Sclerodactyly is a localized thickening and tightness of the skin of the

finger A finger is a limb of the body and a type of digit, an organ of manipulation and sensation found in the hands of most of the Tetrapods, so also with humans and other primates. Most land vertebrates have five fingers ( Pentadactyly). Chambers ...

s or

toe Toes are the digits (fingers) of the foot of a tetrapod. Animal species such as cats that walk on their toes are described as being ''digitigrade''. Humans, and other animals that walk on the soles of their feet, are described as being ''plan ...

s that yields a characteristic claw-like appearance and spindle shape of the affected digits, and renders them immobile or of limited mobility. The thickened, discolored patches of skin are called morphea, and may involve connective tissue below the skin, as well as muscle and other tissues. Sclerodactyly is often preceded by months or even years by

Raynaud's phenomenon Raynaud syndrome, also known as Raynaud's phenomenon, eponymously named after the physician Auguste Gabriel Maurice Raynaud, who first described it in his doctoral thesis in 1862, is a medical condition in which the spasm of small arteries cau ...

when it is part of systemic scleroderma. The term "sclerodactyly" comes from the combination of the

Greek Greek may refer to: Greece Anything of, from, or related to Greece, a country in Southern Europe: *Greeks, an ethnic group. *Greek language, a branch of the Indo-European language family. **Proto-Greek language, the assumed last common ancestor ...

words "skleros" meaning hard and "daktylos" meaning a finger or toe – "hard fingers or toes". It is generally associated with systemic

scleroderma Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas of ...

and

mixed connective tissue disease Mixed connective tissue disease, commonly abbreviated as MCTD, is an autoimmune disease characterized by the presence of elevated blood levels of a specific autoantibody, now called anti-U1 ribonucleoprotein (RNP) together with a mix of symptoms of ...

, and

auto-immune disorder An autoimmune disease is a condition arising from an abnormal immune response to a functioning body part. At least 80 types of autoimmune diseases have been identified, with some evidence suggesting that there may be more than 100 types. Nearly a ...

s. Sclerodactyly is one component of the limited cutaneous form of systemic sclerosis (lcSSc), also known as CREST syndrome (CREST is an acronym that stands for

calcinosis Calcinosis is the formation of calcium deposits in any soft tissue. It is a rare condition that has many different causes. These range from infection and injury to systemic diseases like kidney failure. Types Dystrophic calcification The most c ...

esophageal dysmotility An esophageal motility disorder (EMD) is any medical disorder causing difficulty in swallowing, regurgitation of food and a spasm-type pain which can be brought on by an allergic reaction to certain foods. The most prominent one is dysphagia. E ...

, sclerodactyly, and

telangiectasia Telangiectasias, also known as spider veins, are small dilated blood vessels that can occur near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. These dilated blood vessels can develop anywhere on ...

.) Sclerodactyly is also one component of Huriez Syndrome, along with palmoplantar keratoderma and skin cancer. Sclerodactyly sometimes arises as a complication of the microvascular changes seen in

diabetes mellitus Diabetes, also known as diabetes mellitus, is a group of metabolic disorders characterized by a high blood sugar level ( hyperglycemia) over a prolonged period of time. Symptoms often include frequent urination, increased thirst and increased ap ...

, and is in this case referred to as diabetic sclerodactyly. Treatment of sclerodactyly is by physical therapy, phototherapy, surgery, topical corticosteroids or vitamin D analogues, and systemic immunosuppressive drugs when the condition is part of systemic scleroderma. Localized treatment won't halt systemic disease, but can restore function and cosmetic aspects of the affected digits. The mild to moderate proximal interphalangeal (PIP) joint flexion and extension contractures and stiff distal interphalangeal (DIP) joints in slight flexion often seen in sclerodactyly can be addressed somewhat with physical therapy. In a few cases when immune involvement isn't apparent (in these cases environmental causes are suspected), the condition may gradually clear up by itself If the trigger is avoided. In other cases, early treatment while the disease is in the inflammatory stage is much more likely to be successful than on established lesions.

References

Cutaneous conditions {{cutaneous-condition-stub

See also

References