C. Limited systemic sclerosis – Explanation
This is not a clear cut question and the most likely diagnosis here is limited
systemicsclerosis.
Both limited and diffuse systemic sclerosis may present with pain in hands
whenexposed to cold (Raynaud’s phenomena). The classical features of
Raynaud’sphenomenon are episodic pallor of the digits due to ischaemia, followed by
cyanosisdue to deoxygenation and then followed by redness with pain and tingling
which iswhen there is reperfusion.
The presence of the pink and red dots on the lips and tips of fingers
representtelangiectasia which is seen as part of the CREST syndrome in limited
systemic sclerosis.These are visibly dilated blood vessels that usually appear on the
fingers, palms, face,lips and chest.
Arthralgia is usually seen more in diffuse systemic sclerosis which goes against
theanswer of limited systemic sclerosis. In limited systemic sclerosis, the complaint
isusually the occasional joint stiffness rather than arthralgia. However, given the
slowprogression of the disease and no other features of organ involvement,
limitedsystemic sclerosis is likely the answer.
Rheumatoid arthritis is also a potential differential because it is another cause
ofRaynaud’s phenomenon and would explain the symmetrical peripheral
arthropathy.Nonetheless, rheumatoid arthritis is a rare cause of Raynaud’s
phenomenon and aneven rarer cause of telangiectasia.
Given the stem, the best answer still remains as limited systemic sclerosis
LIMITED SCLERODERMA VS DIFFUSE SCLERODERMA
| Limited Scleroderma – CREST Syndrome |
Diffuse Scleroderma | |
|---|---|---|
| Skin involvement | Limited areas of skin are thick; usually just the fingers and/or face. Skin involvement does not extend above the elbow or above the knee No trunk involvement |
More areas of the skin are involved and thickened. Skin of the arms, legs, and trunk is more more likely to be involved |
| Onset | Slow onset | Rapid onset |
| Progression | Slow progression | Fast progression |
| Length of skin changes from Raynaud’s phenomenon |
Raynaud’s phenomenon for many years before any skin changes |
Skin changes within 1 year of Raynaud’s |
| Organ involvement | Milder form – Less involvement of body organs Lung involvement can be seen but usually less common and more mild |
More severe – involvement of internal organs such as the gastrointestinal tract, heart, lungs, or kidneys |
| Others | CREST | The skin can lose or gain pigment, making areas of light or dark skin |
| Antibodies | Most have positive ANA Antibodies to Scl-70 are usually negative |
Most have positive ANA Antibodies to Scl-70 are usually positive (up to 60% of patients) Anti-Scl 70 is strongly associated with lung fibrosis and with renal disease and a poor prognosis. |
