Quick Answer
Yes, scleroderma often causes stiffness and tightening of the skin and connective tissues. The hallmark symptom of scleroderma is thickening and hardening of the skin, which can limit flexibility and mobility. However, the extent of stiffness varies from person to person.
What is scleroderma?
Scleroderma is an autoimmune disease that causes fibrosis (scarring) and thickening of the skin and connective tissues. In scleroderma, the immune system attacks healthy cells, causing inflammation and overproduction of collagen. This results in hardening and tightening of various tissues and organs in the body.
There are two main types of scleroderma:
Localized scleroderma
– Affects only the skin and tissues under the skin, usually of the face, arms, or legs
– Does not affect internal organs
– Typically develops in childhood or young adulthood
Systemic sclerosis (systemic scleroderma)
– Affects the skin and internal organs, including lungs, heart, kidneys, and digestive system
– Usually develops in middle age
What causes the stiffness in scleroderma?
The main cause of stiffness and tightness in scleroderma is excess collagen production. Collagen is a fibrous protein that provides structure and strength to connective tissues like skin, tendons, and bones.
In scleroderma, fibroblasts (collagen-producing cells) become overactive and produce too much collagen. This builds up in tissues, making them fibrotic and stiff. The mechanisms for increased collagen production include:
– Autoantibodies that stimulate fibroblasts
– Chronic inflammation
– Fibroblast dysfunction
In addition to increased collagen, other processes can contribute to stiffness in scleroderma:
– Swelling and thickening of tissues from inflammation
– Loss of elasticity in connective tissues
– Muscle weakness and atrophy from lack of use due to stiff joints
How does scleroderma cause skin stiffness and tightness?
The most characteristic feature of scleroderma is thick, tight skin that lacks flexibility. This occurs because excess collagen builds up in the skin and the tissues under it.
Some specific ways that scleroderma makes the skin stiff include:
– Increased collagen deposits in the lower dermis and subcutaneous fat
– Crosslinking and aggregation of collagen fibers
– Loss of elastic fibers in the skin
– Swelling and inflammation of skin tissues
This damaged collagen accumulates and forms thick bundles of fibers that are inflexible. It also tethers the skin tightly to underlying tissues.
As a result, the skin becomes rigid and less able to bend and stretch normally. People with scleroderma often have thin skin that looks shiny and stretched taut over bony areas.
Does scleroderma cause joint stiffness?
Yes, many people with scleroderma experience joint pain and stiffness, especially in the small joints of the fingers. There are several reasons why joint stiffness develops:
– Excess collagen deposition in tendons, ligaments, and joint tissues leads to fibrosis. This impairs normal joint movement.
– Inflammation in joint tissues causes swelling, pain, and stiffness.
– Calcium deposits can build up in joint tissues, causing stiffness. These calcium deposits are common around the joints of the fingers.
– Muscle weakness from lack of use allows joints to become stiff and lose range of motion. The fingers are curved into a claw-like position in some cases.
Joint stiffness can range from mild achiness and inflexibility to severe pain with severely limited mobility. Hand function is often impaired due to finger joint stiffness and contractures.
Does scleroderma affect mobility and flexibility?
Yes, scleroderma can have significant effects on a person’s mobility and flexibility:
– Stiff, thickened skin reduces normal mobility in many areas of the body. Bending and stretching can be impaired.
– Joint stiffness limits range of motion and dexterity, especially in the hands and fingers. This impairs activities of daily living.
– Stiff facial skin and mouth opening can impair oral hygiene, eating, and other facial functions.
– Gastrointestinal involvement can cause intestinal stiffness and impaired gut motility, leading to bloating and constipation.
– Stiffening around the chest wall and lungs can restrict breathing and lung capacity.
– Muscle weakness due to lack of use can further reduce mobility.
The extent of reduced mobility and flexibility varies greatly depending on how extensive and severe someone’s tissue involvement is. Mild forms of scleroderma may only cause minor stiffness, while severe systemic sclerosis can profoundly impact mobility.
How is stiffness and tightness treated in scleroderma?
While there is no cure for scleroderma itself, treatments aim to manage symptoms like skin tightness and joint stiffness. Some options include:
– Medications such as corticosteroids, immunosuppressants, and biologics to reduce inflammation and collagen overproduction
– Physical and occupational therapy to improve mobility, strengthen muscles, and prevent joint contractures
– Regular stretching and range-of-motion exercises
– Devices and splints to keep fingers and joints extended and mobile
– Surgery in severe cases to remove excessive collagen, release contractures, and improve joint mobility
– Medications to reduce gastrointestinal symptoms like bloating and constipation
– Breathing exercises and support devices to maximize lung capacity
In addition, protecting and moisturizing the skin helps improve resiliency. Phototherapy, laser treatments, and silicone sheets can improve skin flexibility as well. Treating secondary Raynaud’s phenomenon also aids with finger stiffness and pain.
While not curative, early and active treatment can significantly improve mobility and quality of life for people living with scleroderma. Supportive care from rheumatologists, dermatologists, and occupational therapists is key.
What is the prognosis for stiffness and mobility problems in scleroderma?
The prognosis for stiffness and related mobility impairment varies:
– In mild localized scleroderma, skin changes may plateau after 2-5 years. Mobility can improve.
– In systemic sclerosis, skin thickness often peaks around 5 years after onset but mobility issues may persist or worsen over time.
– Aggressive immunosuppression early in systemic sclerosis may help prevent progression of skin and joint problems.
– Physical therapy and stretching can help maintain range of motion even if stiffness cannot be reversed.
– Joint contractures and deformities are difficult to reverse if they become severe.
– Declines in mobility can have significant impacts on quality of life over time.
Overall, early diagnosis and treatment provide the best chance of controlling symptoms and maintaining mobility in scleroderma. The course of skin and joint involvement is highly variable between individuals. Close monitoring by a rheumatologist is important.
Coping with stiffness and mobility impairment
Living with scleroderma-related stiffness and reduced mobility can be challenging both physically and emotionally. Some tips for coping include:
– Learning about your specific symptoms and triggers to avoid activities that worsen pain and stiffness.
– Setting a regular exercise routine with stretching, range-of-motion, and low-impact cardio exercises.
– Using recommended splints and devices to maintain mobility.
– Asking for help with tasks made difficult by reduced mobility or dexterity.
– Making lifestyle adjustments to reduce strain on joints and skin (e.g., sitting instead of standing).
– Practicing good posture and body mechanics to avoid injury.
– Working with occupational therapy to use adaptive techniques and devices.
– Joining a support group to discuss challenges and solutions with others who understand.
– Focusing on abilities rather than disabilities and setting realistic goals.
– Making time for enjoyable hobbies that accommodate limitations.
– Sharing feelings with loved ones and asking for support when needed.
– Getting counseling if dealing with depression or emotional difficulties related to illness.
While scleroderma presents daily challenges, taking an active role in self-care and staying positive can help overcome mobility issues. With patience and support, many people learn to adjust and live full lives.
Conclusion
Scleroderma frequently causes significant skin tightening and hardening along with joint stiffness and restricted mobility. This occurs due to excess collagen deposition and inflammation in the skin, joints, and other connective tissues. Stiffness can range from mild to disabling based on the severity and extent of tissue involvement. Treatments like immunosuppression, physical therapy, and surgery can help improve mobility and function. However, scleroderma-related stiffness is often an ongoing challenge. Working closely with a rheumatologist and taking an active self-management approach offers the best quality of life for people living with scleroderma.