What is Morphea?

Morphea, also known as localized scleroderma, causes hardening and discoloration of the skin leading to thickening and lighter or darker skin than the surrounding area. It is thought to be isolated to the skin without internal organ involvement as seen in systemic sclerosis (commonly known as ‘scleroderma’). This disease is thought to be autoimmune in origin, such as systemic lupus erythematosus and vitiligo.  While not a health threat in terms of mortality, morphea can significantly affect a patients functional status with scarring, disfigurement or other associated symptoms.  It is often under appreciated in its early stages as it commonly appears as a bruise, which is the time at which it is most amendable to treatment.

Morphea has a various manifestations with seven recognized subtypes.  The five most common are:

• Plaque: Oval/round, solitary indurated areas that do not coalesce
• Linear: Band-like induration or coalescing plaques that form a linear pattern, may involve deeper tissues. Facial varieties include En Coup de Sabre and Progressive hemi-facial atrophy (Parry ROmberg syndrome).
• Generalized: Four or more coalescing plaques (lesions) larger than 3cm on two or more anatomic regions
• Mixed: Presence of lesions consistent with the definition of linear morphea plus either plaque or generalized lesions concomitantly. (New classification)
• Lichen Sclerosus/Morphea overlap: Plaques of morphea with overlying lichen sclerosus.

What Causes Morphea?

The cause of morphea is not known, but it is postulated that fibroblasts (cells that produce type 1 collagen in the skin) become stimulated to overproduce collagen resulting in areas of sclerosis or hardening.  However, given the numerous appearances of morphea, it is certain that differences exist within morphea patients themselves regarding disease pathogenesis. Researchers are trying to determine what triggers the disease and whether it is genetic in origin or environmental. To be clear, morphea is differentiated from certain conditions, such as nephrogenic fibrosing dermopathy, that are associated with exposures known to elicit sclerosis of the skin.

Who gets morphea?

Morphea is more common in women than in men and approximately 50% of patients are under the age of 18, but all age groups may be affected. There is very little data to support who is at highest risk for getting morphea as there are no known predictors of disease. However, morphea, as well as other autoimmune diseases, may be more common in the relatives of patients with morphea. 

 Are Treatments Available?

There is no cure for morphea.  In some patients, the disease remits after a period of years while in others it may be a chronic condition or relapse years or decades later. It is not possible to predict the disease course of patients at this time as it has not been thoroughly studied.  Although there is no cure, several different approaches have been attempted to treat morphea which focus on controlling the signs and symptoms and slowing the spread of the disease (studies have not determined the success of each treatment in all subtypes and age groups):

• Steroids: These act as agents to decrease inflammation and inhibit fibroblast function. When applied either as a cream/ointment or injected into the lesion, it only treats the areas it is applied to and new lesions may develop elsewhere. Oral or intravenous steroids have been used, more often in children in preparation for methotrexate dosing.  This is usually reserved for more extensive and severe disease (i.e. joint limitation, neurologic complications, potential for disfigurement).
• Tacrolimus, pimecrolmius: These are applied to the skin and act in a similar manner as steroids (anti-inflammation and inhibition of fibroblasts) and have the same limitations as topical steroids.
• Dovonex: This is a form of vitamin D that is applied to the skin with the same goal of suppressing inflammation and decreasing fibroblast function.
• Methotrexate: Used more often in children and is reserved for more extensive and severe disease (i.e. joint limitation, neurologic complications, potential for disfigurement). Requires laboratory follow up and concomitant folate administration.
• Calcipotriene: This Vitamin D derivative is taken orally and acts to decrease inflammation and fibroblast function.
• UVA-1: This form of phototherapy was found to increase the activity of matrix metallo-proteinase 1 (MMP-1) which breaks apart collagen.  Given as a skin directed therapy, it targets the MMP-1 in the skin and therefore leads to collagen breakdown.  It also has anti-inflammatory effects and therefore has shown promise in morphea patients. It only treats areas exposed to the light (thus, whole body units are advisable if there is concern of generalized lesions). Limited by the institutions with such units: UT Southwestern, Michigan University, Henry Ford, and the University of Alabama at Birmingham.
• PUVA, UVB phototherapy: These forms of phototherapy have also been utilized, but do not show as much promise as that of UVA-1.
• Physical therapy: Aimed at decreasing the degree of immobility that may be associated with cases of morphea.