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Morphea

Morphea is an autoimmune inflammatory disease of the skin. It leads to sclerotic alterations of the skin, hence also called localized scleroderma.


Presentation

The typical presentation of individuals with morphea includes a single or a plethora of inflammatory/sclerotic plaques. Though disease activity is usually limited after an average period of five years, there are some cases of patients with more persistent episodes or a condition with relapses. Aesthetic disfigurement and/or damage that leads to a certain loss of functionality are common consequences after active disease has subsided.

Hardened skin is one of the cardinal signs of morphea. The formed lesions are round, extended patches with a subtle pink/yellow appearance, along with a purplish rim. With the progression of the disease, the affected area becomes sleek, shiny and assumes and can be mistaken for a scar.

One report of the first stages of a morphea case described the dermatologic symptoms of the patient as resembling a case of poikilodermatous mycosis fungoides (MF). Histopathological and molecular analysis also produced findings compatible with MF. However, as the condition progressed, well-defined lesions and histopathological findings indicated the presence of morphea.

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Workup

Diagnosis of morphea is usually based on clinical data. A biopsy sample from the affected area may aid to establish a definitive diagnosis. Typical findings include irregular deposition of collagen, massive vascular walls and white cell infiltration at the border of the vessels. Laboratory tests are usually unremarkable.

Treatment

As discussed earlier, there is no known effective cure for morphea. Nevertheless, a wide range of therapeutic measures are available, which can be used supportively to reduce the profound symptomatology. Drugs usually help to avoid organ injuries and lessen joint pain. Other possibilities include surgical laser intervention and light therapy, which can be attempted to aesthetically correct the appearance of the skin. When joints are affected, patients can also benefit from physiotherapy, although a little degree of joint freezing is inevitable.

In cases with advanced stage, surgical intervention might be reserved for the treatment of extensively injured organs, such as a stenosed esophagus or a gangrenous finger due to Raynaud's syndrome.

Treatment aiming at the reduction of inflammation at the initial stages of morphea has shown better results than measures applied to diminish scars at a later stage. In cases where the disease has not yet reached its final phase, local application of some balms can fight the inflammation and symptoms: creams containing corticosteroids, tacrolimus ointment such as Protopic and imiquimod cream (Aldara) are all acceptable choices. UVA phototherapy can also be beneficial.

Several new possibilities for the supportive care have recently emerged. Combinations of anti-inflammatory medications, immunomodulators or anti-fibrotic drugs have been tried. IFN-γ targets the overproduction of collagen both directly and indirectly, while stimulating the Th1 response type. Additionally, IFN-γ has beneficial effects on fibroblast reproduction, that can lead to the development of normal fibroblasts. IFN-γ could also be applied locally, because it has the capability of constructing an effective protein delivery structure and can also transport the active ingredients to the diseased tissue with no adverse effects of systemic nature.

Patients should also be advised to adapt their lifestyles accordingly: physical activity, a healthy diet, stress reduction and the protection of the skin from excessive cold or sun, may all offer important improvement of the condition. Smoking should also be avoided as it can cause considerable vascular injuries, which can worsen scleroderma.

Prognosis

The prognosis of morphea is usually good, as it seldom leads to death. However, it can affect a patient's life quality, especially in the case of pediatric patients, because of the possible disfigurement and disability; disability is mostly caused by the involvement of the subcutaneous regions in the sclerodermic areas. Linear lesions continue to exist for longer periods of time than plaque lesions.

Etiology

Morphea is a disease characterized by chronicity and its etiology is unclear to this day. It has been identified that the primary alterations involve collagen and extra-cellular matrix components, which are produced at a higher-than-normal rate and are increasingly deposited on the skin. In some occasions, morphea can extend as far as the subcutaneous tissue. It leads to disfigurement and a certain level of disability, which adversely affects the quality of life. It is rarely life threatening.

Although the pathogenetic background of localized scleroderma is unknown, it is potentially associated with borreliosis.

Epidemiology

Morphea is a relatively rare disease, that is usually diagnosed in the age of childhood or early adulthood [4] [5] [6] [7]. Its incidence has been calculated to amount to 3 per 100,000 population per year in the USA [8].

Morphea is found more often in children than in adults. Although morphea can affect individuals of any age, almost half of the patients diagnosed with it develop the disease in their childhood years [7] [8] [9]. A study that assessed patients diagnosed in the adult life and patients with early onset disease, reported that the disease was diagnosed approximately at 45 years and 10 years, respectively [6].

Morphea exhibits a predilection for female patients, as they seem to be affected by it four times more frequently than male patients do. Other studies have estimated the ratio of men to women to be around 1:2.6 [8]

Sex distribution
Age distribution

Pathophysiology

As localized scleroderma starts to develop, a great amount of mononuclear cells are influxed into the skin and adjacent blood vessels. This phenomenon has not yet been etiologically defined yet. Several studies indicate that localized scleroderma is characterized by autoimmunity. Histopathological analysis has revealed activated T-lymphocytes among the mononuclear cells, which also supports the theory of an autoimmune factor [10]. Other potential etiologic factors comprise genetic predisposition and environmental triggers [11] [12].

Following the penetration of the mononuclear cells, the skin is subject to various alterations. Small vessels underneath the epidermis are profoundly damaged, hypoperfused and exhibit considerable damage of the endothelium [13].

The endothelium displays subsequent inflammation, with a typical up-regulation of adhesion molecules, including the intercellular adhesion molecule 1 (ICAM-1) and vascular cell adhesion molecule 1 (VCAM-1). This occurs due to the activation of mediators and cytokines, for example IFN-γ, IL-1 and TNFs [14]. A particular adhesion molecule, E-selectin, has been found in an considerable number of patients suffering from morphea [15]: it influences the degree of mononuclear cells invasion. Furthermore, its presence seems to lead to an escalated number of sclerotic lesions [15]. With a reference to ICAM-1 and VCAM-1, they mediate the attachment of monocytes to the endothelial wall. ICAM-1 displays an elevation of 25% in patients with localized scleroderma and VCAM-1 an elevation of 19% [16].

