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A WOMAN WITH PAIN AND STIFFNESS OF HANDS

Esha Das Gupta FRCP, International Medical University, Seremban, Malaysia

Address for correspondence: Associate Professor Esha Das Gupta, International Medical University, Jalan Rasah, 70300 Seremban, Negeri Sembilan Darul Khusus, Malaysia. Tel: 06-7677798, Fax: 06-7677709, Email: eshadas_gupta@imu.edu.my

Das Gupta E. A woman with pain and stiffness of hands. Malaysian Family Physician. 2008;3(3):xx-xx

This 45 years old house wife presented with pain and stiffness of her hands. The symptoms started 14 yrs ago. She noticed the pain increases when she is in an air-conditioned room when her fingers become white temporarily. Recently she is having frequent ‘indigestion’ and vomiting. Below are the pictures of her hands.

Questions

1. Describe the features shown in the above picture of hands.
2. What is your diagnosis?
3. What would be x-ray appearance of these hands?

Answers

1. There istightening and thinning of skin over the knuckles, fingers and finger joints causing the appearance of ‘sclerodactyly’ and curving of the digits. There is hypopigmentation of the overlying skin. There is presence of pseudo-clubbing in both thumbs and index finger due to resorption of terminal part of the phalanx. There is suggestion of Raynaud’s phenomenon in Figure 2.
2. Scleroderma. Scleroderma is a chronic, degenerative, autoimmune disorder that leads to the over-production of collagen in the body's connective tissue. The word "scleroderma" means "hardening of the skin" and refers to one of the possible physical effects of the disease. If systemic (throughout the body), scleroderma is known as progressive systemic sclerosis. Raynaud's phenomenon is the most common presentation of scleroderma. Typical attacks are characterised by well demarcated blanching of the fingers. As the fingers recover their blood supply, they become blue and finally red. There are two forms of scleroderma: localized and generalized (also called systemic sclerosis).
1. Limited scleroderma affects mainly the skin in different areas of the body. It affects mainly the skin in different areas of the body, may affect muscles and bone but does not usually affect internal organs. CREST is an acronym derived from the syndrome's five most prominent symptoms:
   • C – Calcinosis
   • R – Raynaud’s phenomenon
   • E – Esophageal dysfunction
   • S – Sclerodactyly, tightening of the skin on the fingers and toes
   • T – Telengiectasia, presence of red spots on the hands, palms, forearms, face and lips
2. Generalized scleroderma affects the skin and/or internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints.
3. X-ray may show sub-cutaneous calcification as a part of the CREST syndrome (Figure 3).

figure 3

Further reading:

1. Hinchcliff M, Varga J. Systemic sclerosis/scleroderma: a treatable multisystem disease. Am Fam Physician. 2008;78(8):961-8 [PubMed]
2. Li Q, Sahhar J, Littlejohn G. Red flags in scleroderma. Aust Fam Physician. 2008;37(10):831-4 [PubMed] [Full text]
3. Margolis M, McLennan MK. Radiology rounds. Scleroderma. Can Fam Physician. 1994;40: 667, 671-4 [PubMed] [Full text]
4. Eschenbach S, Pope TL Jr. Scleroderma with calcinosis. Appl Radiol. 2007;36(3) [Full text]