What Is Scleroderma?

Welcome Back Rheumatology Fans,

While it is not a common question that I get because no one knows what it is, it doesn’t change that no one knows what it is…

Scleroderma

Scleroderma is a group of diseases that are essentially connective tissue disorders. The main unifying symptoms are thickening of the skin and connective tissues, typically affecting the palms of the hands.

The thickened palms of the hands restrict function affecting extension of the fingers, this is thick and shiny with variable levels of pain. Different variations of scleroderma also affect other areas, particularly Systemic Sclerosis which also causes thickening of the connective tissues around the internal organs.

Some patients with Scleroderma will also develop arthropathy that is very similar to a Rheumatoid Arthritis presentation, the combination of the two is obviously problematic particularly for hand function.

Onset and Demographics

Typically onset is between 30 and 50 years old and is much more common in Female patients than Male. A common early symptom is Raynaud’s Phenomenon, also consider the demographics are similar to those of Rheumatoid Arthritis. It is possible that the onset begins with Arthropathy and Raynaud’s with the skin thickening going unnoticed.

Assessment and diagnosis under Rheumatology is essential as the systemic symptoms can cause severe issues with the internal organs. Commencing appropriate medical treatment as soon as possible is essential to a good outcome.

Non-Medical Treatment

Non-Medical Treamtent options are possible, they are typically going to be led by hand specialists aiming to retain function with the thickened skin, arthropathy and Raynaud’s Phonomenon.

Resting splints can be useful to retain range of motion, particularly extension of the digits.

Massage might also provide short term improvements in function to enable in session exercise and functional work.

General Health interventions are extremely important to address systemic inflammatory factors - smoking, obesity, low activity levels, poor diet etc.

General function and cardiovascular health are also vital components to maintain and starting early after diagnosis is key to long term adherence. Getting this back once it is reduced is very difficult.

Patient Information Site: https://jamanetwork.com/journals/jamadermatology/fullarticle/2832863

Further Resources