Sclerodactyly is the thickening and tightening of skin on the fingers and sometimes the toes. It is a symptom, not a standalone disease, leading to restricted movement in affected digits.
Understanding Sclerodactyly
The skin often appears shiny, taut, and hardened. This tightening can make bending fingers challenging, causing them to curl inward, forming a claw-like shape. Fingertips may also taper, and underlying soft tissue and muscles can waste.
Before skin hardens, individuals might notice swelling or puffiness in hands and fingers. Progressive skin stiffness leads to difficulty moving fingers or toes. Reduced mobility impacts daily activities, making tasks like grasping small objects or making a fist difficult. Grip strength can also be reduced, affecting tasks such as opening jars or carrying items.
The Underlying Conditions
Sclerodactyly is most often a symptom of systemic sclerosis, also known as scleroderma. This autoimmune disease causes the immune system to attack healthy tissues. This leads to collagen overproduction, a protein providing skin strength and elasticity, making skin hard and leathery.
Beyond the skin, systemic sclerosis can affect various internal organs and systems, including blood vessels, the digestive tract, lungs, kidneys, heart, joints, and muscles. Sclerodactyly indicates a broader systemic issue.
While systemic sclerosis is the most common association, sclerodactyly can also occur in rarer conditions. It is a component of CREST syndrome, a limited form of systemic sclerosis. Other conditions like Huriez Syndrome, mixed connective tissue disease, and diabetic sclerodactyly can also cause digital skin hardening.
Diagnosis and Management
Diagnosing sclerodactyly and its cause involves physical examination and medical history review. Healthcare professionals look for skin changes and assess finger range of motion. Blood tests check for autoantibodies like antinuclear antibodies (ANA) or specific scleroderma-related antibodies (anti-centromere, anti-Scl-70, anti-RNA polymerase III), indicating autoimmune conditions.
Nailfold capillaroscopy, a non-invasive technique visualizing tiny blood vessels at the fingernail base. It reveals abnormalities like enlarged capillaries, hemorrhages, or reduced vessel density, characteristic of microvascular changes in systemic sclerosis. Additional tests, such as lung function tests, may assess internal organ involvement.
No cure exists for underlying conditions causing sclerodactyly; management focuses on alleviating symptoms and slowing disease progression. Physical therapy plays a key role, with hand exercises and stretching to maintain flexibility and range of motion. Specialized casts or splints may prevent severe contractures, and therapies like hot wax treatments can reduce pain and stiffness.
Occupational therapy assists individuals in adapting to daily limitations, recommending assistive devices. Lifestyle adjustments, including regular activity and diligent skin care, help manage the condition. Medications that dilate blood vessels or suppress the immune system may be prescribed for systemic symptoms. Ultraviolet light therapy or surgical interventions may also improve hand function and alleviate discomfort.