All these steps lead to hardened skin, overproduction and deposition of collagen. This is the final phase of this condition, when most of the lesions are formed. It is also the phase most targeted by means of therapeutic measures.

Fibroblasts are responsible for collagenic synthesis. In morphea, they increase the rate of collagen production, possibly due to infection, damaged cells or an autoimmune process.

Prevention

There are no specific measures to prevent scleroderma, as the etiology of the condition remains unknown. Silica dust and paint thinners have been incriminated for the triggering of morphea and their avoidance is believed to prevent the development of the disease to a certain extent.

Summary

Morphea is a disease that belongs to the wider category of scleroderma. In general, scleroderma has two types: the systemic and localized one, which can be divided into other categories as well [1].

Systemic scleroderma itself features two further categories, limited systemic sclerosis and diffuse systemic sclerosis. The diffuse type involves clinically observable lesions on the thorax, face, distal and proximal extremities, whereas the limited type is restricted to the face and arms or hands. Systemic symptoms that accompany both types include Raynaud's phenomenon or the participation of internal organs in the inflammatory process.

Localized scleroderma, on the other hand, solely affects the skin and no other organs. Morphea is one of the categories of localized scleroderma [2] [3]; it is an autoimmune disease, that induces the formation of sclerotic lesions on the skin. It causes aesthetic impairment due to the sclerotic skin and disability of varying degrees, particularly in the cases where the subcutaneous regions are involved as well.

Patient Information

Morphea is an autoimmune disease that affects the skin and, in some cases, the subcutaneous tissue. The disease leads to the formation of hardened lesions on various sites of the skin; this is caused by an abnormally increased production of collagen and several other substances, which are then transported and laid down on the skin.

It is not known what causes morphea, otherwise known as scleroderma. The theory of autoimmunity, presently linked to morphea, stipulates that in some cases, the organism starts to recognize parts of the body as foreign and attacks them.

Morphea, on the other hand, does not lead to life-threatening complications and is usually limited to the skin, accompanied by some phenomena which indicate a systemic involvement. A symptoms that is usually present in the patients is Raynaud's phenomenon: fingers turn white, blue and red when they are exposed to a cold temperature. Lastly, there is no really effective treatment for scleroderma: most creams, ointments, drugs and light therapies used, aim at the alleviation of the symptoms. The disease frequently stops being active after 3 to six years.

References

Article

  1. Falanga V. Systemic sclerosis (scleroderma). In: Bolognia JL, Jorizzo JL, Rapini RP, et al. (eds). Dermatology.New York, NY: Mosby, 2003:625-32
  2. Tu JH, Eisen AZ. Scleroderma. In: Freedberg IM, Eisen AZ, Wolff K, et al. (eds). Fitzpatrick's Dermatology in General Medicine, Fifth Edition, Volume II. New York, NY: McGraw-Hill, 1999:2023-33.
  3. Marzano AV, Menni S, Parodi A, et al. Localized scleroderma in adults and children. Eur J Dermatol2003;13(2):171-6
  4. Provost TT, Greenberg AS, Falanga V. Localized cutaneous sclerosis. In: Cutaneous manifestations of rheumatic diseases, 1st ed, Sontheimer RD, Provost TT (Eds), Williams & Wilkins, Baltimore 1996. p.125.
  5. Sehgal VN, Srivastava G, Aggarwal AK, et al. Localized scleroderma/morphea. Int J Dermatol 2002; 41:467.
  6. Christen-Zaech S, Hakim MD, Afsar FS, Paller AS. Pediatric morphea (localized scleroderma): review of 136 patients. J Am Acad Dermatol 2008; 59:385.
  7. Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases. Arch Dermatol 2009; 145:545.
  8. Peterson LS, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993. J Rheumatol 1997; 24:73.
  9. Dharamsi JW, Victor S, Aguwa N, et al. Morphea in adults and children cohort III: nested case-control study--the clinical significance of autoantibodies in morphea. JAMA Dermatol 2013; 149:1159.
  10. Roumm AD, Whiteside TL, Medsger TA Jr, Rodnan GP. Lymphocytes in the skin of patients with progressive systemic sclerosis. Quantification, subtyping, and clinical correlations. Arthritis Rheum 1984;27:645–53.
  11. Feghali-Bostwick CA. Genetics and proteomics in scleroderma. Curr Rheumatol Rep 2005;7:129–34.
  12. Silman AJ, Newman J. Epidemiology of systemic sclerosis. Curr Opin Rheumatol 1996;8:585–9.
  13. Fleischmajer R, Perlish JS. Capillary alterations in scleroderma. J Am Acad Dermatol 1980;2:161–70.
  14. Gruschwitz MS, Hornstein OP, von Den Driesch P. Correlation of soluble adhesion molecules in the peripheral blood of scleroderma patients with their in situ expression and with disease activity. Arthritis Rheum 1995;38:184–9.
  15. Yamane K, Ihn H, Kubo M, et al. Increased serum levels of soluble vascular cell adhesion molecule 1 and E-selectin in patients with localized scleroderma. J Am Acad Dermatol 2000;42:64–9.
  16. Ihn H, Fujimoto M, Sato S, et al. Increased levels of circulating intercellular adhesion molecule-1 in patients with localized scleroderma. J Am Acad Dermatol 1994;31:591–5.

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Last updated: 2017-08-09 18:18