DERMATOLOGY

NAME: MUHAMMAD NOORAIMAN AHMAD NOORDIN

GROUP : 30 A

SCLERODERMA

Definition

• Disease of the connective tissue with skin thickening spontaneous scarring, blood vessel disease, varying degrees of inflammation, associated with an overactive immune system.

Major Forms• Limited systemic sclerosis

/scleroderma involves cutaneous manifestations that mainly affect the hands, arms and face. It was previously called CREST syndrome in reference to the following complications: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias

• Diffuse systemic sclerosis/scleroderma is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart and lungs

Classification

• Localized scleroderma skin changes are in isolated areas, either as morphea patches or linear scleroderma.

• The diffuse form of scleroderma (diffuse systemic sclerosis) involves symmetric thickening of skin of the extremities, face, and trunk (chest, back, abdomen, or flanks) that can rapidly progress to hardening after an early inflammatory phase.

Localized Scleroderma

• Morphea is scleroderma that is localized to a patchy area of the skin that becomes hardened and slightly pigmented. Sometimes morphea can cause multiple lesions in the skin. Morphea is not associated with disease elsewhere in the body.

• Linear scleroderma is scleroderma that is localized usually to a lower extremity, frequently presenting as a strip of hardening skin down the leg of a child. Linear scleroderma in children can stunt bone growth of the affected limb. Sometimes linear scleroderma is associated with a "satellite" area of a patch of localized scleroderma skin, such as on the abdomen

Systemic Scleroderma

• Organ disease can occur early on and be serious and significantly decrease life expectancy. Organs affected include the esophagus, bowels, scarring (fibrosis) of the lungs, heart, and kidneys. High blood pressure can be a troublesome side effect and can lead to kidney failure (renal crisis)

Example of localized form

– Localized morphea– Morphea-lichen sclerosus et atrophicus overlap– Generalized morphea– Atrophoderma of Pasini and Pierini– Pansclerotic morphea– Morphea profunda– Linear scleroderma

Localized Morphea Morphea-lichen sclerosus et atrophicus overlap

Generalized morphea Atrophoderma of Pasini and Pierini

Pansclerotic morphea Morphea profunda

Linear scleroderma

Example of systemic form

– CREST syndrome– Progressive systemic sclerosis

CREST• Calcinosis refers to the formation of tiny deposits of calcium in the

skin. This is seen as hard, whitish areas in the superficial skin, commonly overlying the elbows, knees, or fingers. These firm deposits can be tender, can become infected, and can fall off spontaneously or require surgical removal. This is the least common of the CREST scleroderma variant features.

• Raynaud's phenomenon refers to the spasm of the tiny artery vessels supplying blood to the fingers, toes, nose, tongue, or ears. These areas turns blue, white, then red after exposure to extremes of cold, or even sometimes with extremes of heat or emotional upset. This can lead to tiny areas of damage to the tips of the fingers (digital ulcers) or larger areas of dead skin on the ends of the fingers.

• Esophagus disease in scleroderma is characterized by poorly functioning muscle of the lower two-thirds of the esophagus (the swallowing tube through which food passes from the mouth to the stomach). This can lead to an abnormally wide esophagus that allows stomach acid to backflow into the esophagus to cause heartburn, inflammation, and potentially scarring that narrows the esophagus. This can eventually lead to difficulty in passing food from the mouth through the esophagus into the stomach. Symptoms of heartburn are treated aggressively in patients with scleroderma in order to prevent injury to the esophagus.

• Sclerodactyly is the localized thickening and tightness of the skin of the fingers or toes. This can give them a "shiny" and slightly puffy appearance. The tightness can cause severe limitation of motion of the fingers and toes. These skin changes generally progress much slower that those of patients with the diffuse form of scleroderma.

• Telangiectasias are tiny red areas, frequently on the face, hands, and in the mouth behind the lips. These areas blanch when they are pressed upon and represent widened (dilated) capillaries.

• Patients with limited systemic sclerosis can have variations of CREST with differing manifestations, for example, CRST, REST, ST, etc. Patients can also have "overlap" illness with features of both CREST and the diffuse form of scleroderma. Some patients have overlaps of scleroderma and other connective tissue diseases, such as rheumatoid arthritis, systemic lupus erythematosus, and polymyositis. When features of scleroderma are present along with features of polymyositis, systemic lupus erythematosus, and certain abnormal blood tests the condition is referred to as mixed connective tissue disease (MCTD)

Secondary Complications of Systemic Scleroderma

• Pulmonary (lung) complications• Cardiac (heart) complications• Renal (kidney) complications• Gastrointestinal complications• Vascular complications• Oral and dental complications

Causes of Scleroderma

• Generally unknown• However areas being investigated include

autoimmunity, environmental exposures, genetics, and infections

General Symptoms of Scleroderma

Skin changes, including:• Hardening of your skin, particularly on your

hands, arms and face, and occasionally on your trunk and legs

• Loss of hair over the affected area• Change in skin color• Ulcers or sores on your fingers• Swelling or puffiness in your hands or feet, often

occurring in the morning

A condition called sclerodactyly, (thickening or hardening of the skin of the fingers and toes). You may experience:

• Skin on your fingers and toes becoming hard and shiny• Difficulty bending your fingersRaynaud's Phenomenon, a condition associated with poor blood flow

to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time in response to cold or emotional stress. Raynaud's Phenomenon may cause:

• Your fingers, toes, tips of your ears, nose or tongue to be very sensitive to cold

• Your fingers to turn bluish or very pale• Your fingers to feel tingly, numb or coldTelangiectasia, (tiny blood vessels become dilated and show through

your skin. Small reddish spots may appear on your fingers, palms, face, lips and tongue).

