Examine  the clinical photograph.

a)  List the abnormalities visible in this patient’s hands.

b)  What is the most likely diagnosis?

c)  List 4 other clinical features associated  with this condition?

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College Answer

a) 
•    Evidence of Raynaud’s or ischaemia with pallor of digits,
•     Sclerodactaly 
•     Calcinosis, 
•    Multiple amputations of distal digits
•    Distal ulceration with gangrene
•    Fixed flexion deformity of ring finger right hand.

b)     Systemic sclerosis or Scleroderma

c) 
•    Telangiectasia, 
•    Fine creps bibasally due to lower lobe fibrosis,
•    Beaking of the nose
•    Limitation of mouth opening
•    Thickening of the skin and pigmentation changes
•    Features consistent with Pulmonary H/T,
•    Sjogrens syndrome
•    Oesophageal involvement
•    Renal failure

Discussion

Scleroderma is also the subject of Question 28 from the first paper of 2012, but the information requested there is rather different.

Who cares if the hands don't demonstrate all the signs in the college answer? The cutaneous manifestations of scleroderma are as follows:

  • Skin thickening
  • Oedema of the digits
  • Sclerodactyly
  • Pitting ulceration, particularly at the fingertip
  • Calcinosis over joints
  • Telangiectasia
  • Raynauds phenomenon

Other clinical features:

  • Sjogren syndrome
  • difficult intubation (limited mouth opening)
  • Pulmonary fibrosis, restrictive lung disease, pulmonary hypertension
  • Cardiac arrhythmias, myocardial fibrosis, pericardial stricture
  • Corticosteroid-associated psychosis; chronic fatigue
  • hyponatremia and fluid retention due to corticosteroid therapy
  • renal failure, renal artery stenosis
  • Oesophagitis, poor gut motility, decreased feed tolerance, risk of aspiration
  • Difficult vascular access
  • Immunosuppressive therapy leading to increased infection risk

References

References

Farber, Harrison W., Robert W. Simms, and Robert Lafyatis. "Analytic Review: Care of Patients With Scleroderma in the Intensive Care Setting." Journal of intensive care medicine 25.5 (2010): 247-258.

Legerton 3rd, C. W., Edwin A. Smith, and Richard M. Silver. "Systemic sclerosis (scleroderma). Clinical management of its major complications."Rheumatic diseases clinics of North America 21.1 (1995): 203-216.

TUFFANELLI, DENNY L., and R. K. Winkelmann. "Systemic scleroderma: a clinical study of 727 cases." Archives of Dermatology 84.3 (1961): 359-371.

Silver, Richard M. "Clinical aspects of systemic sclerosis (scleroderma)." Ann Rheum Dis 50.suppl 4 (1991): 854-61.

In order to replace the college photograph, I have had to misappropriate a series of images from various sites:

The first set of (ulcerated) hands are from Health in Plain English;

The hands with digital pallor and telangiectasia are from... I can't remember where. Google?

The final set of digits with amputations and calcinosis are from Pathguy. Thank you, Pathguy!