Sclerodactyly is a localized
thickening and tightness of the
skin
Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation.
Other animal coverings, such as the arthropod exoskeleton, have different ...
of the
finger
A finger is a prominent digit (anatomy), digit on the forelimbs of most tetrapod vertebrate animals, especially those with prehensile extremities (i.e. hands) such as humans and other primates. Most tetrapods have five digits (dactyly, pentadact ...
s or
toes 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
Connective tissue is one of the four primary types of animal tissue, a group of cells that are similar in structure, along with epithelial tissue, muscle tissue, and nervous tissue. It develops mostly from the mesenchyme, derived from the mesod ...
below the skin, as well as
muscle
Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...
and other tissues. Sclerodactyly is often preceded by months or even years by
Raynaud's phenomenon when it is part of
systemic scleroderma
Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. There are ...
.
The term "sclerodactyly" comes .
It is generally associated with systemic scleroderma and
mixed connective tissue disease
Mixed connective tissue disease (MCTD) is a systemic autoimmune disease that shares characteristics with at least two other systemic autoimmune diseases, including Systemic scleroderma, systemic sclerosis (Ssc), systemic lupus erythematosus (SLE), ...
, and
auto-immune disorders.
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, Raynaud's phenomenon,
esophageal dysmotility
An esophageal motility disorder (EMD) is any medical disorder resulting from dysfunction of the coordinated movement of esophagus, which causes dysphagia (i.e. difficulty in swallowing, regurgitation of food).
Primary motility disorders are:
...
, 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 ...
.) 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 mellitus, commonly known as diabetes, is a group of common endocrine diseases characterized by sustained hyperglycemia, high blood sugar levels. Diabetes is due to either the pancreas not producing enough of the hormone insulin, or th ...
, 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.
See also
*
Hand eczema
Hand eczema presents on the palms and soles, and may sometimes be difficult or impossible to differentiate from atopic dermatitis, allergic contact dermatitis, and psoriasis, which also commonly involve the hands. Even a biopsy of all these cond ...
*
Atopic dermatitis
Atopic dermatitis (AD), also known as atopic eczema, is a long-term type of inflammation of the skin. Atopic dermatitis is also often called simply eczema but the same term is also used to refer to dermatitis, the larger group of skin conditi ...
References
Cutaneous conditions
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