Named Signs in Systemic Sclerosis
The classic named signs in systemic sclerosis include the CREST syndrome acronym (Calcinosis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias), which historically described limited cutaneous systemic sclerosis, along with several other eponymous findings including tendon friction rubs and the modified Rodnan skin score assessment. 1, 2
CREST Syndrome Components
The CREST acronym represents five cardinal features traditionally associated with limited cutaneous systemic sclerosis (lcSSc): 2, 3
Calcinosis - Calcium deposits in subcutaneous tissues, particularly in fingers and pressure points, which has no proven medical therapy and may require surgical intervention for symptomatic cases 2
Raynaud phenomenon - Present in over 95% of SSc patients, characterized by episodic vasospasm causing color changes in digits 1
Esophageal dysmotility - The esophagus is the most commonly involved gastrointestinal organ, with nearly 90% of SSc patients experiencing GI involvement 1
Sclerodactyly - Skin thickening and tightening of the fingers and hands, a hallmark cutaneous manifestation 4
Telangiectasias - Dilated superficial blood vessels visible on skin and mucous membranes 4
Additional Named Clinical Findings
Tendon Friction Rubs
- Palpable or audible creaking sensation over tendons, particularly at wrists, knees, and ankles 1
- Indicates more aggressive disease and is a risk factor for scleroderma renal crisis 1
- Associated with rapidly progressive skin involvement 1
Modified Rodnan Skin Score (mRSS)
- A standardized assessment tool measuring skin thickness on a 0-3 scale at 17 anatomical sites (total range 0-51) 1
- Used to quantify and monitor skin involvement over time 1
- In diffuse cutaneous SSc, skin thickening typically increases over the first 4 years and may regress thereafter 1
Puffy Fingers
- Early manifestation of "prescleroderma" presenting as non-pitting edema of digits 1
- Patients with this finding should undergo nailfold capillaroscopy 5
Cardiac Examination Findings
- Pericardial rub - Audible friction sound indicating pericardial inflammation 1
- Muffled heart sounds - May indicate pericardial effusion 1
Capillaroscopic Patterns
While not a "named sign" in the traditional sense, nailfold capillaroscopy reveals characteristic patterns: 5
- Capillary loss, dilation, and hemorrhages visible on magnified examination 5
- Included in the 2013 EULAR/ACR classification criteria for systemic sclerosis 5
- Can be performed using otoscope, ophthalmoscope, dermatoscope, or formal capillaroscopy 5
Clinical Context and Pitfalls
The CREST acronym, while historically useful, is somewhat outdated terminology - the condition is now more accurately termed limited cutaneous systemic sclerosis (lcSSc), as patients may not exhibit all five features and the acronym oversimplifies disease heterogeneity. 2, 3
Limited cutaneous disease typically has a slower pace with long-standing Raynaud phenomenon before other symptoms emerge, whereas diffuse cutaneous SSc presents more acutely with constitutional symptoms and rapid progression. 3
Despite being considered a "milder" form, lcSSc can still cause life-threatening complications including pulmonary arterial hypertension and small intestine hypomotility leading to malnutrition. 3