Can Morphea Turn Into Systemic Scleroderma?

Usually morphea involves the skin, subcutaneous tissue and rarely the muscles and joints. Usually internal organs are not affected by morphea and it is very rare for morphea to progress into systemic scleroderma. There are only very few cases reported, even in those cases it is not confirmed if they actually had morphea in the first place.

Morphea that involves the subcutaneous tissue can extend into the muscle layers and rarely can involve the internal organs, but this very rare. If you have morphea you are not at increased risk of getting systemic scleroderma although both conditions have similar etiology.

A study done in Italy “From Localized Scleroderma to Systemic Sclerosis. Coexistence or Possible Evolution” on 330 patients diagnosed with systemic sclerosis found that 8 female patients had both systemic sclerosis and morphea, 6 patients out of that developed morphea before the development of systemic sclerosis. The study concluded that systemic sclerosis and morphea are two different clinical entities that can co-exist together.

Morphea usually goes away on its own within a couple of years; however, the skin changes and the damage that occurred during the active stages of the disease will be present even after morphea is cured. Sometimes there can be serious disfigurations and skin color changes present.

Let us see what is morphea and scleroderma is.

Morphea. Morphea is a localized type of scleroderma which occurs due to excessive deposition of collagen in the deeper layers of the skin (dermis and/or subcutaneous tissue). Systemic sclerosis features such as Raynaud phenomenon, sclerodactyly, telangiectasia and involvement of the internal organs are absent in morphea. The incidence of morphea is approximately 0.4-2.7 cases per 100,000 people in the United states. It’s seen in almost all races, but common in Whites and all forms of morphea are common in females. Morphea can be seen at any age but more common from the age 2-14 years and in the mid 40’s.

There are 3 types of different sub categories of morphea, this is classified according to the patter and the depth of tissue involved.

  1. Blistering morphea
  2. Generalized morphea
  3. Deep morphea.

The exact etiology of morphea is unknown, it is said that an autoimmune status due to the increase production of autoantibodies in the body might be the reason. Most of the patients with morphea have other autoimmune disease and have positive serology tests for other auto immune diseases.

The following are believed to be some of the causes and associations

  • Radiation Therapy – morphea has occurred on sites that were given radiation therapy.
  • Chimerism – immature chimeric cells have been present in the morphea skin lesions.
  • Infections – Epstein-Barr virus, varicella virus, measles virus, and hepatitis B virus infections have been associated with morphea.

Systemic Scleroderma. Systemic scleroderma (systemic sclerosis) is a chronic connective tissue disease which is categorized under autoimmune rheumatic diseases. The incidence of scleroderma in the United States is about 20 cases per million population. Roughly 300,000 cases every year are diagnosed with scleroderma. Localized scleroderma (morphea) is more common in children and systemic scleroderma is more common in adults and overall scleroderma is seen more in females than in males (4.1)

The exact reason for scleroderma is not known but believed to be an autoimmune condition which stimulates excess collagen production. Systemic scleroderma is multi-organ disease which affects most of the organs in the body. It causes tissue scarring, abnormal connective tissue growths in muscles and joints, damages to blood vessels. It affects the skin, subcutaneous layers of the skin, muscles, joints and internal organs such as heart, lung, gastrointestinal tract and kidneys.

Conclusion

Morphea which is a localized type of scleroderma occurs due to excessive deposition of collagen in the deeper layers of the skin (dermis and/or subcutaneous tissue). Systemic scleroderma (systemic sclerosis) is a chronic connective tissue disease, which is categorized under autoimmune rheumatic diseases. It is a multi-organ disease which affects the skin, subcutaneous layers of the skin, muscles, joints and internal organs such as heart, lung, gastrointestinal tract and kidneys. Usually morphea involves the skin, subcutaneous tissue and rarely the muscles and joints. Usually internal organs are not affected by morphea and its very rare for morphea to progress into systemic scleroderma.

Also Read:

Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: March 11, 2019

This article does not provide medical advice. See disclaimer

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