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Therapeutics in Dermatology
A reference textbook in dermatology

  Health professionals

Alvaro Machado is the author of this chapter.

ACKNOWLEDGEMENTS

Chapter written with the help of the EADV, the Fondation René Touraine and the Therapeutics in Dermatology

OVERVIEW

Morphea is a rare, autoimmune skin disease characterized by inflammation and sclerosis (hardening) of the skin and soft tissues and may lead to scar-like lesions. This disease should not be confused with systemic sclerosis, which is a different disease that affects internal organs and the blood circulation, as well as the skin. Morphea usually affects a few small areas of the skin. Very rarely, it can occur over larger areas of the body in a form called “generalized morphea”.

SIGNS AND SYMPTOMS

Morphea tends to develop slowly. The size of the lesions varies and usually they are oval in shape. Initially, the affected areas can be itchy, uncomfortable or numb. The lesions usually appear as bruise-like pink areas in the skin. Sometimes the affected areas of skin turn slightly darker and beige coloured. Over time, the affected areas become thicker and turn pale and waxy in the middle and the skin feels firmer than normal when pinched. In some areas, such as over a joint, mobility can be reduced. Hair is usually lost in the affected areas and the skin feels dry because the sweat is no longer produced. Rarely, fatty tissue and muscle under the lesions disappear and the skin becomes stuck onto the underlying bone.

Morphea can be divided into several subgroups: plaque-type morphea, linear morphea, generalized morphea, and other less common variants, such as deep, guttate and nodular.

Plaque-type morphea is the most common variant. It is generally asymptomatic and, consequently, frequently goes unno­ticed by the patient. The plaques most commonly develop on the trunk and in general are between 2 and 15 cm in diameter. They are usually mul­tiple and asymmetric. Individual lesions may enlarge significantly or remain stable in size.

Linear morphea frequently begins as a harmless-appearing lesion that extends as a series of plaques that then join to form a scar-like band. This variant tends to involve the deep structures, namely fascia, muscle and tendons, which may result in muscle weakness and decreased joint mobility. This form can have prolonged periods of disease inactivity followed by reactivation. When linear morphea affects the head it has the name of en coup de sabre. It is most often unilateral and extends from the forehead into the frontal scalp. Of note, the eye can be involved. In this form, patients may also develop seizures and neurologic problems.

Generalized morphea normally begins insidiously on the trunk as plaque morphea. An individual lesion is indistinguishable from classic plaque morphea, except that it does not stop expanding. Multiple plaques rapidly join and can affect nearly the entire trunk, often only sparing the nipples. As the disease progresses, it may result in disabling constrictions that even cause difficulty in breathing due to impaired thorax mobility and inflammation of the intercostal muscles. This variant is usually persistent given its often limited response to treatment.

WHO GETS AND CAUSES

The cause of morphea is unknown. It is not contagious. Some patients with family history of autoimmune diseases are more likely to get morphea, so there may be a genetic link. The affected areas of skin become hard because of increased production of a substance called collagen. Usually, collagen gives the skin its normal strength and structure, so when excess collagen is produced the skin feels harder and rigid than usual. Morphea can be triggered by skin injury such as an insect bite, a burn, radiation therapy, or viral infections. It may start at any age, including childhood and in adulthood, and is more common in women than men. Also, the morphea usually affects white skinned people and is rare in African-American people.

Approximately 2 out of 10 patients with morphea are children and teen­agers. The plaque-type morphea in this group has a mean age of onset of 7 years. On the other hand, in most patients with linear morphea the disease onset is prior to age 18 years. Thus, if left untreated, linear morphea may result not only in decreased mobilty of joints but also in per­manent limb asymmetry. Generalized morphea of children is similar to generalized morphea of the adult. It usually starts before 14 years of age and tends to cause lifelong severe disability due to persistent atrophy of the under­lying muscles and contractures of the involved joints.

In adult-onset morphea, localized morphea involves one to three affected areas. It is the most common subtype in adults and is unlikely to involve the muscle or bone. Generalized morphea presents as four or more areas of skin and subcutaneous involvement. Adults can also get linear morphea.

DIAGNOSIS AND TREATMENT

Diagnosis can be usually made through skin examination. A skin biopsy (a small piece of the skin removed under a local anesthesia) can be taken from the affected area if there is uncertainty or to rule out other skin diseases. Blood tests may also be helpful.

The treatment for morphea depends on the type (linear, plaque or generalized), the age of the patients and whether it has spread underneath the skin. Plaque morphea does not always need treatment as untreated disease normally progresses over 3–5 years and then regresses very slowly over years. However, strong steroid creams or ointments, non-steroid agents such as tacrolimus or imiquimod are sometimes indicated. Injections with steroid into the affected skin can also help. Early treatment is especially important for linear and generalized morphea to prevent further consequences, like loss of mobility. Options include phototherapy or intravenous treatment with steroids and methotrexate. Other treatments include penicillin and its derivatives or vitamin A derivatives, such as acitretin or isotretinoin. In addition to care from a dermatologist, expert treatment may be needed from a plastic surgeon or orthopaedic surgeon; in the case of persistent contractures, reconstructive surgery may be needed, and surgical revision may also be required in linear or en coup de sabre morphea. Finally, expert and longstanding physiotherapy is obligatory for patients in whom morphea threatens to impair mobility.

TIPS FOR MANAGING

Morphea dries the affected skin, so moisturizers may help soften and improve symptoms like itch. It is important to find a thick moisturizing product that patients are comfortable applying twice a day to decrease symptoms. It is a good idea to avoid long, hot showers or baths, as these can dry your skin.

Because morphea affects patients’ appearance, it can be an especially difficult condition to live with. Patients may start by seeing their primary care doctor. They may refer them to a doctor who specializes in skin disorders (dermatologist) or a specialist in diseases of the joints, bones and muscles (rheumatologist). Also, psychological support may be provided by primary care doctor or a mental health professional.

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