VA AM Report: Dermatomyositis!

Kresh presented a great case of a patient presenting with dysphagia who turned out to have . . . dermatomyositis! So many pearls to pluck out of this oyster of a case . . .

First, dysphagia remember: try to pinpoint whether oropharyngeal or esophageal (is food getting stuck? or difficulty with swallowing?) and whether solids or liquids. The patient also had double vision, remember monocular (isolated to one eye) double vision usually indicates an error of refraction and is an ophthalmologic problem, while binocular diploplia indicates a neurologic problem. Ask whether the images appear side by side or on top of one another to focus on which cranial nerve is indicated.

Bonus pearl: A CN III palsy is a ruptured Pcomm aneurysm until proven otherwise, look for dilated non-reactive pupils as well, generally pupils are spared in microvascular (diabetes) causes of CN III palsy.

Dermatomyositis

-Can be diagnosed clinically if a heliotrope rash or gottron’s papules are present along with demonstrable proximal muscle weakness

-However the presence of positive antibodies (anti-Jo, anti-RNP, anti-Mi2) along with clinical findings of reynaud’s, mechanic’s hands, arthritis, or ILD (as in this case, pt had dx of NSIP three months prior) comprises the “anti-synthetase syndrome,” which portends a worse prognosis

-Remember the association with malignancy, in addition to age-appropriate cancer screening consider pelvic ultrasound in women and CT abdomen/pelvis in patients with extensive skin involvement especially necrosis, the absence of ILD, older age at onset, history of malignancy, or lack of response to treatment (steroids, cyclophosphamide)

Lots more here but that’s enough for today, remember the association w/ ILD (NSIP, IPF) and the need for close f/u with malignancy.

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