Dermatomyositis

 

Thank you for asking me to examine Harry who presented with weakness/hand lesions etc.

 

The most salient findings are symmetrical proximal muscle weakness with characteristic cutaneous features suggesting of dermatomyositis with/without evidence of complicating pulmonary fibrosis. I could/could not appreciate any underlying malignancy.

 

In further detail...

 

On general inspection, Harry was comfortable and had no Cushingoid features, and was not cachectic.  There were/were not any gait aids.

 

On inspection of the hands, there were erythematous plaques on the extensor surface of the fingers, consistent with Gottren’s papules, as well as mechanic hands, and nailfold capillaries. These were associated with the characteristic heliotropic rash of dermatomyositis, as well as poikiloderma (hyperpigmentation and telangiectasias - Shawl’s sign) on the back.

 

On palpation, there was not evidence of active synovitis.

 

There was / was not restriction in movement of the PIP, MCP, wrist flexion/extension and thumb movements.

 

Function was impaired / intact with/without deficits in grip strength, pincer grip, and opposition strength, and no difficulty / difficulty in opening a jar and undoing a button.

 

In the upper limbs, there was/was not muscle wasting. Tone was normal. There was muscle tenderness. There was a symmetrical weakness in the proximal muscles. Reflexes were intact (reflexes can be intact or reduced). Sensation was normal.

 

This was/was not associated with proximal weakness in the legs.

 

On further examination:

 

There was a heliotropic rash on the face, and shawl’s sign on the back. There was/was not evidence of bulbar dysfunction.

 

In regard to the cardiorespiratory system, chest expansion was normal/reduced. There was/was not evidence of pulmonary fibrosis and pulmonary hypertension.

 

There was/was not any lymphadenopathy or abdominal masses suggestive of a underlying cancer.

 

There was / was not signs of steroid complications, such as a Cushingoid appearance, bruising, skin atrophy, cataracts, or proximal myopathy. 

 

In summary, my findings are in keeping with dermatomyositis with proximal myopathy without evidence of pulmonary fibrosis or an underlying malignancy.

 

My differentials include:

• Other inflammatory myopathies - inclusion body and immune mediated necrotizing myopathy

• Acquired myopathies

  • Alcohol

  • Paraneoplastic

  • Endocrine - thyroid, Cushing’s

  • Drugs - steroids

• Hereditary muscular dystrophies - Becker’s, myotonic dystrophy

• NMJ

• Anterior horn cell - motor neuron disease, and spinal muscular atrophy (Kugelberg-Welander disease).

 

I would proceed from here by:

• Confirming my diagnosis with investigations including myositis antibodies and a biopsy

• Assess for suspected complications and screen for others with:

  • RFTs, HRCT - ILD

  • TTE - pulmonary hypertension

  • Age appropriate malignancy screening

  • Steroid - BMD, HbA1c, lipids

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