Scleroderma

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. Raynaud’s phenomenon, localized morphea, local or generalized oedema, hyperpigmentation, telangiectasia, subcutaneous calcification, ulceration, particularly at the fingertips. Ten-year survival rate is 71% with skin tightness limited to fingers but 21% with diffuse truncal skin involvement. Ilioprost, a prostacyclin analogue, helps to heal digital ulceration. Penicillamine improves the skin and prolongs survival in patients with early, rapidly progressive, systemic sclerosis.


Musculoskeletal system. Arthritis, myositis, myopathy, bone ischaemia with resorption of the phalanges.


GI tract. Dysphagia, reflux oesophagitis, large or small bowel obstruction.


Lung. Fibrosis, atelectasis, pulmonary hypertension; pneumonia.


Kidney. Glomerulonephritis, malignant hypertension (poorest prognosis with renal involvement). ACE inhibitors dramatically improve renal crisis.


Heart. Myocardial fibrosis.

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Dec 4, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Scleroderma

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