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-BMP6
-Possible therapeutic agent
-Fairly new data

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Bone morphogenetic protein-6 inhibits fibrogenesis in scleroderma offering new treatment options of fibrotic skin disease

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Bone morphogenetic protein-6 (BMP6) is known to be crucial for regulating embryonic skin development. This study assessed the role of BMP6 in dermal f...

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-Scleroderma info page with current news and topics pertaining to the disease!
-Check out the pinterest and tag me on anything you think I should read further! @MariannaS13

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-66 yr old woman
-Numi Organic Dry Desert Lime Tea
-Positive results after drinking the tea
-Intake and positive results were an accident initially

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-Foundation looking for a cure with many efforts.
-Support from celebrities, board of directors and a scientific advisory board
-"America's largest nonprofit investor in Scleroderma research

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Led by a Scientific Advisory Board comprised of some of the most highly regarded scientists in the nation, the SRF's research program actively seeks out and recruits experts from the fields of immunology, genetics, and fibrosis, as well as experts in cutting-edge technologies to join the scleroderma research community.

In the past few years, in addition to its core research program, the SRF has led the formation of two large-scale projects aimed at accelerating scleroderma research: the CONQUER Registry, the first nationwide longitudinal registry for scleroderma patients in the United States, and the GRASP Project.

The SRF's unique collaborative approach is enabling scientists from leading institutions across the nation-and around the world-to work together and develop an understanding of how the disease begins, how it progresses and what can be done to slow, halt or reverse the disease process.

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Led by a Scientific Advisory Board comprised of some of the most highly regarded scientists in the nation, the SRF's research program actively seeks out and recruits experts from the fields of immunology, genetics, and fibrosis, as well as experts in cutting-edge technologies to join the scleroderma research community.

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-Bob Saget's sister died from Scleroderma
-He was able to make jokes about it
-Connected to the Scleroderma Research Foundation

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No one should have to suffer as my sister Gay did.

She was 44 in 1992 when she was diagnosed with systemic scleroderma, a disease that strikes mostly women in the prime of their lives. She had been living in Bucks County, Pennsylvania, but moved back to Los Angeles to be with my parents as they sought medical care for her.

Two years later, she passed away.

Scleroderma is an incurable chronic disease which means "hard skin." In some patients scarring forms in the lungs and on the skin, changing a person's appearance. In other patients, the blood vessels are predominantly affected, leading to profound loss of lung function over an extended period of time.

Related: "Fuller House" cast reveals 'biggest change' since original show and reboot

I became familiar with scleroderma several years before my sister came down with it. It began 25 years ago when I met Sharon Monsky, a woman who was to become one of the dearest friends of my life. She was the founder and then CEO of the Scleroderma Research Foundation - a great woman, a mother of three who'd been stricken with the disease herself as a young mom.

Sharon had originally cold-called me to perform standup at a benefit in Santa Monica she had titled "Cool Comedy-Hot Cuisine." I knew nothing about scleroderma. Performing and hosting benefits were the only ways to fund research dedicated to finding a cure, or, at the very least, finding ways to put the disease into some kind of remission. That was the best way to fund-raise for this difficult disease - through humor.

I said, yes.

It was a night of comedians and amazing food by well-known chefs Susan Feniger and Mary Sue Milliken.

The beautiful, generous Robin Williams was the first performer to stand up for this cause. I was a performer and later served as its host. Robin returned for a total of five times, helping Sharon to raise millions for research programs and the Centers of Excellence at Johns Hopkins, Duke, Stanford and UCSF, among other universities and major medical institutions.

The first year I went, Ellen DeGeneres and Rosie O'Donnell performed as well. It was such a moving night for me that I personally promised Sharon that I would be there the following year. I have since hosted "Cool Comedy-Hot Cuisine" over 30 times.

Related: Ancient virus may cause crippling disease

'There are no accidents'

The more you experience, the more you tend to believe, "there are no accidents." Except occasionally in your pants. (Apologies, but that's a long time for me to be serious without a shot of gallows humor to get me through the pain of discussing it.)

So, just three years after I found out what 'scleroderma' was, my sister was sitting in the audience at the benefit, now actually diagnosed with this orphan disease.

One year later, Gay lost her life to it. She was only 47.

I wanted to do something about it - to share our family's story with others and let them know that they aren't alone in this battle.

