Hard to Feel Scleroderma

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Each day you care for your skin. No blemish escapes your notice. Now, your skin begins harden­ing or flaking.

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Imagine patches of your skin or internal organs slowly turning into jerky—a process that preserves dried salted meat without refrigeration. However, instead of preserva­tion, this leads to atrophy and loss of mobility. The autonomic disorder called “scleroderma” is derived from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Some refer to it as ‘the disease that turns people to stone.’ Biologically, the body’s fibro­blasts produce too much collagen, leading to fibrosis of the skin and sometimes the internal organs.

Doctors will want to rule out other causes of skin dryness. It could be a reversible reaction to a drug prescription or over-the-counter cosmetic. You may need to drink more water. Exact causes of sclero­derma are still unknown. Not all scleroderma hardens all of your skin or internal organs. Skin problems associated with scleroderma can sometimes fade away on their own in two to five years. The type of scleroderma that affects internal organs usually worsens with time. Researchers in the field of dermatology classify sclero­derma into four main types:

Classifications of Scleroderma

1. Localized Scleroderma is usually limited to a few places on the skin or muscles, and rarely spreads else­where. It is relatively mild without generally affecting internal organs. Persons with localized sclero­derma rarely develop systemic scleroderma.

Morphea localized scleroderma
Morphea localized scleroderma on the torso

2. Morphea is a form of localized sclero­derma, charac­terized by waxy patches on the skin of varying sizes, shapes, and color. Skin beneath patches can thicken. The patches may enlarge or shrink, and often disappear spon­taneously. Morphea usually appears between the ages of 20 and 50, but is often seen in young children.

3. Linear scleroderma is a form of localized sclero­derma that frequently begins as a streak or line of hardened, waxy skin on an arm or leg or on the forehead. Some­times it forms a long crease on the head or neck, referred to as en coup de sabre because it resembles a saber or sword wound. Linear sclero­derma tends to penetrate through deeper layers of the skin. It some­times affects the motion of the joints, which lie under­neath. Linear sclero­derma oftn develops in childhood, where the growth of involved limbs is compromised.

Linear scleroderma
Early development of linear scleroderma on forehead

4. Systemic scleroderma (systemic sclerosis) can affect the skin, esophagus, gastro­intestinal tract (stomach and bowels), lungs, kidneys, heart, and other internal organs. It can also affect blood vessels, muscles and joints. The connective tissues of involved organs become hard and fibrous, causing them to function less efficiently. The term systemic sclerosis indicates that “sclerosis” (hardening) may occur in the internal systems of the body. Systemic scleroderma can lead to pulmonary hypertension—high blood pressure in the blood vessels of the lungs.

There are two major recognized patterns that the illness can take—diffuse or limited disease. In diffuse sclero­derma, skin thicken­ing occurs more rapidly and involves more skin areas than in limited disease. In addition, people with diffuse sclero­derma have a higher risk of developing “sclerosis” or fibrous hardening of the internal organs.

💊 Omeprazole may cause erythematosus

March 29, 2016 – Some people who take proton pump inhibitor (PPI) med­i­cines, includ­ing Omeprazole delayed-release capsules (generic for Prilosec), develop subacute cutaneous lupus erythema­tosus (SCLE), an auto­immune disorder, or worsen­ing types of lupus erythema­tosus. This may be manifest as skin reddening or hardening. The U.S. FDA does not recognize SCLE as an adverse event associated with PPIs. In testing, discon­tinua­tion of PPI resulted in remis­sion, with PPI re-challenge causing SCLE to reoccur. Call your doctor right away if you have new or worsen­ing joint pain or a rash on your cheeks or arms that gets worse in the sun.Drug-Induced Subacute Cutaneous Lupus Erythematosus Associated with Proton Pump Inhibitors, Nitish Aggarwal.

About half of patients have a slower and more benign limited sclero­derma, with less wide­spread skin thickening. It is typically confined to the fingers, hands and face, and develops slowly over years. Although internal problems occur, they are less frequent and tend to be less severe than in diffuse sclero­derma, and are usually delayed in onset for several years. However, persons with limited sclero­derma, and occa­sionally those with diffuse sclero­derma, can develop pulmo­nary hyper­tension, a condition in which the lung’s blood vessels become narrow, leading to impaired blood flow through the lungs resulting in shortness of breath.

Finger calcinosis
Finger calcinosis

Limited scleroderma is some­times (formerly) called CREST syndrome. CREST stands for the initial letters of five common features:

  • Calcinosis
  • Raynaud Phenomenon
  • Esophageal dysfunction
  • Sclerodactyly
  • Telangiectasia

Diagnosing Scleroderma

Despite clearly articulated sclero­derma categories, some people with diffuse disease develop calcinosis and telan­giec­ta­sias so that they also have the features of CREST. Although most patients can be classi­fied as having either diffuse or limited disease, patients may have different combina­tions of symptoms.

No single test ensures a definitive diagnosis, but serologic testing for auto­anti­bodies is helpful. About 300,000 people in the United States have a form of sclero­derma, and nearly one-third of these (perhaps 75,000 to 100,000) are believed to be affected by its systemic variant. Patients diagnosed with advanced systemic disease have a progno­sis of anywhere from three to 15 years or more depending on the severity of the complications involving the lungs or other internal organs.

Raynaud’s phenomenon, charac­terized as episodic constric­tion of blood vessels in response to environ­mental factors such as cold, stress, or emo­tional changes causing to cold extremi­ties and cyanosis, may be present for many years before any other clinically signifi­cant symptoms or systemic manifesta­tions occur.

Scleroderma Treatment

Not all skin hardening or dryness is due to scleroderma. The lack of nutrients, need for probiotics, or inadequate water consumption is implicated in dry skin. Collagen gives skin its elasticity. When your body makes collagen, it combines amino acids—nutrients you get from eating protein-rich foods, like beef, chicken, fish, beans, eggs, dairy products, bone broth, nuts, and leafy vegetables. As we age, our bodies decrease ability to synthesize nutrients. Supplements like hydrolyzed collagen (or “collagen peptide”) powder may be useful. You may need to eliminate caffeine from your diet. For a topical solution, try using a chemical-free moisturizer.

Presently, there is no cure for sclero­derma. Doctors prescribe creams, ointments, pain relievers, and provide other treat­ments to slow fibrotic damage and manage symptoms. Steroid creams or pills may help reduce swelling and joint pain, or loosen stiff skin. Immuno­suppres­sive drugs are sometimes used. Joining a sclero­derma support group, such as one affiliated with the Scleroderma Foundation, enables sclero­derma patients to meet and exchange informa­tion with others who have similar problems.

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Kevin Williams is a health advocate and writer of hundreds of articles for multiple web­sites, including: A Bit More Healthy, KevinMD (WebMD), and Sue’s Nutrition Buzz. He is a prior 15-year con­sul­tant for Neutrogena Research and Scientific Affairs.

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