Acquired coronary artery fistula: a sign of mural thrombus

This is a case report documenting an acquired coronary artery fistula from the left anterior descending artery through a mural thrombus into the left ventricle by serial coronary arteriography. This abnormality may be an early clue to mural thrombus formation and help provide guidance in making therapeutic anticoagulation decisions.     

Primary anetoderma: a cutaneous sign of antiphospholipid antibodies

Although a few reports in recent years have suggested that patients with antiphospholipid antibodies (aPL) are prone to developing primary anetoderma (PA), it is still unclear how often aPL are detected in unselected PA patients. We studied nine consecutive PA patients for the presence of autoimmune antibodies and disorders in general and the presence of aPL in particular. Six of the nine patients had clinical evidence of associated autoimmune disorders (Graves'disease and autoimmune haemolysis in one, systemic scleroderma in one, Hashimoto's thyroiditis in one, alopecia areata in one) and/or signs of hypercoagulability (recurrent fetal loss in two, recurrent stokes in one, recurrent deep vein thrombosis in one). In four ofthese six patients the onset of PA preceded these signs. Positive aPL was found in all: anticardiolipin (aCL) in six, anti-beta2-glycoprotein-I (a(beta)2GPI) in six and lupus anticoagulant (LAC) in four. The most frequent isotype was IgA. Among other autoantibodies found the most frequently was antinuclear antibodies. Four ofthe nine patients fulfilled the criteria for antiphospholipid syndrome (APS). It is concluded that PA is an important cutaneous sign for autoimmune disorders in general and the presence of aPL in particular. Hence, the work-up of these patients should include testing for LAC as well as for all different isotypes ofaCL and a(beta)2GPI. We recommend that PA be added to the list of the cutaneous manifestations of APS.     

Acquired ichthyosis and livedoid palmoplantar keratoderma: two unusual skin manifestations of systemic lupus erythematosus

Acquired ichthyosis and livedoid palmoplantar keratoderma have rarely been described in systemic lupus erythematosus (SLE). In the first case, a 51-year-old man presented with generalized acquired ichthyosis associated with renal glomurelosclerosis and neurolupus. rituximab allowed total resolution of the acquired ichthyosis. A livedoid palmoplantar keratoderma was observed in a 45-year-old woman as the initial clinical manifestation of a systemic lupus erythematosus associated with Raynaud's phenomenon, photosensitivity and inflammatory polyarthralgia. Although the pathophysiology is not well-defined, these unusual cutaneous manifestations should be considered by physicians when diagnosis of SLE is suspected.     

Acquired neuromyotonia: a new autoantibody-mediated neuronal potassium channelopathy

Neuromyotonia (Isaacs syndrome) is a rare and heterogenous syndrome of continuous motor unit activity of peripheral nerve origin that manifests as various combinations of muscle stiffness, cramps, twitching, weakness, and delayed muscle relaxation. Although neuromyotonia may accompany an assortment of inherited diseases, most cases are acquired. The observation that the acquired form is often associated with an autoimmune disorder, and the demonstration that some cases improve after plasma exchange, has led to a search for an immune-mediated etiology. This review summarizes the recent immunological and electrophysiological evidence that autoantibodies to voltage-gated potassium channels produce the peripheral motor nerve hyperexcitability that characterizes neuromyotonia and thus establishes acquired neuromyotonia as an autoantibody-mediated disorder. In the nervous system, ion channels and neurotransmitter receptors that function as ligand-gated ion channels seem to be favored targets for autoantibody attack, and neuromyotonia can now be added to the growing list of autoimmune channelopathies.     

A new diagnostic sign for rheumatoid arthritis: Avicenna sign

Aim: The status of a new sign for diagnosing rheumatoid arthritis is discussed for the first time in this study. It is defined as marked tenderness on the anterior surface of leg (shin) on pressure of about 4 kg by thumb. The sign has been denoted as ‘Avicenna sign’. Methods: All cases were selected by convenient sampling from patients who were referred to two rheumatology clinics in Tabriz, Iran. Sample size was calculated as 618 using Cochran's sampling equation. Diagnosis of rheumatoid arthritis was made according to American Rheumatism Association criteria. Two physicians were asked to examine the sign on all of the selected patients separately. One of them (a general physician) was not informed about methodology and objectives of the study, whereas a rheumatologist was well informed and he collected data using a written questionnaire. Test–retest method and discriminant analysis were used to test reliability and validity of the sign for diagnosing rheumatoid arthritis. The effects of intervening variables including osteoporosis and consumption of some medications were controlled employing Chi‐square test. Results: When the general physician examined patients with Avicenna sign to diagnose rheumatoid arthritis, sensitivity and specificity of the sign were reported as 77.1% and 98.4%, respectively. The corresponding values were 81.3% and 98.4%, respectively, when the rheumatologist examined the patients. Conclusions: This study suggested considerable diagnostic value of the sign for rheumatoid arthritis. Consumption of acetaminophen or NSAIDs had no effect on the status of Avicenna sign while taking gluccocorticoids made it unreliable for diagnostic purposes.     

