Not everything that glitters is gold, and not everything that’s bigger and thicker is good. A skin that’s extraordinarily thick, which is commonly accompanied with one or more worse symptoms, is alarming.
Scleroderma – the skin thickening disease – is an autoimmune disease involving the connective tissue/s. One’s immune system behaves abnormally and overly active, that it attacks its own cells – thus, the term, autoimmune. Aside from skin thickening, the disease also features spontaneous scarring, blood vessel affectation, inflammation, and damages to internal organs such as the esophagus, kidneys and lungs. Truly, beauty is not the only thing which is skin deep, scleroderma too.
This skin thickening disease is classified according to the degree and location of the skin and organ involved. Currently, it has been categorized into localized scleroderma and systemic sclerosis or widespread. The latter is further divided into either limited or diffused forms based upon the extent of skin involvement. In both categories, overactive immune cells attack body cells and form scar tissue or fibrosis in the skin or on the internal organ affected. Scar tissue may form haphazardly that it leads to thickness and firmness of the involved organ.
What causes scleroderma is an FAQ. Like most autoimmune diseases, the long-awaited answer is still far unknown. But brilliant researchers have identified the potential causes, and even risk factors. Some evidence has associated the skin thickening disease with genes. A study for Choctaw Native Americans showed that this race has a high prevalence for the disease. However, environment still seems to take a major role as it contains most of our body’s stressors. When an individual becomes susceptible, his immune system is activated – but in a more intense manner – thereby damaging tiny blood vessels and injuring tissues leading to scar formation and collagen accumulation. This process continues until thickening and other worse symptoms appear!
These signs and symptoms will then be the reference for classifying the disease. Localized scleroderma includes skin changes in isolated areas. These can be morphea patches – hardened and slightly pigmented multiple skin lesions, or linear scleroderma – localized and hardened skin patches usually at the lower extremity. Sometimes, the latter is accompanied with a ‘satellite’ lesion, such as on the abdomen.
Whilst localized type involves only the skin, the diffuse form may afflict important internal body organs. It includes symmetric skin thickening of the extremities, face and trunk that can quickly proceed to hardening after the inflammatory phase. With this fast pace type, organ affectation may occur early and seriously, such as scarring of the lungs, esophagus, heart and kidneys. Elevation of blood pressure is definitely a danger sign and may lead to kidney failure.
The limited form of scleroderma is not that limited at all. There might be lesser skin involvement but remains as serious as the diffuse form. Its characteristic manifestation pattern is better known as CREST, or Calcinosis (tiny calcium deposits on the skin), Raynaud’s phenomenon (spasm of tiny arterial vessels due to heat or cold), Esophageal (weaker lower esophagus leading to acid reflux from the stomach), Sclerodactyly (localized thickening and tightness of the skin of the fingers and toes), and Telangiectasis (tiny red portions on the face, hands and mouths which blanch when pressed). Patients may have symptom variation of CREST and worse, may overlap to a diffuse form of this skin thickening disease.
Patients are diagnosed according to the signs and symptoms present and verified with a blood test that suggest autoimmunity and presence of antinuclear antibodies (ANA). Anticentromere antibody can be found on limited form and Anti-Sci 70 antibody for diffuse scleroderma.
Can it be treated? A universal treatment has not been identified yet, but sign/symptom – directed method has been used, especially for those life threatening manifestations. Indeed, this skin thickening disease is still a thicker and bigger challenge both for its patients and medical scientists.