Reddish-brownish lesions on the left mandibular region with recalcitrant cutaneous sarcoidosis (Photo credit: Wikipedia)
Wikipedia The Free Encylopedia:
Sarcoidosis is a systemic inflammatory disease that can affect any organ. Common symptoms are vague, such as fatigue unchanged by sleep, lack of energy, weight loss, aches and pains, arthritis, dry eyes, swelling of the knees, blurry vision, shortness of breath, a dry hacking cough or skin lesions. Sarcoidosis and cancer may mimic one another, making the distinction difficult.[4] The cutaneous symptoms vary, and range from rashes and noduli (small bumps) to erythema nodosum or lupus pernio. It is often asymptomatic.
The combination of erythema nodosum, bilateral hilar lymphadenopathy and arthralgia is called Löfgren syndrome. This syndrome has a relatively good prognosis.
Renal, liver (including portal hypertension), heart[5] or brain involvement may cause further symptoms and altered functioning.
Lungs
Pulmonary localization is by far the most common of individuals with sarcoidosis. 90 percent of patients have an abnormal chest x-ray at some time during their course. Overall, approximately 50 percent develop permanent pulmonary abnormalities and 5 to 15 percent have progressive fibrosis of the lung parenchyma. Sarcoidosis of the lung is primarily an interstitial lung disease in which the inflammatory process involves the alveoli, small bronchi, and small blood vessels. In acute and subacute cases, the physical examination usually reveals dry rales.[6]
Liver
Although liver biopsy reveals liver involvement in 60 to 90 percent of cases, liver dysfunction is usually not important clinically. Approximately 20–30% have hepatomegaly and/or biochemical evidence of liver involvement. Usually these changes reflect a cholestatic pattern and include an elevated alkaline phosphatase level; the bilirubin and aminotransferases are only mildly elevated. Jaundice is rare.[6]
Skin
Sarcoidosis involves the skin in about 25 percent of patients. The most common lesions are erythema nodosum, plaques, maculopapular eruptions, subcutaneous nodules, and lupus pernio. Treatment is not required, since the lesions usually resolve spontaneously in 2 to 4 weeks. Although it may be disfiguring, cutaneous sarcoidosis rarely causes major problems.[6]
Heart
Although cardiac involvement is present in 20% to 30% of patients with sarcoidosis, only about 5% of patients with systemic sarcoidosis are symptomatic.[7] The presentation of cardiac sarcoidosis can range from asymptomatic conduction abnormalities to fatal ventricular arrhythmia.[8] Myocardial sarcoidosis can be a rare cause of sudden cardiac death.[9][10] Recently, retired hockey player Gaetano Orlando received a “total artificial heart” to replace ventricles damaged by sarcoidosis.
Eye
Manifestations in the eye include uveitis, uveoparotitis, and retinal inflammation, which may result in loss of visual acuity or blindness. The combination of anterior uveitis, parotitis, VII cranial nerve paralysis and fever is called uveoparotid fever, and is associated with Heerfordt-Waldenstrom syndrome. (D86.8)
Blood
Abnormal clinical blood tests are frequent but not diagnostic. Anemia occurs in 4-20% of patients with sarcoidosis. Leukopenia (due to a reduced number of circulating lymphocytes [11] or lymphopenia) occurs in as many as 40% of patients but is rarely severe. In the absence of splenomegaly, leukopenia may reflect bone marrow involvement, however, the most common mechanism is a redistribution of blood T cells to sites of disease.[12] Other non-specific findings include monocytosis, occurring in the majority of sarcoidosis cases,[13] increased hepatic enzymes or alkaline phosphatase. Hypercalciuria and hypercalcemia are seen in <10% of patients.[14]
Lymph nodes
Lymphadenopathy is very common in sarcoidosis. Intrathoracic nodes are enlarged in 75 to 90 percent of all patients; usually this involves the hilar nodes, but the paratracheal nodes are commonly involved. Peripheral lymphadenopathy is very common, particularly involving the cervical (the most common head and neck manifestation of the disease [15]), axillary, epitrochlear, and inguinal nodes. Palpation causes no pain.[6]
Nervous system
All components of the nervous system can be involved in sarcoidosis. Sarcoidosis affecting the brain or nerves is known as neurosarcoidosis. Neurologic findings are observed in about 5 percent of patients. Cranial nerves are predominantly affected, and peripheral facial nerve palsy, often bilateral, is the most common neurological manifestation of sarcoidosis.[16] It occurs suddenly and is usually transient. Other common manifestations of neurosarcoid include optic nerve dysfunction, papilledema, palate dysfunction, hearing abnormalities, hypothalamic and pituitary abnormalities, chronic meningitis, and peripheral neuropathy.[6] Intramedullary sarcoidosis is rare and occurs in less than 1% of cases. There is usually granulomatous involvement of the basal meninges that subsequently affects the cranial nerves. Myelopathy may be the initial clinical presentation of intramedullary neurosarcoidosis.[17] Read more
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