So, in 1996, I directed a television movie for ABC called "For Hope," a story 'loosely based' on events that directly affected my family. The writer was Susan Rice, the producer, Karen Moore.

It was a very emotional and fulfilling project, a moment in my life that I will always treasure. I believe that TV movie helped put scleroderma on the map for many.

Still, it's such a long road for the tens of thousands of people who have this terrible disease. My heart goes out to the patients and families of those afflicted. They always say "terrible disease," yet as we all know, there are no "good" ones.

Once my friend Sharon passed away 13 years ago, we were fortunate enough to have the brilliant Dr. Luke Evnin take over as Chairman of the SRF. I became a board member the day of Sharon's funeral.

I knew then I would be working the rest of my life to do whatever I could to help those affected.

Related: 11 "Fuller House" moments that will give you nostalgia

I better live a long-ass time.

I'm told I was very funny at Sharon's funeral. I don't recall; you never remember a good set. I do remember that nobody heckled me.

Since that time, the Scleroderma Research Foundation has raised over $35 million dollars. Thanks to the amazing research we fund at John's Hopkins, Stanford and UCSF, new drugs and new therapies have been developed, helping thousands of those struggling with this sometimes fatal disease.

After the loss of my sister, I have had the good fortune of meeting hundreds of scleroderma patients who I feel connected to on a deep level - as though it's some sort of horrific rite of passage to be in "this club."

We have been touched by all of the comedians and other performers who have given of their time to perform at all of our "Cool Comedy-Hot Cuisine" benefits over the years, which take place annually in New York and every other year in Los Angeles, San Francisco, and now Las Vegas.

So many dear friends have been there for me and for all of us at the foundation including: Robin Williams; Whoopi Goldberg; Dana Carvey; Jimmy Kimmel; Seth Meyers; Jeff Ross; John Oliver; Jeff Garlin; John Mayer; Jim Gaffigan; Jimmy Fallon; Rodney Dangerfield; Jay Leno; John Stamos; Bill Bellamy; Dave Koz; Sarah Silverman; Jerry Seinfeld; Conan O'Brien; Craig Ferguson; Jon Stewart; Lily Tomlin; Ben Folds; Pat Monahan; Train; The Goo Goo Dolls; Adam Duritz and Counting Crows; Sheryl Crow - and at our New York event last December: Andy Cohen; Kevin Nealon; Michael Che; and Louie C.K., just to name-drop 32 celebrities.

I will spend the rest of my life dedicated to helping our amazing Board of Directors at the incredible Scleroderma Research Foundation to work with the best medical minds as we fund a cure. We are still at the stage where we need attention and much more funding.

But as much as I love the Foundation, I want even more to hear that we have found a cure and that we have put the Scleroderma Research Foundation out of business.
For more information, visit the Scleroderma Research Foundation

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Bob Saget 's side project: Using comedy to find a cure for scleroderma

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No one should have to suffer as my sister Gay did. She was 44 in 1992 when she was diagnosed with systemic scleroderma, a disease that strikes mostly women in the prime of their lives. She had been living in Bucks County, Pennsylvania, but moved back to Los Angeles to be with my parents as they sought medical care for her.

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-Extensive physical exam
-Blood test (antibodies)
-Biopsy of affected skin

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Scleroderma - Diagnosis and treatment - Mayo Clinic

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Scleroderma - Comprehensive overview covers symptoms and treatment of disorders that involve skin hardening.

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-Overproduction of collagen resulting in build up
-The immune system attacks itself
-Can be triggered by pesticide, epoxy resins or solvent exposure
-Environmental factors
-Causes found through Mayo Clinic and the Case Repost to the left.

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-40 yr old woman
-Description of the disease, symptoms, and visuals

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Abstract

Scleroderma is an autoimmune connective tissue disorder which is characterized by fibrosis of visceral organs, skin and blood vessels. This condition can be localized or systemic. Its estimated prevalence is 250 cases in a million and it is more common in women than in men. Resorption of the mandibular angle and coronoid process can be observed in patients with scleroderma. Pressure of fibrous mucocutaneous tissues is thought to be the cause of the resorption. Decreased number of wrinkles due to sclerosis and distinct facial features because of the atrophy of ala nasi are among common clinical characteristics of this condition. The aim of this case report is to present a 40-year-old female patient with scleroderma who presented with signs of resorption at the angle of mandible, coronoid process, as well as widening of the periodontal space.

