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Conditions Associated with Morphea Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Additional Symptoms
Autoimmune Conditions
Cancer: T-Cell Lymphoma
Infections
Melanonychia
Skin Conditions
Systemic Scleroderma

Overview

Leaf PrintAbout 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.

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

(Case Report) Coexistence of Ankylosing Spondylitis (AS) With Morphea. Morphea and AS with an inflammatory background and active immunity can exist in the same patient and recent studies have showed the importance of Th17 and associated cytokines in the pathogenesis of these two diseases. Archives of Rheumatology 2014, Volume 29, Number 2, P 143-146. (Also see Ankylosing Spondylitis, and Causes of Scleroderma: Cytokines)

Inflammatory morphea in the context of Raynaud phenomenon. Even in the absence of initial symptoms to support systemic disease, patients presenting with morphea in the setting of Raynaud phenomenon or anti-centromere antibodies deserve close surveillance for the possibility of CREST syndrome and systemic sclerosis. Dermatology Online Journal. (Also see Raynaud's)

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, VeryWell.

Longitudinal melanonychia is the presence of a pigmented stripe, usually brown or black, along the length of the nail bed in darker-skinned individuals. VeryWell.

Skin Conditions Associated with Morphea

Associated skin conditions include alopecia areata, vitiligo, dystrophy of the nails, and ichthyosis.

Eosinophilic fasciitis, morphea and vitiligo in a single patient. The association of morphea/fasciitis with eosinophilia is a classical finding; the presence of vitiligo raises the question of possible association between these different disorders. PubMed, Ann Dermatol Venereol, 2014 Oct;141(10):598-602.

Psoriasis and concomitant fibrosing disorders: Lichen sclerosus, morphea, and systemic sclerosis. In this population, a predisposition toward autoimmunity is seen as 38.5% of patients had a personal history of a third concomitant autoimmune disease, in addition to psoriasis and fibrosing disorder, whereas 42.3% reported a history of a first-degree relative with an autoimmune disease. Journal of the American Academy of Dermatology Volume 67, Issue 5 , Pages 1079-1083, November 2012. (Also see Psoriasis, Lichen Sclerosus, Systemic Sclerosis, and Multiple Autoimmune Syndrome)

Systemic Scleroderma Associated with Morphea

Introduction

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!

Abstracts

Coexistence of Recurrent Generalized Morphea and Systemic Sclerosis. There have been several previous reports concerning the coexistence of SSc and morphea, including that nine (6.7%) of 135 cases of SSc had additional lesions of morphea, three of which exhibited multiple morphea lesions on their trunks. They concluded that morphea was one of the skin involvements of SSc, because a percentage of 6.7% was high enough to suggest such a relationship. Our case demonstrated that morphea activity was independent of the preceding SSc. ACTA, Volume 89, Issue 3, Pages: 329-330.

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