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Scleroderma - Pictures, Symptoms, Causes, Diagnosis, Treatment

Updated on December 22, 2013

Pictures

What is Scleroderma?

Scleroderma is a complex disease and is a group of disease with similar set of symptoms and involves the skin and connective tissues characterized by its hardening and tightening. It is also a disease that can also result to problems in the blood vessels, scarring of the tissue and an unstable degree of inflammation while the disease also involves the immune system. Scleroderma is disease that can affect anyone at any age level without racial predilection although the disease is more common in women ad with the peak onset between the ages 30 to 50 years. It is not a contagious disease and mostly occurs in an individual without history of the disease in the family. The prognosis for scleroderma in limited form is rather positive in outlook except that the disease can hinder the everyday activity of an individual while affecting the self-esteem. The limited scleroderma o the other hand still needs close monitoring and consideration of the overall health status of the individual to further monitor for its progression and complications which may involve the internal organs especially the lungs and may pose as life-threatening if proper medical intervention has not been applied. The prognosis on systemic form however, has poor or negative prognosis especially when the onset of the disease is during the later years in life or in late adulthood at the time of diagnosis.

Symptoms

The symptoms of scleroderma vary depending on the involved organ and skin while the symptoms differ from one person to another. However, scleroderma has been divided into to two major forms and classified according to the location of skin or organs involved and according to degree of the disease. Each form are also subdivided and classified according to the extent of the disease and areas of involvement.

Localized Scleroderma

This is a form of scleroderma that only affects the skin although in some cases, the muscles beneath the skin can also be affected with the connective tissue hardening. It is a non-life threatening form without generally affecting the internal organs. This form is usually less serious and usually resolves overtime although damage can be done as a result of scar in left by the hardening of the connective tissue after the disease has receded. Localized scleroderma on the other hand is subdivided according to the presentation and areas of the affected:

  • Linear scleroderma is common in children while this type is marked by bands of thick skin usually found in the extremities, stomach, buttocks and forehead.
  • Localized morphea is characterized by oval shaped reddish patches of inflammation that can be located in the chest or in the back and may sometimes develop in the face and arms.
  • Generalized morphea is a formation of dark colored and large patches of inflammation and more severe than the localized morphea although this type is less common while these patches can be located in the trunk, legs and arms.

Systemic Scleroderma

This is the hardening of connective tissues that involve not only the skin but also involves the internal organs and blood vessels. It affects multiple system of the body and is regarded as the most dangerous of scleroderma with majority of patient in this form suffer from Raynaud’s syndrome which is the key symptom of this form of scleroderma. It is subdivided into two categories classified according to the areas of involvement and severity although both of which are marked by severe inflammation and fibrosis including vascular injury. The manifestation of this scleroderma is referred as CREST which is an acronym that stands for the significant symptoms.

  • Calcinosis is the formation of painful and hard deposits in the skin commonly in the fingers, elbow or knees
  • Raynaud’s syndrome is the symptom that shows blanching of feet and hands in reaction to cold or stress where the skin color transforms from white to blue then red.
  • Esophagus dysfunction resulting from internal scarring and poor function of the muscle of the esophagus that patient find it difficult to swallow while allowing backflow of acid into the stomach resulting to heartburn
  • Sclerodactyly is characterized by tightening of the skin in the fingers and toes and later extends to the face, hands, neck and feet
  • Telangiecstasia is the formation of tiny red spots on the palm of the hand, face, lips, fingers and forearms while these red spots blanch when pushed

Systemic scleroderma is also subdivided into two categories and these are:

Limited scleroderma is a slow progressing type of the disease although it is still life-threatening due for potentials in serious complications.

Diffuse scleroderma is marked by internal inflammation during the early phase and skin hardening covers large areas of the body including the extremities.

Causes

The exact cause of scleroderma is unknown although genetic predisposition has been implicated and evidenced. Excessive production and accumulation of collagen in the tissue of the body is the reason for the development of scleroderma. The trigger for this atypical overproduction of collagen remains unknown and immune system of the body has also been implicated to play a role in this disease. Environmental factors are also considered to have a role in the disease such as exposure to certain drugs, dust and organic solvents.

Diagnosis

Scleroderma is rather difficult to diagnose although it is basically based on the clinical presentation in which the skin involvement is being evaluated while determining any internal organ involvement including life-threatening complications.

Blood tests are also being recommended especially in detecting the presence of autoimmunity antinuclear antibodies which is mostly present in the blood samples of majority of scleroderma patients.

Treatment

No direct treatment has been relevant in the cure of scleroderma or in impeding the overproduction of collagen in the body tissue. Some case of localized scleroderma usually resolves without medical intervention while other types of scleroderma are treated for its symptoms to relieve and prevent progression to serious medical complications.

Medications used for dilating blood vessels are given to prevent complications in the lungs and kidneys while also treating the symptom of Raynaud’s syndrome. Some symptoms of scleroderma are being addressed through drugs meant in suppressing the immune system. Strength and mobility can be helped with physical and occupational therapy while surgical amputation is inevitable if gangrene has developed.

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