Bechet's Disease
and
Arrhythmogenic Right Ventricular Dysplasia
Hmmm....I did not fit the mold!!!
Arrhythmogenic Right Ventricular Dysplasia
(ARVD)
Arrhythmogenic right ventricular dysplasia (ARVD) is a genetically inherited heart disease. ARVD is caused by a genetic defects of the parts of the heart muscle (also called myocardium or cardiac muscle) known as desmosomes, areas on the surface of the heart muscle cells which link the cells together. The desmosomes are composed of several proteins and many of those proteins can have harmful mutations.
The disease is a type of nonischemic cardiomyopathy that involves primarily the right ventricle. It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.
ARVD can be found in association with diffuse palmoplantar keratoderma, and woolly hair, in an autosomal recessive condition called Naxos Disease, because this genetic abnormality can also affect the integrity of the superficial layers of the skin most exposed to pressure stress.
ARVD/C is an important cause of ventricular arrhythmias in children and young adults. It has been seen predominantly in males, and 30-50% of cases have been familial distribution.
Behcet's Disease
(Beh-shitz)
Behcet's Disease is a rare immune-mediated small vessel systemic vasculitis that often presents with mucous membrane ulceration and ocular problems. The disease was named in 1937 after the Turkish dermatologist Hulusi Behcet, who described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis-a disease of the eye. As a systemic disease, it can also involve the visceral organs such as the gastrointestinal tract, pulmonary, musculoskeletal, cardiovascular and neurological systems. This syndrome can be fatal due to ruptured vascular aneurysms or severe neurological complications.