Keywords: Scleroderma, bone resorption, lamina dura

Introduction

The word scleroderma originates from Greek words "scleros" meaning hard and "derma" which means skin ( 1). Scleroderma is a connective tissue disorder which is characterized by fibrosis of the skin, internal organs and blood vessels. It is categorized into two subgroups which are described as systemic and localized. Systemic sclerosis differs from localized scleroderma because it is accompanied by Raynaud's phenomenon, acrosclerosis and internal organ involvement ( 2, 3, 4). Normal collagen type I and III are thought to be deposited excessively at various tissues by immunologically over-activated fibroblasts ( 1).

Although the etiology of scleroderma is not clear, genetic and environmental factors have been held responsible for the onset of this condition. First degree relatives of scleroderma patients have a high probability of developing the disease. Silica powder, vinyl chloride, L-tryptophan, silicone breast implants and organic solvents are among the environmental factors which were suspected to cause this condition ( 4). Scleroderma is mostly seen in women whose ages are between 30 and 50 and its prevalence is 250 cases in a million ( 2, 5).

Scleroderma causes various changes at the oral and facial tissues both clinically and radiographically. Radiographic changes in scleroderma patients are the widening of periodontal space, loss of lamina dura, and resorption of the mandibular angle ( 6). The first specific clinical sign of scleroderma is the swelling on the skin of hands and fingers ( 5). The aim of this case report is to present some clinical and radiographic characteristics of a patient diagnosed as having scleroderma.

Case Report

A 40-year-old female patient was referred to Oral and Maxillofacial Radiology Department, Istanbul University Faculty of Dentistry for dental complaints. Her medical history and the documentation she presented, revealed that the patient was diagnosed with scleroderma and she had been treated in the Department of Rheumatology at Istanbul University Faculty of Medicine, for 20 years. She had goiter and gastric disease. Extra-oral examination showed that the patient was not able to open her hands because of sclerosis () and had a narrow nose because of atrophy of ala nasi ().

Decreased number of wrinkles were also observed. Upper lip was insufficient to cover maxillary teeth, which is also known as "mouse-like face". Anamnesis revealed that the patient had Raynaud phenomenon. She had used cortizon and acetylsalicylic acid for twenty years due to the vascular decomposition. Although Raynaud's phenomenon and acrosclerosis were present in our case, there was no internal organ involvement. Patient complained about the dryness of her mouth and multiple caries lesions were observed. Trismus and increased overjet were observed in the intra-oral examination (). Panoramic radiography disclosed bone resorption in the right mandibular angle region of the mandible and in both ramus (. The patient was referred to the departments for specialized dental treatment.

Discussion

Scleroderma is an autoimmune disease in which the antibodies target blood vessels and connective tissues ( 5). There is no consensus on the treatment protocol ( 2, 4, 5). Although being controversial, penicillamine has been used to inhibit collagen deposition. Corticosteroids have no effect on the progression of the disease ( 4, 7). Other treatment options are based on controlling the symptoms ( 7). Treatment for scleroderma is selected and organized according to the affected organs ( 4).

Resorption of the terminal phalanxes, short and claw-like fingers because of acro-osteolisis, ulcers on finger tips are common in patients with scleroderma ( 7, 8). About 80% of them present with symptoms in the head and neck region ( 8). Dysphagia and gastroesophageal reflux are frequently reported complaints in this group of patients. Trismus, widening of the periodontal space, decrease in facial wrinkles owing to fibrosis of skin, orofacial telengiectasia, resorption of mandibular angle are some of the changes which may occur in the oral and maxillofacial region. Xerostomia and increased frequency of dental caries might be seen due to the secondary Sjögren syndrome ( 1, 2, 8). Rarely, mandibular resorption can lead to pathologic fractures ( 1). Although xerostomia is not present, high frequency of dental caries is attributed to inadequate oral hygiene by virtue of sclerosis of hands. Resorptions at the right mandibular angle, coronoid process and ramus region were present in our case and the patient's oral hygiene was poor.

