Localized Scleroderma

Page 1 2 3

What is Localized Scleroderma?

Scleroderma is a disorder with an unknown cause. It is characterized by thickening of the skin due to extra tissue being deposited within it. The excess tissue is called connective tissue, connective tissue is the framework which keeps much of the body together. Scleroderma is a group of conditions that are linked by the changes in the skin, however there are many other effects of the disease, and as a result it is classified into a variety of distinct conditions depending on the pattern of disease.

Classification of Scleroderma

Scleroderma is classified broadly into two categories:

Localized Scleroderma : This condition is usually localized to the skin and its underlying tissues, it rarely progresses systemically
Systemic Sclerosis : In this condition there is skin and/or organ involvement that is more severe and widespread than the localized form of the condition.

Localized scleroderma is classified into the following categories:

Localized cutaneous sclerosis
- Morphea
  - Plaque-type morphea
  - Guttate morphea
  - Subcutaneous morphea or morphea profundus
  - Nodular or keloidal morphea
  - Atrophoderma of Pasini and Plerini
  - Bullous and vesicular
- Linear Scleroderma
  - Craniofacial
    - En coup de sabre
    - With cranial atrophy
  - Acral
  - With arthritis, myositis and growth defects of bone
  - Linear meiorheostotic
- Generalised Morphea
  - Pansclerotic
  - Edematous
  - With lichen sclerosis et atrophicans
Localized Cutaneous Sclerosis as a part of an overlap syndrome
- Mixed connective tissue disease
- Undifferentiated connective tissue disease
- Sclerodermatomyositis
Cutaneous sclerosis as a component of eosinophilic fasciitis

Who gets Localized Scleroderma?

Localized scleroderma is an uncommon condition, and affects approximately 20 people in every million. Although uncommon, localized scleroderma occurs approximately 20 times more often than systemic sclerosis. Both the incidence of morphea and linear scleroderma are more common in females, with the male to female ratio being approximately 3 to 4: 1. Plaque morphea is the most common form of localized scleroderma. However in childhood linear scleroderma is the most common.

Predisposing Factors

Risk Factors ⁵

Other autoimmune disease e.g. Hashimoto's Thyroiditis, vitiligo & type 1 diabetes mellitus
Family history of autoimmune disease (diseases where the immune system attacks the body)
History of trauma, which is seen in 3 to 13% of patients with localized scleroderma
Drugs and environmental toxins e.g. bleomycin
Infections e.g. Borrelia Burgdorferi (this has yet to be proven)

Progression

Pathogenesis

The reason why localized scleroderma occurs is still relatively unknown. However it is known that the condition develops due to abnormalities in connective tissue as well as the immune system and blood vessels. Early on in the disease there is damage to the blood vessels and release of certain molecules that stimulate cells called fibroblasts (connective tissue producing cells) to produce more connective tissue. There are also increased numbers of inflammatory cells that cause damage to the tissue.

Clinical Features

Morphea

Plaque Morphea

In this condition there is reddening of the skin with scwelling. As the disease goes on the skin turns white and sunken. It most commonly occurs on the trunk but can also occur on the extremities. The size of the lesions rages from 0.5cm to 30cm. Later in the disease the lesions may become overly pigmented or stay underpigmented. In lesions that are progressing a surrounding area of redness is seen called the 'lilac ring'. There is rarely ever internal organ involvement.

Guttate Morphea

This condition usually produces multiple and small 'confetti' like lesions, 2 to 10mm in diameter. The lesions are under-pigmented or over-pigmented spots with a thin overlying skin. This condition frequently involves the shoulders and the chest.

Morphea Profundus

In this condition the skin becomes thickened and tighter. The skin loses it colour and becomes sunken in.

Nodular (Keloidal) Scleroderma

This condition most commonly involves the chest and appears as distinct papules (small areas of elevated skin) or plaques (large areas of elevated skin). They are difficult to distinguish from keloids (an abnormally aggressive healing response). These lesions can be many centimeters in diameter, there may be single or multiple lesions.

Bullous Morphea

The formation of bullae (fluid filled lesions) can occur in most types of localized scleroderma. Common sites where the lesions can appear are on the extremities, trunk, face, or neck. These lesions may be superficial or extend deep into the skin. It is postulated that the bullae form in response to trauma or lymphatic obstruction.

Vesicular Morphea

In localized scleroderma vesicles (smaller fluid filled lesions) can develop, these are most often seen on the arms.

Atrophoderma of Pasini and Pierini

Lesions in this condition are described as being depressed, over-pigmented areas of skin, usually on the trunk, with a 'cliffdrop' border. This condition can occur alone or with other forms of localized scleroderma.

Linear Scleroderma

Lesions form straight bands on the skin causing the skin harden & change pigmentation. The pattern of the lesions tends to be horizontal on the trunk of the body and longitudinal when seen on the arms and legs. This condition can spread and cause destruction of the underlying bone and muscle. These lesions are usually present on the limbs, face and scalp of patients. In 10 to 22% of cases of linear scleroderma there is shortening of the affected limbs due to impaired growth. There are many complications of linear scleroderma, these can include the following:

Tissue destruction that can involve: soft tissue, muscle, bone and sometimes the lining of joints.
Defects in growth in different areas of the limb
Deformities of the limb
Involvement of the skin surrounding the mouth and spread of lesions into the oral cavity causing severe dental problems such as the premature loss of primary teeth, eruption of the permanent teeth and reduced growth of bone.

En Coup De Sabre

This form of linear scleroderma describes lesions that resemble a scar from a sabre blow to the head. These lesions appear on the patients face and/or scalp, usually on one side, with accompanied loss of the hair in affected areas. The lesions are ivory in colour and have a depressed appearance. These lesions last for decades.

Generalised Morphea

In this form of localized scleroderma the lesions may gather in a single area or involve three or more areas. Common sites where these lesions are seen are on the trunk and the legs. Occasionally the acral (fingers, toes and ears) areas of the patient's body are spared. This condition can cause deep involvement of tissues causing disfigurement.

Page 1 2 3