelling, and redness,
sparing the lobules. The pain and redness usually resolve within 2-4 weeks but
may recur. The ear cartilage softens and collapses forward. The external
auditory canal can collapse after 1 or more episodes. If the damage is less,
nodularity of the auricle may develop. Calcification occurs in 40% of patients.
What was your treatment?
Prednisone. A rapid response is one of the
diagnostic criteria for RP.
What tests might you order?
Because RP is associated with many multisystemic diseases, a
laboratory evaluation commensurate with the spectrum of reported symptoms is
indicated to ascertain the presence of complicating conditions.
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Use antinuclear antibody reflexive panel,
rheumatoid factor, and antiphospholipid antibodies (if history of thrombosis
is found) for evaluation of other autoimmune connective tissue diseases.
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For a vasculitis workup, perform a CBC
count with differential; metabolic panel; creatinine, liver transaminase,
and serum alkaline phosphatase levels; urinalysis dipstick and microscopic
evaluation of sediment; cryoglobulins; viral hepatitis panel; and
antineutrophil cytoplasmic antibody (ANCA) tests (eg, cytoplasmic ANCA,
perinuclear ANCA, antimyeloperoxidase and antiproteinase 3 antibody titers).
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Use the purified protein derivative test
for exposure to tuberculosis (tuberculosis often is overlooked as an
infectious cause of perichondritis).
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Use serologic tests for syphilis if it is
suspected, including rapid plasma reagent or VDRL testing. Saddle-nose
deformity is a clinical manifestation of congenital syphilis and can go
undiagnosed into adulthood; however, it can also be a consequence of gumma
formation in adulthood.
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Cultures may be indicated, depending on
the clinical presentation.
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Sputum
cultures for bacteria and acid-fast bacilli may be needed for patients
with respiratory complaints.
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Bacterial, acid-fast bacilli, and fungal cultures may be appropriate for
cartilage biopsy samples, especially from the respiratory tree.
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Blood
cultures may be useful in the assessment of febrile episodes that are
combined with nausea, vertigo, and/or muscle weakness.
Special Bonus Question:
What is this man looking at?
A snake.
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