Excessive collagen deposition in the cutaneous tissues around the mouth causes microstomia which prevents the patient from opening and closing the mouth. Fibrosis of salivary glands leads to xerostomia, dysphagia, and subsequently periodontal infections ( 1, 5). Although microstomia and severe periodontitis were present in our case, xerostomia and dysphagia were not observed. Radiographic changes in scleroderma patients are widening of the periodontal space, loss of lamina dura, resorption of the mandibular angle, zygomatic arch, digastric region, caput mandible, and coronoid process. Blunting of mandibular angles leads to radiographic features which are described as "tail of the whale" ( 1). White et al. ( 6) reported widening of the periodontal space in 37% of the scleroderma patients. Wood et al. ( 9) observed mandibular resorption in 29% of the patients. Excessive deposition of collagen and oxytalan fibers and the resorption of the alveolar crest around tooth roots might cause periodontal widening ( 9, 10). In our patient, periodontal widening, tail of the whale appearance, and mandibular resorption were present.

Conclusion

Scleroderma is a systemic disease which causes significant radiographic and clinical changes in the oral and maxillofacial structures. Dental professionals must be aware of these changes since xerostomia and limited mouth opening are especially important for the right diagnosis and treatment. Multidisciplinary approach is of utmost importance for the scleroderma patients because of the complications of disease.

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Scleroderma is an autoimmune connective tissue disorder which is characterized by fibrosis of visceral organs, skin and blood vessels. This condition can be localized or systemic. Its estimated prevalence is 250 cases in a million and it is more common ...

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-Women more than men
-Native Americans and African Americans (internal damage)
-Not hereditary, but can run in families with other autoimmune disorders

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Overview

Scleroderma (skleer-oh-DUR-muh) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues - the fibers that provide the framework and support for your body.

In some people, scleroderma affects only the skin. But in many people, scleroderma also harms structures beyond the skin - such as blood vessels, internal organs and the digestive tract. Signs and symptoms vary, depending on which structures are affected.

Scleroderma affects women more often than men and most commonly occurs between the ages of 30 and 50. While there is no cure for scleroderma, a variety of treatments can ease symptoms and improve quality of life.

Symptoms

Scleroderma's signs and symptoms vary, depending on which parts of your body are involved:

Skin. Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.

Fingers or toes. One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud's disease, this condition also occurs in people who don't have scleroderma.

Digestive system. In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren't moving food properly through the intestines.

Heart, lungs or kidneys. Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.

Causes

Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.

Doctors aren't certain what prompts this abnormal collagen production, but the body's immune system appears to play a role. In some genetically susceptible people, symptoms may be triggered by exposure to certain types of pesticides, epoxy resins or solvents.

Risk factors

Scleroderma occurs much more often in women than it does in men. Choctaw Native Americans and African-Americans are more likely than Americans of European descent to develop the type of scleroderma that affects internal organs.

Complications

Scleroderma complications range from mild to severe and can affect your:

Fingertips. The variety of Raynaud's disease that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). In some cases, gangrene and amputation may follow.

Lungs. Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).

Kidneys. When scleroderma affects your kidneys, you can develop elevated blood pressure and an increased level of protein in your urine. More-serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.

Heart. Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis). Scleroderma can also raise the pressure on the right side of your heart and cause it to wear out.

Teeth. Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of dental decay increases even more.

Digestive system. Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing - some describe feeling as if food gets stuck midway down the esophagus - as well as bouts of constipation alternating with episodes of diarrhea.

Sexual function. Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.

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Scleroderma (skleer-oh-DUR-muh) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues - the fibers that provide the framework and support for your body. In some people, scleroderma affects only the skin.

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-Medications: blood pressure drug, immunosuppressive drug, proton pump inhibitors (steroid), antibiotic ointment and OTC pain relievers
-Therapy: physical or occupational
-Surgery: amputation or lung transplants

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Scleroderma - Diagnosis and treatment - Mayo Clinic

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Scleroderma - Comprehensive overview covers symptoms and treatment of disorders that involve skin hardening.

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-Hardening, patchy skin
-Shiny skin
-Exaggerated responses known to be Raynaud's disease
-Acid reflux and lack of nutrient absorption
-Function of the heart, liver and kidneys could be affected
-Symptoms found through Mayo Clinic and the Case Repost below

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-Autoimmune disease that affects the connective tissue
-It can be localized or systemic
-Formation of excess fibrous connective tissue in skin, organs and blood vessels
-Build up of scar tissue
-Can last up to 3-5 years