What is Sclerodactyly?

Sclerodactyly is a condition in which the skin gradually hardens and becomes rigid. It typically affects the skin surrounding the hands and fingers. The condition does not usually develop on its own, but is generally a symptom of a rare disorder known as scleroderma.

Scleroderma, also referred to as scleriasis, is a type of disorder that causes tightening of internal organs, connective tissues, and skin. There are two main varieties of scleroderma: systemic and localized. Systemic scleroderma is more likely to cause hardening of internal organs and connective tissues, while localized scleroderma tends to occur on the skin only and result in sclerodactyly.

The main cause of sclerodactyly is collagen, a naturally occurring protein in the body that comprises the skin and connective tissues. Collagen has a rigid texture, similar to a very firm rubber. If the body makes too much collagen, it can build up and make the skin feel stiff and inflexible. It is usually the most noticeable in the hands and fingers because it prevents them from being able to bend properly.

When sclerodactyly first starts to develop, a person may notice his or her fingers start to swell, but not subside over time. As the condition progresses, a person’s fingers and hands may seem hard to the touch and have a shiny appearance. In the most severe cases, the hard texture of the skin on the fingers and hands may result in a person having difficulty moving or bending his or her fingers and hands.

No proven treatment exists to prevent the body from making the excessive collagen that causes scleroderma to form. The disorder is progressive, meaning it gradually gets more severe over time. A person with localized scleroderma and the resulting sclerodactyly will generally participate in physical therapy to learn how to deal with his or her decreased ability to use his or her fingers or hands. Flexible hand molds may be worn over the affected hands like gloves, which can enable a person with the condition to feel less self-conscious.

Sclerodactyly can have potential serious health complications. If the amount of collagen in the skin becomes high enough, it can prevent proper blood flow to the hands and fingers. An insufficient amount of blood flow to the hands and fingers can result in tissue damage. This tissue damage can cause gangrene, or decay of the tissue, which will often require the affected areas to be amputated.