Calcinosis, (small white calcium lumps form in or under the skin). It is caused by scleroderma and not by too much calcium in your diet. If injured, they may become infected.

Arthritis and muscle weakness, including:• Pain, stiffness and swelling• General fatigue• Muscle weakness, often in your upper arms or thighsIf the heart and lungs are affected, you may experience:• Shortness of breath• Persistent cough• Chest pain

Digestive problems including poor function of GIT. Symptoms may include:

• Difficulty swallowing• Heartburn• Bloating• Nausea or vomiting• Weight loss• Diarrhea or constipationKidney problems, such as kidney failure and high blood pressure.

Symptoms may include:• Fatigue• Severe headache• Shortness of breath• Trouble seeing• Mental confusion

Prevalent Signs & Symptoms of Scleroderma

• Raynaud's phenomenon. An exaggerated response to cold temperatures or emotional distress, this condition constricts the small blood vessels in the hands and feet and causes numbness, pain or color changes in the fingers or toes.

• Gastroesophageal reflux disease (GERD). In addition to acid reflux, which can damage the section of esophagus nearest your stomach, you may also have problems absorbing nutrients if your intestinal muscles aren't moving food properly through your intestines.

• Skin changes. These changes may include swollen fingers and hands; thickened patches of skin, particularly on the fingers; and tight skin around the hands, face or mouth. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.

Localized sclerodermaThe two main varieties of localized scleroderma, which affects only the skin, are distinguished by very distinctive signs and symptoms:

• Morphea. This variety features oval-shaped thickened patches of skin that are white in the middle, with a purple border. Skin patches could appear in only a few spots on your body or be more widespread, appearing all over your body. Morphea generally fades out after three to five years, but you may retain areas of darkened skin where the areas of thickened skin appeared.

• Linear scleroderma. This variety, which is more common in children, features bands or streaks of hardened skin on one or both arms or legs, or on the forehead. Linear scleroderma often appears mostly on one side of the body.

• Systemic sclerodermaThis type of scleroderma, also called systemic sclerosis, affects not only your skin but also your blood vessels and internal organs. Subcategories are defined by what portions of your body are affected. One variation is known as limited scleroderma, or CREST syndrome

Treatment

• Scleroderma has no known cure — no drug will stop the overproduction of collagen. But the localized variety of scleroderma sometimes resolves on its own. And a variety of medications can help control the symptoms of scleroderma or help prevent complications.

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The primary goals of treatment for people with scleroderma are as follows:

• Relieve symptoms to the greatest extent possible.• Maintain the patient's overall functional capacity.• Prevent long-term complications of scleroderma.• Manage and treat complications, if they develop.• Enable patients to lead a reasonably good quality

of life

Treatment of Localized Scleroderma

• The treatment options that are currently available for the management and treatment of localized scleroderma, including:– Psoralen photochemotherapy (PUVA)– Topical photodynamic therapy– Drug therapy

Treatment of Systemic Sclerosis

• The medications that are prescribed for the management and treatment of systemic sclerosis, including:– Immunosuppressive agents– Antifibrotic agents– Anti-infammatory agents– Vasodilator agents– Angiotensin-converting enzyme (ACE) inhibitors

Treatment of Secondary Complications

• The management and treatment of secondary complications that may develop in people with scleroderma, including:– Pulmonary fibrosis– Pulmonary hypertension– Raynaud's phenomenon– Heartburn– Joint pain– Limb contracture due to stiffness– Impaired kidney function– Dry mouth and dry eyes in people with scleroderma who also

suffer with Sjogren's syndrome

Treatment of Systemic Sclerosis

• A detailed overview of new treatments that are currently under investigation for the treatment of systemic sclerosis, including:– Interferons– Tumor necrosis factor alpha blockers– Halofuginone– Plasmapheresis– Photopheresis– Autologous stem cell transplantation

Medications

• Dilating the blood vessels. Blood pressure medications that dilate blood vessels may help prevent lung and kidney problems and may help treat Raynaud's disease.

• Suppressing the immune system. Drugs that suppress the immune system, such as those taken after organ transplants, may help reduce scleroderma symptoms.

Therapy

• Physical or occupational therapy. Therapists can help you to manage pain, improve your strength and mobility, and work on performing essential daily tasks to maintain your independence.

• Cosmetic procedures. The appearance of skin lesions associated with scleroderma may be helped by exposure to ultraviolet light. Laser surgery also may help camouflage or eliminate these lesions

Surgery

• Amputation. If finger ulcers caused by severe Raynaud's disease have developed gangrene, amputation may be necessary.

• Lung transplants. People who have developed high blood pressure in the arteries to their lungs (pulmonary hypertension) may be candidates for lung transplants