Diseases

Ichthyosis- keratosis follicularis spinulosa decalvans

Keratosis follicularis spinulosa decalvans (KFSD) is a rare, inherited, skin disorder that affects men predominately and is characterized by hardening of the skin (keratosis) in several parts of the body. Most frequently, the face, neck, and forearms are involved. The thickening of the skin is accompanied by the loss of eyebrows, eyelashes and beard. Baldness (alopecia) usually occurs.

Idiopathic acute eosinophilic pneumonia

Eosinophilic pneumonia (EP) is a disease in which a certain type of white blood cell called an eosinophil accumulates in the lung. These cells cause disruption of the normal air spaces (alveoli) where oxygen is extracted from the atmosphere. Several different kinds of eosinophilic pneumonia exist and can occur in any age group. The most common symptoms include cough, fever, difficulty breathing, and sweating at night. EP is diagnosed by a combination of characteristic symptoms, findings on a physical examination by a health provider, and the results of blood tests and x-rays. Prognosis is excellent once most EP is recognized and treatment with corticosteroids is begun

Idiopathic atypical mycobacterial infection

Atypical mycobacteria are a group of bacteria that are widely distributed in nature. They can be found in water, soil, animals and man, usually without evidence of disease.

Idiopathic basal ganglia calcification 1

Basal ganglia calcification, idiopathic 1: Abnormal calcium deposits in the part of the brain called the basal ganglia. Type 1 results in psychiatric, cognitive or neurological problems associated with the calcification. The symptoms experienced are variable

Idiopathic CD4 Lymphopenia

Idiopathic CD4 lymphopenia is a rare and heterogeneous syndrome defined by low levels of CD4 T cells in the absence of HIV infection, which predisposes patients to infections and malignancies.Idiopathic CD4 lymphopenia is a rare and heterogeneous syndrome defined by low levels of CD4 T cells in the absence of HIV infection, which predisposes patients to infections and malignancies.

Idiopathic dilation cardiomyopathy

Idiopathic dilation cardiomyopathy: A rare chronic heart muscle condition where one or both heart ventricles are dilated and the heart is unable to contract normally. In idiopathic forms, the condition occurs for no apparent reason

Idiopathic double athetosis

Idiopathic double athetosis: A rare nervous system disorder characterized by slow, writing, involuntary movements which involves both sides of the body at the same time. It occurs for no apparent reason.

Idiopathic edema

Idiopathic edema is a pitting edema of unknown cause that occurs primarily in pre-menopausal women who do not have evidence of heart, liver, or kidney disease. In this condition, the fluid retention at first may be seen primarily pre-menstrually (just prior to menstruation), which is why it sometimes is called "cyclical" edema. However, it can become a more constant and severe problem. Patients with idiopathic edema often take diuretics to decrease the edema in order to lessen the discomfort of bloating and swelling. Paradoxically, however, the edema in this condition can become more of a problem after the use of diuretics. The patients can develop fluid retention as a rebound phenomenon each time they discontinue diuretics. It is important to talk to your doctor before using any diuretics.

Idiopathic eosinophilic chronic pneumopathy

Idiopathic eosinophilic chronic pneumopathy: A rare disorder where eosinophils (a type of white blood cell) accumulate in the lungs and cause problems. The symptoms occur gradually over a period of weeks or even months.

Idiopathic facial palsy

Idiopathic facial palsy: Weakness or paralysis of the facial muscles that occurs for no apparent reason. The condition is usually temporary and tends to resolve itself with the majority recovering fully within three weeks and the rest within a year. Usually only one side of the face is affected.

Idiopathic hypereosinophilic syndrome

Hypereosinophilic syndrome is a group of rare blood disorders characterized by increased levels of eosinophils (a type of white blood cell that plays a role in the human immune system) persisting for more than six months. The signs and symptoms are due to involvement of several internal organs and there is usually no evidence of parasites, allergy, or other known causes of an elevated eosinophil count. This condition largely occurs in males, typically at middle age. It usually presents with fever, weight loss, fatigue, and rash. An enlarged liver and spleen and liver is often present. The lungs, kidneys, heart, and nervous system can also be affected.

Idiopathic minimal change nephrotic syndrome

Idiopathic minimal change nephrotic syndrome: A rare kidney disorder which has no apparent cause. The filtering structures of the kidneys are only slightly damaged and appear to be almost normal. Progression to kidney failure is very rare.

Idiopathic myopathy

Idiopathic myopathy: A rare condition involving inflammation of the skeletal muscles which become weak and wasted.

Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive decline in lung function. The term pulmonary fibrosis means scarring of lung tissue and is the cause of worsening dyspnea (shortness of breath). Fibrosis is usually associated with a poor prognosis.

The term 'idiopathic' is used because the cause of pulmonary fibrosis is still unknown. IPF usually occurs in adult individuals of between 50 and 70 years of age, particularly those with a history of cigarette smoking, and affects more men than women. The diagnosis of IPF requires exclusion of other known causes of ILD and the presence of a typical radiological pattern identified through high resolution computed tomography (HRCT). In the right clinical setting, it is possible to make the diagnosis of IPF by HRCT alone, obviating the need for surgical lung biopsy.

