There are several types of Connective Tissue Disorders:
CLE can be broadly classified into 3 categories - Acute Lupus Erythematosus (ACLE), Subacute Lupus Erythematousus (SCLE), and Discoid Lupus Erythematosus (DLE). In this condition, the body's immune system produces auto-antibodies which attacks itself.
Dermatomyositis is a rare autoimmune condition that causes inflammation in both the skin and the muscles. Dermatomyositis affects the skin and the muscles.
Morphea is a rare skin disorder where areas of skin become much thicker and firmer than normal.
Systemic Sclerosis is a multisystem disease that results in fibrosis and vascular abnormalities in association with autoimmune changes. These lead to breakdown of the skin, subcutaneous tissue, muscles and internal organs (e.g. digestive tract, heart, lungs and kidneys). The skin becomes thickened and tightly bound to underlying structures. Other than skin changes patients often manifest symptoms due to dysfunction in other organs such as the lungs, kidneys and heart etc.
ACLE is characterised by a rash over the face, sparing the nasolabial folds (“butterfly-rash”). SCLE usually affects areas exposed to sunlight, such as the face, "v" of the neck, scalp, arms and upper back. It causes a red, circular and occasionally scaly rash. DLE commonly affects the face and areas of the scalp containing hair but occasionally can spread to other body sites including arms, legs and torso. The rash consists of red scaly patches, which tend to clear eventually, resulting in thinning, scarring or colour change in the skin. When the scalp is involved, hair in the affected area may be permanently lost.
A skin rash usually appears before the muscle weakness starts; however, some people may only have the skin rash. Due to the inflammation in the muscles they become weak and may also be tender. The Dermatomyositis rash may be patchy and usually a bluish-purple colour; is sometimes very itchy; affects the skin around the eyes, over the knuckles, on the face, the “V” of the neck and the cuticles at the base of the nails which may become fragile and frayed. The rash is often made worse by sunlight and is therefore more obvious on areas of the skin which are exposed to the sun.
Morphea usually appears as bruise-like pink patches of skin that thicken and turn pale and waxy in the middle, leaving a pink/ pale purple border. The skin feels firmer than normal when gently pinched. Hair is usually lost from the affected areas and the sweat glands stop working so the skin feels dry. In rare cases, fatty tissue and muscle under the Morphea disappears so the skin becomes indented and stuck onto the underlying bone. Morphea usually develops slowly, and there may be few symptoms. The affected area(s) can be itchy, uncomfortable or numb. When Morphea affects the skin on a limb or over a joint, it can restrict growth and mobility and in severe cases can cause contractures and long-term disability.
Other than skin changes, patients often manifest symptoms due to dysfunction in other organs such as the lungs, kidneys and heart etc.
See your doctor if:
The dermatologist may organise a number of tests including a blood test, skin biopsy, MRI scan to look at the muscles, muscle biopsy, or an electromyography (EMG) to record the electrical impulses that control the muscles. A dermatologist should also be also fully aware of the risk of a tumour developing in association with Dermatomyositis and should check to make sure it is discovered early.
Co-management with a rheumatologist and relevant specialists is often necessary.
Treatment includes
Local: Strong steroid creams or steroid injections as well as steroid-sparing creams and ointments. Examples of this include calcineurin Inhibitors. These treatments do not contain steroids and act on the immune system to help reduce inflammation.
Systemic treatments such as anti-malarial tablets like hydroxychloroquine. These medications were originally introduced to treat malaria but were found also to have a powerful effect on inflammation and so help to control CLE. More severe cases can be managed with steroid tablets and non-steroid medications that modify the immune system such as dapsone, methotrexate, acitretin or mycophenolate mofetil.
Steroids are often used to lower the response of the immune system which reduces the amount of inflammation-causing antibodies. Strong steroid ointments are usually given for the skin rash for all areas including the face. Other immune suppressants such as methotrexate, mycophenolate, mofetil, and azathioprine are also used. These immune suppressants that have different side effects to steroids. They can be used on their own, or in combination with steroids to help Dermatomyositis. Hydroxychloroquine may also be useful for treating the rash.
The treatment for Morphea depends on the type (linear, plaque or generalised),and whether it has spread underneath the skin. Strong steroid creams or ointments or a non-steroid cream such as Tacrolimus are often used as they may relieve any irritation and stop the patches enlarging. Intralesional injections of steroid (into the affected skin) can also help. Early treatment is important for linear and generalised Morphea to prevent later problems especially loss of mobility. Options include ultraviolet therapy, oral steroids, hydroxychloroquine, Cyclosporine and Methotrexate.
Treatment of skin changes in Systemic Sclerosis is similar to that of Morphea. Stronger immunosuppressive medications and systemic steroids are also often used.
Click here to access our Find A Doctor directory for a list of doctors treating this condition across our NUHS institutions.
