Scleroderma is an autoimmune disease characterized by patches of scar-like thick skin caused by the build-up of collagen in the area between cells, called the stroma.
Sclerodactyly is the thickening and hardening of the fingers, and sometimes toes, as a result of excessive collagen buildup in the skin. It is among the five symptoms that mark limited scleroderma, also known as CREST syndrome. (CREST stands for calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias).
How sclerodactyly develops
Signs of sclerodactyly usually begin with swelling in the fingers. The skin feels tight, gets thicker and harder, and appears shiny.
Later phases of sclerodactyly may involve skin discoloration and limited ability to move the fingers.
In advanced stages, it may cause sores on the skin, and the fingers may curl inwards and remain fixed in a clawed shape.
How sclerodactyly is treated
There are several treatment approaches that aim to manage the symptoms of sclerodactyly. It is very important that patients begin a treatment as early as possible.
Medications such as corticosteroids, nonsteroidal anti-inflammatory medicines, D-penicillamine, cyclosporine, IFN-gamma, and cytostatics can help reduce the pain and lessen skin changes.
Hand exercises instructed by a specialized physical therapist can reduce the swelling and skin tightness.
Hand casts, which keep the hands in a less restrictive position than a clawed shape, may benefit some patients with advancing sclerodactyly.
Ultraviolet (UV) light therapy (phototherapy) can stimulate the production of a molecule that works to breakup excess collagen and suppresses the immune system.
Finally, surgery may provide some relief of symptoms and hand limitations for some patients with sclerodactyly.
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