| Conditions Associated with Morphea Scleroderma | ||
| This page was written by Shelley Ensz and has not yet been medically edited. See Disclaimer. | ||
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| Overview of Associated Conditions | ||
| About 25% of morphea patients will have at least one extra-cutaneous (non-skin) manifestation, but less than 4% of morphea patients will develop more than two non-skin manifestations. | ||
| Morphea is usually a benign skin condition of one or two colored skin plaques which affect only the skin and that gradually fade in three to five years, even without any treatment. It normally occurs by itself and not in conjunction with any other symptoms or illnesses, and generally speaking, the cause of most cases of morphea is still unknown. | ||
| About 25% of morphea patients will have at least one extra-cutaneous (non-skin) manifestation, but less than 4% of morphea patients will develop more than two non-skin manifestations. | ||
| Approximately 2% of morphea patients have anti-centromere (ACA) antibodies, which means that they may be at risk for eventually developing systemic scleroderma. | ||
| It is very common for morphea to occur along with linear scleroderma, since they are both forms of localized scleroderma. (See Types of Scleroderma.) | ||
| Morphea has been known to occur in patients who also have dermatomyositis, linear scleroderma, systemic lupus erythematosus, melanonychia, T-cell lymphoma, pemphigus, primary biliary cirrhosis, systemic scleroderma, and toxoplasma gondii infection. | ||
| Associated skin diseases include alopecia areata, vitiligo, dystrophy of the nails, and ichthyosis. | ||
| Rare Types of Morphea, such as generalized, guttate, keloid, nodular, pansclerotic or profunda may have different associations, in particular, keloid morphea which is strongly associated with systemic scleroderma. | ||
| Additional Symptoms Associated with Morphea Scleroderma | ||
| A 2003 large multinational study found that 25% of localized patients had at least one other manifestation, such as osteoarticular, neurological (epilepsy, headache, peripheral neuropathy), ocular, vascular, gastrointestinal (heartburn), respiratory, cardiac, or renal. | ||
| Less than 4% of the morphea patients had more than two non-cutaneous (non-skin) manifestations, and none of the patients in the study developed systemic scleroderma during the follow-up. | ||
| Autoimmune Conditions Associated with Morphea | ||
| Systemic sclerosis and localized scleroderma in childhood. Juvenile scleroderma syndromes, including the systemic and the localized varieties, represent the third most frequent chronic rheumatic conditions in pediatric rheumatology practice. (PubMed) Rheum Dis Clin North Am. 2008 Feb;34(1):239-55. (Also see: Juvenile Scleroderma) | ||
| Is Juvenile Localized Scleroderma really "LOCALIZED"? One fourth of JLS patients in this data series presented various kind of extra-cutaneous manifestations, sometimes with multiorgan involvement. For this reason, the term "localized" is somehow inappropriate. These findings should change our clinical approach to this disease and underline the need for systemic immunosuppressive treatment for some patients. Francesco Zulian. ACR Conference Oct. 2003. (Also see: Linear) | ||
| Morphea: Clinical History. Occasionally, localized morpheic lesions occur in the uninvolved skin of patients with systemic sclerosis. Morphea has also been reported in association with systemic lupus erythematosus, primary biliary cirrhosis, pemphigus, and dermatomyositis. eMedicine.com. (Also see: Systemic Scleroderma, Lupus, Primary Biliary Cirrhosis, and Dermatomyositis.) | ||
| Cancer: T-Cell Lymphoma Associated with Morphea | ||
| Morphea has been associated with T-cell lymphoma in several case reports. A very rare form of T-cell lymphoma is due to a virus (an HTLV-1 infection) and this virus has been associated in at least one case with guttate morphea. See: Causes of Morphea: Cancer. | ||
| Infections Associated with Morphea | ||
| Some cases of morphea may be associated with infection. Generally speaking, though, the cause of the majority of cases of morphea is unknown and it is not considered to be contagious or cancerous. See: Causes of Morphea: Infections. | ||
| Melanonychia Associated with Morphea | ||
| Morphea might be associated with longitudinal melanonychia, which is a long name for a dark stripe in a fingernail. Occasionally, melanonychia is associated with subungual melanoma. See Subungual Melanoma, About.com. | ||
| Longitudinal melanonychia is the presence of a pigmented stripe, usually brown or black, along the length of the nail bed in darker-skinned individuals. AboutDermatology.com. | ||
| Skin Conditions Associated with Morphea | ||
| Associated skin conditions include alopecia areata, vitiligo, dystrophy of the nails, and ichthyosis. | ||
