Scleroderma

Sclero-hardening Derma-relating to the skin

Disease

Scleroderma is an autoimmune (the body attacking itself) group of diseases from the body making to much collagen (scar tissue). This hardens or tightens the skin and or connective tissues in the human body.

Affects women (80%) more than men (20%). Starts between 30-50 years old. Approximately, 100, 000- 300, 000 cases exist in the United States. No cure exists.

Causes

genetics, exposure to virus or bacteria infections, silica, and certain medications, and abnormal immune system

Diagnosis

Scleroderma is diagnosed by symptoms and ruling out other causes. This can be a long and frustrating process, estimated to be about 3 years.

Rhuematologists are specialized doctors that are often involved in the diagnosis and treatment of scleroderma. They or other doctors caring for you may order blood work, computer tomography (CT) and Xrays to rule out other diseases. Ultrasound and magnetic resolution imagery (MRI) may be used to look at tissue deep within your body.

Image from Medscape

Signs and Symptoms

skin The skin becomes hardened and tight. It may look shiny. Movement may become difficult because of the tight, hard skin.

Image by Merck Manuals

Raynaud’s Phenomenon Small blood vessels in hands and feet contract (become smaller) in cold temperatures or because of emotional distress. Toes and fingers may feel cold to touch, become numb, painful and even have a blue color. Raynaud’s phenomenon may happen when a person does not have scleroderma.

digestive symptoms Symptoms vary based on which tissues are affected but can include: difficulty swallowing, heart burn, cramping, bloating, constipation, and difficulty absorbing nutrients.

heart Scarring on the heart can create irregular and deadly heart rhythms. High blood pressure (hypertension) . Swelling in legs and shortness of breath are common in congestive heart failure (CHF).

lungs Scaring on the lungs can cause difficulty breathing and dangerously low oxygen levels in the body. This may be called pulmonary hypertension by your doctor.

kidneys Scarring in these organs can lead to high blood pressure and difficulty eliminating toxins in the form of urine.

mouth Tightening of skin on the face can lead to difficulty brushing teeth or having them cleaned professionally. Decreased amounts of saliva (spit) are produced. This increases the risk of cavities.

sex organs Men may experience difficulty getting or maintaining an erection (getting hard). Women are more likely to decreased vaginal lubrication and narrowing of the vaginal opening.

Treatment

Image by Dreamstime

Treatment of scleroderma is based on symptoms a person is having.

sildenafil (Viagra), tadalafil (Cialis) These medications are know to help men get and maintain erections. They are also used to treat the symptoms of Raynaud’s Phenomenon and pulmonary hypertension.

omeprazole (Prilosec) Proton-pump inhibitors such as Prilosec help reduce heart burn symptoms.

enalapril (Vasotec), lisinopril (Zesteril) ACE inhibitors such as these medications may be used to control high blood pressure, congestive heart failure, and damage to the kidneys.

Immune suppressants and immune globulin are medications being used to treat muscle pain and weakness

Resources and References:

https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952

https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Scleroderma

https://www.scleroderma.org/site/SPageServer/;jsessionid=00000000.app30131b?NONCE_TOKEN=60C20EFC158B414B40DFD1994734C15B#.Xylq4ihKjIU