Fountains in the heart-biventricular coronary cameral fistulae and bilateral coronary artery to pulmonary artery fistulae.

We describe about an elderly male presented to us with effort intolerance. He was diagnosed to have multiple coronary cameral fistulae and coronary pulmonary fistulae that gives an appearance of "Fountains In The Heart". Such a combined existence of biventricular coronary cameral fistulae and bilateral coronary artery to pulmonary artery fistulae is an unforeseen entity that has never been described before in an individual.

Pyoderma gangrenosum.

Pyoderma gangrenosum (PG) is an idiopathic, ulcerative, noninfective chronic inflammatory skin disorder of unknown etiology. It is associated with systemic medical illness in 50% of cases like inflammatory bowel disease, systemic arthritis, haematological diseases and malignancies. Characteristic lesions begin as pustule or vesiculopustule and progresses to an ulcer or deep erosion with violaceous overhanging or undermined borders. Diagnosis of pyoderma gangrenosum is clinical and depends on exclusion of other causes of cutaneous ulceration. The management of PG is treatment of underlying systemic medical illness and judicious use of immunosuppressants. Association of PG with these medical illnesses and treatment with immunosuppressants make the clinical utility for internists, gastroenterologists, haematologists and rheumatologists.

Raynaud's phenomenon.

Raynauds phenomena (RP) is a commonly encountered clinical manifestation which may be primary or secondary to underlying disease. There is imbalance between vasoconstricting and vasodilating factors. Physical examination, nailfold capillaroscopy and immunological tests can differentiate primary from secondary RP. Treatment involves prevention of RP so that permanent ischemic damage i.e. gangrene can be avoided. Avoidance of exposure to cold, emotional stress and certain drugs is mandatory and if attacks are occurring then vasodilators, prostaglandin analogues, anticoagulants and antiplatelet drugs may be added. An attempt has been made to guide the clinician to diagnose and treat a patient of RP through this article.

Pachydermoperiostosis with myelofibrosis and empty sella.

A case of pachydermoperiostosis presented to us in rheumatology clinic with complaints of pain and swelling in knee joints unresponsive to treatment, characteristic facial features, grade four clubbing of nails and broadening of distal parts of extremities. He also complained of fatiguability which was due to anemia. The natural history of the disease was reviewed and investigated.

Antiphospholipid antibody syndrome.

The 2006 International Consensus Statement on an Update of the Classification Criteria for Definite Antiphospholipid Syndrome has increased the time between the two laboratory studies required for diagnosis from 6 to 12 weeks. Antibody to beta2 glycoprotein 1 has been included as a criterion. Various non-criteria diagnostic clues such as livedo reticularis, heart valve disease, thrombocytopenia, renal thrombotic microangiopathy, neurological manifestations, non-criteria antibodies (IgA aCL, IgA anti-beta2 glycoprotein I) and some research laboratory-identified antibodies (antiphosphatidylserine antibodies, antiphosphatidylethanolamine antibodies, antibodies against prothrombin alone and antibodies to the phosphatidylserine-prothrombin complex) have been recognised. New concepts of pathogenesis now implicate complement activation and participation of the innate immune system upstream to thrombosis. Warfarin remains the treatment of choice for patients who have suffered thrombosis, but antiplatelet agents and heparin are other options. Target INR is 2.0-3.0. The other drugs which are used in resistant cases are: rituximab, hydroxychloroquine, thrombin inhibitors and statins.