| Systemic Scleroderma Associated with Morphea | ||
One of the most pressing concerns of morphea patients and caregivers is concern that morphea will "progress" into systemic scleroderma, and few people feel very reassured by hearing that it "almost never" happens. Just the slightest chance that it could happen is disarming to some and very worrisome to others. A great article on this topic is in ISN's book, Voices of Scleroderma Volume 2 by Dr. Vanessa Malcarne, entitled, Developing Empathy: A Researcher's Perspective on Being Diagnosed with Scleroderma, pages 177-181. When it comes down to it, nobody can guarantee a morphea patient that they will never develop other symptoms or illnesses in their lifetime, especially since the bottom line is that eventually everyone dies. And if there is the slightest chance something more serious might be associated with an illness or symptom, most of us would want to know about it. Generally speaking, research indicates that about 75% of people with morphea do not have additional symptoms or any other illness. However, about 25% may develop one or two symptoms outside of morphea, and about 2% or less—particularly, those with anti-centromere (ACA) antibodies—may be at risk of also developing systemic scleroderma, or have morphea along with some other serious disease. Therefore, with morphea you don't need to be always on guard for signs of trouble nor feel doomed to develop other serious symptoms or diseases. It is reasonable to take precautions such as having an annual physical and to report any new signs or symptoms to your medical team, the same as you would whether or not you had morphea. After all, the odds are 75% of the people with morphea will not develop additional problems, and 98% likelihood that they will not develop systemic scleroderma—and although 2% is a very slight risk, it is still many times greater than that of people who do not have morphea. Perhaps the greatest hazard is that a patient or caregiver may become obsessed or depressed or hypervigilant about the possibility of developing further symptoms, and thus complicate things with anxiety, depression or rounds of endless and unproductive medical tests. Usually, reasonable medical care, solid information and support are enough to overcome a temporary reaction such as this. The general rule of thumb is that if feelings of anxiety, uneasiness, depression, or obsession with health thoughts last more than two weeks, then it is time to consult medical advisers for help in combatting the sense of dis-ease, which can often be worse than the ailment itself and lead to things such as insomnia or oversleeping, edginess, fatigue, eating changes, or depressed mood. While it is healthy to gain information and knowledge about an illness and even its potential complications from responsible sources, it is unhealthy to dwell on it for extended periods of time, to lose interest in daily activities, or to not be able to put it in perspective. Our advice? It's reasonable to have some concern, but not to worry endlessly over it. Polish your flexible mental attitude with the confidence of being able to "roll with the punches" of whatever life delivers. There are no guarantees for any of us in life. Although it sounds trite, there are always people worse off than we are, and focusing on others in a worse plight is a healthier way to cope than to reduce our inner world to only our own concerns. Don't hesitate to seek professional guidance if you need help adjusting to the effects of morphea. Few of us are born with all the coping mechanisms necessary to adjust with great aplomb to significant changes in our looks, abilities, or health that morphea can impose through effects on the skin, underlying joints, or even from the side effects of treatments. An interesting thing that occurs with morphea, though, is that well-meaning doctors and others are likely to phrase it that you are "lucky" because systemic scleroderma is so much worse. Bah humbug! Nobody is "lucky" to have any illness, even one that is usually mild and self-limiting. Everyone with any symptom or illness has their own cross to bear. Don't feel obliged to be grateful for not having systemic or other awful illnesses. There's plenty enough to deal with, without that! |
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| Anticentromere antibodies are detected in less than 2% of morphea patients, and, most importantly, they identify people who may be at risk for progression to systemic scleroderma. | ||
| Morphea: Clinical History. Occasionally, localized morpheic lesions occur in the uninvolved skin of patients with systemic sclerosis. Morphea has also been reported in association with systemic lupus erythematosus, primary biliary cirrhosis, pemphigus, and dermatomyositis. eMedicine.com. (Also see: Systemic Scleroderma, Lupus, Primary Biliary Cirrhosis, and Dermatomyositis.) |