Sharma's parachute sign a new laparoscopic sign in abdomino pelvic tuberculosis

AimsTo demonstrate a new laparoscopic sign “Sharma's Parachute sign” in abdominopelvic tuberculosis in women with infertility.MethodsA total of 104 women who were diagnosed to have abdominopelvic tuberculosis, on endometrial sampling or on laparoscopy were enrolled in this ongoing study on tuberculosis in infertility. A new laparoscopic “Sharma's parachute sign” was looked for in these cases on laparoscopy.ResultsThe mean age, pairty and duration of infertility was 27.6 years, 0.58 and 4.1 years respectively. Menstrual dysfuction were common especially hypomenorrhoea (34.61%), oligomenorrhoea (36.53%) along with constitutional symptoms and abdomino pelvic pain or lump. Diagnosis of abdominopelvic tuberculosis was made by identification of acid fast bacilli (AFB) on microscopy or culture of endometrial aspirate or peritoneal biopsy or positive gene Xpert or positive polymerase chain reaction (PCR) or histopathological demonstration of epithelioid granuloma on endometrial or peritoneal biopsy, various laparoscopic findings on pelvic and abdominal organs were tubercles and shaggy areas (white deposits, caseous nodules encysted ascites, abdominal and pelvic adhesions, tubal findings (hydrosalpinx, pyosalpinx, beaded or calcified tubes). A new “Sharma's parachute sign”in which ascending colon was totally adherent to anterior abdominal wall with its mesocolon looking like an open parachute with small caseous nodule was seen in 11 (10.5%) cases.ConclusionDiagnostic laparoscopy is an important investigation for abdominopelvic tuberculosis showing various adhesions including new parachute sign.     

Managing ichthyosis: a case study

Ichthyoses-- rare, genetic, incurable dermatologic diseases characterized by dry, thickened, scaling skin-- affect more than 1 million Americans and can cause devastating disfigurement with numerous physical, social, and emotional consequences. Topical treatments typically focus on symptom management. A 44-year-old man presented with severe X-linked type ichthyosis. When his condition did not improve with the use of a variety of topical and systemic treatments, a once-a-day, over-the-counter skin care regimen consisting of a surfactant-free cleanser followed by the application of moisturizer containing amino acids, vitamins, antioxidants, and methylsulfonylmethane was prescribed. After 4 weeks of using this management regimen on a test area on his left arm, the skin was clear and discomfort and itching had subsided. Subsequent application of the skin care products on other affected areas also did not cause any adverse reactions and his skin continued to improve. Symptom resolution and patient satisfaction indicate the product line may be a viable consideration for use in patients with similarly compromised skin.     

Acquired diseases of aberrant subclavian arteries

The anatomic, clinical, and radiologic findings of aberrant subclavian arteries (ASAs) are well known. Acquired diseases such as aneurysms, dissections, or stenosis can complicate these anomalies. We propose that these complications are a result of atherosclerosis caused by abnormal turbulence around the ASA. Comparisons of computed tomographic and angiographic measurements of subclavian arteries in adults with and without arch anomalies and angiographic measurements of ASAs in children show that ASAs dilate in older patients. We describe six patients with acquired diseases of ASAs or the aorta adjacent to the ASA. Aneurysms, aortic dissections, and stenosis were each found in two patients. Problems in the radiologic diagnosis and clinical management of these diseases are emphasized. Radiologists should be cognizant of the significance of ASAs in patients with aortic aneurysms and dissections.     

"Coated aorta": a new sign of Erdheim-Chester disease

Erdheim-Chester disease is a rare, non-Langerhans cell form of histiocytosis characterized by osteosclerosis of the metaphyseal regions of long bones, diabetes insipidus, proptosis, and retroperitoneal fibrosis. The latter usually involves the perirenal area and leads to hydronephrosis. Periaortic fibrosis is less frequent. We describe 3 unusual cases of Erdheim-Chester disease with periaortic fibrosis involving the whole aorta and leading to a "coated aorta" appearance on computed tomography scans. Faced with such a singular "coated aorta," bone scintigraphy can be very helpful when searching for Erdheim-Chester disease.     

Thoracic succussion splash: a new symptom and sign of achalasia

A patient with a "thoracic succussion splash" due to achalasia is described. She noted a splashing or sloshing sensation in her chest related to jogging and bending. On examination a splashing sound could be heard over the mid sternum and the posterior chest when the patient was rocked vigorously back and forth.