IPF belongs to a large group of more than 200 lung diseases known as interstitial lung diseases (ILD), characterized by the involvement of lung interstitium. The interstitium, the tissue between the air sacs in the lung, is the primary site of injury in ILDs. However, these disorders frequently affect not only the interstitium, but also the airspaces, peripheral airways, and vessels. Lung tissue from people with IPF shows a characteristic histopathologic pattern known as usual interstitial pneumonia (UIP). UIP is therefore the pathologic counterpart of IPF.

Idiopathic pulmonary hemosiderosis

Idiopathic pulmonary hemosiderosis: A rare disorder involving bleeding into the lungs which can eventually cause damage to the lungs.

Idiopathic sclerosing mesenteritis

Idiopathic sclerosing mesenteritis: A rare disorder that affects the lining of the digestive tract. The lining of the small bowel mesentery (membrane that joins small intestine to back of abdominal wall) becomes thickened and inflamed. The condition may be mild or severe enough to cause death. The idiopathic form of the condition occurs for no apparent reason.

Idiopathic subglottic tracheal stenosis

Idiopathic subglottic stenosis, also known as Idiopathic laryngotracheal stenosis or ILTS, is a narrowing of the portion of the trachea below the glottis (voice box).

Congenital stenosis has two main types, membranous and cartilaginous.

In membranous stenosis, fibrous soft tissue thickening is caused by increased connective tissue or hyperplastic dilated mucus glands with absence of inflammation. Membranous stenosis is usually circumferential and may extend upward to include the true vocal folds.

In cartilaginous stenosis, a thickening or deformity of the cricoid cartilage most commonly occurs, causing a shelflike plate of cartilage and leaving a small posterior opening. Cartilaginous stenosis is less common than membranous stenosis.

 

Source: Medscape, Rightdiagnosis

 

Idiopathic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura is a rare disorder that causes blood clots (thrombi) to form in small blood vessels throughout the body. These clots can cause serious medical problems if they block vessels and restrict blood flow to organs such as the brain, kidneys, and heart. Resulting complications can include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal kidney function, abdominal pain, and heart problems.

Blood clots normally form to prevent excess blood loss at the site of an injury. In people with thrombotic thrombocytopenic purpura, clots develop in blood vessels even in the absence of injury. Blood clots are formed from clumps of cell fragments called platelets, which circulate in the blood and assist with clotting. Because a large number of platelets are used to make clots in people with thrombotic thrombocytopenic purpura, fewer platelets are available in the bloodstream. A reduced level of circulating platelets is known as thrombocytopenia. Thrombocytopenia can lead to small areas of bleeding just under the surface of the skin, resulting in purplish spots called purpura.

This disorder also causes red blood cells to break down (undergo hemolysis) prematurely. As blood squeezes past clots within blood vessels, red blood cells can break apart. A condition called hemolytic anemia occurs when red blood cells are destroyed faster than the body can replace them. This type of anemia leads to paleness, yellowing of the eyes and skin (jaundice), fatigue, shortness of breath, and a rapid heart rate.

There are two major forms of thrombotic thrombocytopenic purpura, an acquired (noninherited) form and a familial form. The acquired form usually appears in late childhood or adulthood. Affected individuals may have a single episode of signs and symptoms, or they may recur over time. The familial form of this disorder is much rarer and typically appears in infancy or early childhood. In people with the familial form, signs and symptoms often recur on a regular basis.

Risk factors:
Idiopathic thrombocytopenic purpura can occur in anyone at almost any age, but these factors increase the risk:

  • Your sex. Women are two to three times more likely to develop ITP than men are.
  • Recent viral infection. Many children with ITP develop the disorder after a viral illness, such as mumps, measles or a respiratory infection.

Complications:
A rare complication of idiopathic thrombocytopenic purpura is bleeding into the brain, which can be fatal.

Pregnancy:
In pregnant women with ITP, the condition doesn't usually affect the baby. But the baby's platelet count should be tested soon after birth.

If you're pregnant and your platelet count is very low or you have bleeding, you have a greater risk of heavy bleeding during delivery. In such cases, you and your doctor may discuss treatment to maintain a stable platelet count, taking into account the effects on your baby.

IgA Nephropathy

IgA nephropathy (IgAN, also known as IgA nephritis, Berger disease (and variations), or synpharyngitic glomerulonephritis), is a disease of the kidney (or nephropathy), specifically it is a form of glomerulonephritis or an inflammation of the glomeruli of the kidney.

IgA nephropathy is the most common glomerulonephritis worldwide. Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus. There are other diseases associated with glomerular IgA deposits, the most common being IgA vasculitis (formerly known as Henoch–Schönlein purpura [HSP]), which is considered by many[citation needed] to be a systemic form of IgA nephropathy. HSP presents with a characteristic purpuric skin rash, arthritis, and abdominal pain and occurs more commonly in young adults (16–35 years old). HSP is associated with a more benign prognosis than IgA nephropathy. In IgA nephropathy there is a slow progression to chronic kidney failure in 25–30% of cases during a period of 20 years.