Morphea

Morphea is a medical phrase for localized scleroderma. The disease involves isolated patches of hardened skin there usually is no internal organ involvement. Morphea, also known as contained scleroderma, is a thickening and hardening of the skin and subcutaneous tissues from excessive collagen deposition. Morphea includes exact conditions ranging from very small plaques only connecting the skin to widespread disease causing functional and cosmetic deformities. Morphea discriminates from systemic sclerosis by its supposed require of internal organ involvement. Morphea is an uncommon situation that is thought to affect 1 in 1,000 people. Adequate studies on the incidence and prevalence have not been performed.

Morphea also may be under-reported as physicians may be unaware of this disorder and smaller morphea plaques may be less often referred to a dermatologist or rheumatologist. As in many other connective tissue or autoimmune disorders, morphea mainly involves women with a W:M ratio of 3:1. Physicians and scientists don’t know what causes morphea. Case reports and observational studies suggest there is a higher occurrence of family history of autoimmune diseases in patients with morphea. Tests for autoantibodies associated with morphea have shown results in higher frequencies of anti-histone and anti-topoisomerase IIa antibodies.

Case information of morphea co-existing with other systemic autoimmune diseases such as primary biliary cirrhosis, vitiligo, and systemic lupus erythematosus lend support to morphea as an autoimmune disease. Throughout the years, several different treatments have been tried for morphea including topical, intra-lesional, and systemic corticosteroids. Antimalarials such as hydroxychloroquine or chloroquine had been used. Other immunomodulators such as methotrexate, topical tacrolimus, and penicillamine has been tried. Ultraviolet A (UVA) light, with or without psoralens have also been tried. UVA-1, a more exact wavelength of UVA light, is able to penetrate the deeper portions of the skin.

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Idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura (ITP) is the condition of having a short platelet count of no known cause. As most causes appear to be associated to antibodies against platelets, ITP is also recognized as immune thrombocytopenic purpura or immune-mediated thrombocytopenic purpura. Often ITP is asymptomatic, however a very little platelet count can lead to visible symptoms, such as purpura, or more seriously, bleeding diathesis. In many cases, ITP's cause is not idiopathic but autoimmune, with antibodies beside platelets being detected in approximately 60 percent of patients. Most often these antibodies are against platelet covering glycoproteins IIb-IIIa or Ib-IX, and are of the IgG type. The famous Harrington Hollingsworth experiment recognized the immune pathogenesis of ITP.

The covering of platelets with IgG renders them liable to opsonization and phagocytosis by splenic macrophages. The IgG autoantibodies are also thought to injure megakaryocytes, the precursor cells to platelets, but this is thought to contribute only slightly to the reduce in platelet numbers. The stimulus for auto-antibody making in ITP is probably abnormal T cell activity Preliminary findings suggest that these T cells can be influenced by drugs that target B cells, such as rituximab. The diagnosis of ITP is a procedure of exclusion. First, the clinician has to determine that there are no blood abnormalities other than small platelet count, and no physical signs except for signs of bleeding. Then, the secondary reason should be excluded. Secondary causes could be leukemia, medications, lupus erythematosus, cirrhosis, HIV, hepatitis C, inborn causes, and others.

In approximately one percent of cases, autoimmune hemolytic anemia and ITP coexist, a state referred to as Evans syndrome. A platelet count below 20,000 is usually an indication for treatment. Patients with a count between 20,000 and 50,000 are usually estimated on a case-by-case basis, and, with rare exceptions, there is usually no need to treat patients with a count above 50,000. Hospitalization may be recommended in cases of very low counts, and is highly desirable if the patient presents with significant internal or mucocutaneous bleeding. A count below 10,000 is potentially a medical emergency, as the patient may be susceptible to subarachnoid or intracerebral hemorrhage as a result of moderate head trauma. In most cases, treatment will be administered under the way of a hematologist.

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Genetic fault Tied to Autoimmune Diseases

Rare variations in a single gene can lead to a wide variety of autoimmune disorders, including diabetes, lupus and rheumatoid arthritis, a new study shows. The gene in question encodes an enzyme called sialic acid acetylesterase or SIAE, which regulates the activity of the protected system’s antibody-producing B cells. About 2 percent to 3 percent of people with autoimmune disorders have defects in the enzyme that allow B cells to run amok and make antibodies that attack the body.

A team led by Shiv Pillai of Massachusetts General Hospital in Charlestown and Harvard Medical School reports online June 16 in Nature. “It’s a seminal paper because it is so applicable to a wide multiplicity of autoimmune diseases, says Judy Cho, a Yale geneticist not associated with the study. The finding suggests that enhancing the enzyme’s activity could help pleasure disease in people with autoimmune disorders.

Previously, Pillai’s group showed that mice missing SIAE develop a lupuslike disease in which high levels of antibodies attack the body’s own proteins. The researchers decided to examine the enzyme in people who, like the mice, make high levels of autoimmune antibodies. “One hundred percent, I was sure that we would find nothing,” Pillai says.


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