BMJ 2018;361:k1590 doi: 10.1136/bmj.

k1590 (Published 10 May 2018) Page 1 of 2

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SPOT DIAGNOSIS

Dysphagia and a rash

Michael McFarlane gastroenterology SpR,, Ben Disney consultant gastroenterologist
University Hospital Coventry and Warwickshire, Coventry, UK

A 58 year old man presented with a six week history of muscle

weakness, dysphagia, and a symmetrical widespread rash on
the face, torso, limbs, and hands (fig 1). His creatine kinase was
7070 u/L (reference range 30-175). An
oesophagogastroduodenoscopy showed abnormal findings, and
a positron emission computed tomography (PET CT) scan was
arranged (fig 2). What are this man’s dermatological and
oesophageal diagnoses?

Correspondence to M McFarlane mmcf1982@doctors.org.uk

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BMJ 2018;361:k1590 doi: 10.1136/bmj.k1590 (Published 10 May 2018)
Dorsum of the patient’s right hand
PET CT image
Answer
Gottren’s papules (fig 1) suggest acute dermatomyositis, and
the high uptake of fluorodeoxyglucose in the mid oesophagus
(fig 2) suggests oesophageal cancer.
Discussion
Dermatomyositis is an acute inflammatory polymyopathy
affecting striated muscle, which occurs in the presence of skin
symptoms. It differs from polymyositis, which has the myopathy
component with no skin symptoms. Both diseases are
characterised by the acute or subacute onset of symmetrical
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Page 2 of 2
ENDGAMES
proximal muscle weakness, mononuclear cell infiltrates in
muscles, and increased levels of muscle enzymes such as
creatine kinase.
Oesophageal cancer is a rare cause of dysphagia in
polymyositis/dermatomyositis. However, dysphagia and
abnormal motility can be common symptoms of dermatomyositis
if the smooth and striated muscles of the upper gastrointestinal
tract are involved.
Dermatomyositis and polymyositis increase the likelihood of
developing cancer by six and twofold, respectively,1 making
appropriate investigation a priority, especially in patients with
relevant risk factors.2
Risk factors for developing cancer in
polymyositis/dermatomyositis are increasing age, male sex,
dysphagia, skin necrosis, cutaneous vasculitis, rapid disease
onset, raised creatine kinase, raised C reactive protein, and
increased erythrocyte sedimentation rate.
Sometimes, the diagnosis of cancer predates the diagnosis of
dermatomyositis/polymyositis.2 In these cases, the highest risk
of developing dermatomyositis/polymyositis is in the first year
after the cancer diagnosis, suggesting that
dermatomyositis/polymyositis can develop as a paraneoplastic
process (an aberrant immune response to the presence of
malignancy).2
The cancers most commonly associated with
dermatomyositis/polymyositis in Europe and the USA are breast,
lung, and colorectal. In southern China and south-east Asia,
nasopharyngeal cancers are most common. However, any cancer
can cause a dermatomyositis/polymyositis paraneoplastic
syndrome.1
The treatment of dermatomyositis/polymyositis is glucocorticoid
steroids and disease modifying drugs.1
Patient outcome
The patient was presented at the upper gastrointestinal
multidisciplinary team meeting and the malignant lesion was
investigated further by staging computed tomography and further
evaluation of the PET-CT scan.
Learning points
1. Patients with dermatomyositis have a sixfold higher risk
of developing cancer and there should be a low threshold
for investigation.1
2. Risk factors for malignancy in
dermatomyositis/polymyositis are increasing age, male sex,
dysphagia, skin necrosis, cutaneous vasculitis, rapid disease
onset, and raised creatine kinase, C reactive protein, and
erythrocyte sedimentation rate.
Patient consent not obtained. Patient died 10 years ago. Next of kin have since
moved from the area so cannot be contacted.
Competing interests: the authors have no competing interests to declare.
Provenance and peer review: not commissioned; externally peer reviewed.
1 Jakubaszek M, Kwiatkowska B, Maślińska M. Polymyositis and dermatomyositis as a risk
of developing cancer. Reumatologia 2015;53:101-5. 10.5114/reum.2015.51510 27407235
2 Ungprasert P, Bethina NK, Jones CH. Malignancy and idiopathic inflammatory myopathies.
N Am J Med Sci 2013;5:569-72. 10.4103/1947-2714.120788 24350067
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