Scleroderma (Systemic Sclerosis)
Scleroderma is a systemic autoimmune disease characterized by excessive collagen deposition, leading to fibrosis of the skin and internal organs.
Key Clinical Features
- Sclerodactyly: Tightening and thickening of the skin on the fingers, leading to a “claw-like” hand deformity.
- Raynaud Phenomenon: Often the first symptom; cold/stress-induced vasospasm causing color changes (white to blue to red).
- Facial Changes: “Mask-like” facies with microstomia (small mouth) and “purse-string” radial perioral furrows.
- Telangiectasias: Dilated superficial blood vessels, particularly on the face and palms.
CREST Syndrome (Limited Scleroderma)
| Letter | Feature |
|---|---|
| C | Calcinosis cutis |
| R | Raynaud phenomenon |
| E | Esophageal dysmotility |
| S | Sclerodactyly |
| T | Telangiectasia |
High-Yield Exam Pearl:
- Antibody Associations: Anti-centromere antibodies are highly specific for Limited Scleroderma (CREST). Anti-Scl-70 (Anti-topoisomerase I) is associated with Diffuse Systemic Sclerosis and higher risk of pulmonary fibrosis.
- Renal Crisis: A medical emergency characterized by sudden-onset severe hypertension and acute kidney injury; often triggered by corticosteroid use in systemic sclerosis patients.