Morphea

morpheaMorphea (also known as Localized Scleroderma or Circumscribed Scleroderma) is a rare autoimmune disorder, a form of scleroderma, in which locally the skin and the underlying tissues are affected  


Description


Morphea is a form of scleroderma that doesn’t involve the internal organs and causes sclerosis of the skin.


Causes


The cause of localized scleroderma is not known. Morphea is associated with other autoimmune diseases, cancer, lymphoma
In some cases the disease is a part of multiple autoimmune syndrome that includes three or more defined autoimmune disorders in a single person.
Scientists suggest that morphea may be caused by the increased collagen and extracellular matrix in the derma (the middle layer of the skin), although the pathogenesis is not known.


Risk factors


Women are more likely to develop the disease.
The association of the disease with  local injuries, viral infections including measles, Lyme disease, exposure to radiation, pregnancy, medications intake (bleomycin, penicillamine, valproic acid) was also reported.
People whose relatives are affected or have another autoimmune disorders are tend to develop the disease.

Symptoms and types of morphea

Typically, affected persons have no other complaints except the visible changes of the skin. Rarely itching or pain may be present.

There are several types of morphea according to the type of the skin lesion.In one person may be present several types of the disease at once.

    • Circumscribed or plaque morphea is the most common type of morphea and develops over the course several years. During examination 1-3 scar-like patches of thickened skin are visible (so-called plaques). They are 1-20 cm in diameter. When they occur they have pink/mauve color, after several months they become white. The patches appear smooth and hairless. The patches become brown and the areas of skin appear depressed. These plaques are located asymmetrically on the trunk and extremities (commonly they are found on the breasts and hips);
    • Generalised morphea is diagnosed when more than 4 plaques are located on 2 or more areas of the body;
    • Superficial morphea appears as symmetrical pink patches in the folds of the skin (armpits, groin, under the breasts). This type affects middle-aged women;
    • Linear scleroderma is characterized by the presence of the linear plaques along the lines of Blaschko. Usually the limbs are involved and the lesions appear bilateral. The lesions may include the deposits of calcium;
    • En coup de sabre is a form of linear morphea that affects the scalp and temple. The hair is lost and the central nervous system may be involved;
    • Progressive facial hemiatrophy (known as Parry-Romberg syndrome) involves the shrinkage and degeneration (atrophy) of the subcutaneous tissues on the one side of the face and occasionally the other parts of the body on the same side. The first symptoms of the disorder affect the area of the tissues around the temporal or buccinators muscles. The progression of the disease involves the atrophy of the skin and connective tissues underneath including fat, fascia, cartilage and bones and the muscles themselves;
    • Pansclerotic disabling morphoea affects children. This rare type of morphea leads to hardening of the skin and muscles underneath. Later joint contractures, ulceration and carcinoma may develop;
    • Atrophoderma of Pierini and Pasini causes loss subcutaneous tissues;
    • Bullous morphea is an extremely rare form of morphea. On the lower extremities and inferior abdomen occur bullae, large blisters with hemorrhagic fluid;
    • Guttate morphea involves multiple chalk-like spotty lesions that appear on the neck and upper trunk;

[Read also: Basal cell carcinoma]

Sometimes children and adults experience muscle and joint pain, fatigue. Neurological symptoms such as seizures, headache are typical for persons with en coup de sabre and Parry-Romberg syndrome.

The affected areas of the skin may later become keloid (keloid scleroderma). Severe morphea causes stiffness and weakness of the affected limbs.

Diagnosis


Clinical examination is helpful to evaluate the diagnosis, although sometimes skin biopsy is necessary. Imaging studies are performed to check the condition of other organs and systems of the body.

Blood test to detect antibodies such as antinuclear factor, antitopoisomerase II alpha antibody may be done.

Treatment


Treatment is determined by the type of the lesion and the size of the affected areas. Plaque morphea may fade by itself without any treatment3-5 years after the onset of the disease, although the spread and rapidly progressing forms of morphea require treatment.

The first-line treatment of the disease includes phototherapy especially UVA1. Such therapy may cause side effects: headache, erythema, skin hyperpigmentation.

An active form of morphea requires the administration of methotrexate (in dose 15-25 mg/week orally or by subcutaneous injections) and glucocorticoids (intralesion injections are possible or they may be delivered orally in dose 1mg/kg).
Other treatment options include vitamin D analogues, topical tacrolimus, occupational and physical therapy.
Some patients may need cosmetic measures and plastic surgery.

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