Scleroderma

Published on 02/04/2015 by admin

Filed under Internal Medicine

Last modified 02/04/2015

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149 Scleroderma

Instruction

Look at these hands.

Salient features

History

• Tight skin over face and joints

• Raynaud’s phenomenon (Case 161, p. 587)

• Puffy hands and feet

• Fatigue

• Shortness of breath (lung involvement, cardiac fibrosis), dry cough

• GI symptoms: dysphagia, diarrhoea, bloating and indigestion

• Dry eyes

• History of renal failure.

Examination

• Thickening and tightening of the skin over fingers, sclerodactyly (finger pulp atrophy) (Fig. 149.1), beaking of nails (pseudoclubbing), atrophic nails, telangiectasia (nailfold capillaries) (Fig. 149.2)

• Raynaud’s phenomenon (see Fig. 161.1)

• Subcutaneous calcification (fingers, elbows and extensor aspect of the forearms)

• Vitiligo or pigmentation.

image

Fig. 149.1 Hands in scleroderma.

image

Fig. 149.2 Digital ulcers.

(With permission from Charles et al. 2006.)

Proceed as follows:

• Assess hand function: pincer movements, handgrip, unbuttoning of clothes, abduction of thumb and writing.

• Examine:

• the joints for arthalgia or arthritis
• the face for microstomia, difficulty in opening the mouth, beak-like or pinched appearance of the nose, blotchy telangiectasia
• the abdomen for liver (primary biliary cirrhosis).

Diagnosis

This patient has sclerodactyly, tightening of the skin over hands and face (lesion) caused by scleroderma (aetiology). He has difficulty in buttoning his clothes and has marked dysphagia as a result of oesophageal involvement (functional status).

Questions

What other organ systems are involved?

Skin

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