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Connective Tissue Disorders

What is Connective-Tissue-Disorders?

Cutaneous Lupus Erythematosus (CLE)

Cutaneous Lupus Erythematosus (CLE) is a condition in which the body's immune system produces auto-antibodies that attack the skin. CLE can be broadly classified into three categories – Acute Lupus Erythematosus (ACLE), Subacute Lupus Erythematousus (SCLE) and Discoid Lupus Erythematosus (DLE).

ACLE is characterized by a rash over the face, sparing the nasolabial folds ("butterfly-rash").

SCLE usually affects areas exposed to sunlight, such as the face, the 'V' of the neck, the scalp, the arms and the 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.


Dermatomyositis is a rare autoimmune condition that causes inflammation in both the skin and the muscles. A skin rash usually appears before 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.

The dermatologist may organise a number of tests including a blood test, a skin biopsy, a Magnetic Resonance Imaging (MRI) scan to examine the muscles, a muscle biopsy, or an electromyography (EMG) to record the electrical impulses that control the muscles.

Dermatomyositis carries a risk of tumour development, thus dermatologists know to check regularly to ensure that any such tumours are discovered early.


Morphoea is a rare skin disorder by which areas of skin become much thicker and firmer than normal.  Morphoea 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 morphoea disappears so the skin becomes indented and stuck onto the underlying bone.

Morphoea usually develops slowly and there may be few symptoms. The affected area(s) can be itchy, uncomfortable or numb. When morphoea 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.

Systemic Sclerosis (Scleroderma)

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.

In addition to skin changes, patients often manifest symptoms due to dysfunction in other organs such as the lungs, kidneys and heart etc. Co-management with a Rheumatologist and relevant specialists is often carried out.

When to seek medical advice

See your doctor if:
  • You have a persistent photo-distributed rash, with or without systemic symptoms
  • The rashes heal with scars
  • You have unexplained weight loss
  • You have muscle weakness
  • You have joint immobility
  • You have systemic symptoms in addition to skin changes
Treatment and drugs

Cutaneous Lupus Erythematosus

1) Stop smoking. Smoking can make this condition worse and may also result in a poor response to treatment.
2) Sun Protection: This should be part of one’s daily routine

Local:  Strong steroid creams or steroid injections may be prescribed. Alternately, non-steroidal creams may be administered, such as calcineurin Inhibitors. Such treatments do not contain steroids and act on the immune system to help reduce inflammation.

Systemic Treatments: Anti-malarial tablets such Hydroxychloroquine may be taken orally. These medications were originally introduced to treat malaria but were found also to have a powerful effect on inflammation and thus may 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 as treatment. These immune suppressants may 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.

Morphea and Systemic Sclerosis

The treatment for morphoea 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 morphoea 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.
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