A 14 year old girl on exposure to cold has pallor of extremities followed by pain and cyanosis. In later ages of life she is prone to develop ?
Scleroderma Pallor of extremities on exposure to cold that is followed by pain and cyanosis suggests Raynaud's phenomenon. A Female patient with Raynaud's phenomenon should be evaluated for possibility of scleroderma or any other connective tissue disease. Raynaud's phenomenon is the first manifestation of scleroderma in almost every patient with the disease and it preceedes other symptoms in 70% of casesQ The interval between Raynaud's phenomenon and appearance of other manifestations is generally brief (weeks to months) in diffuse scleroderma, while in limited scleroderma the period of onset between Raynaud's phenomenon and systemic manifestations is commonly several years. Scleroderma is a rare disorder but it initially presents with Raynaud's phenomenon which is quite frequent in the general population. Therefore it is impoant to keep sclerodenna in mind while dealing with Raynaud's phenomenon. More on Scleroderma : Scleroderma is primarily a disease of the female? (female to male ratio of 3 : 1) with a peak incidence in the 50 - 60 year age group. Raynaud's phenomenon occurs due to microvascular involvement?. Microvascular disease is consistently present early in the course of systemic sclerosis. Intimal proliferation is evident in 100% of digital aeries of patients with systemic sclerosis. Capillary dilatation with leaking as well as destruction is also common. Stress and cold temperature induce an exaggerated vasoconstriction of the small aeries, aeioles and aeriovenous shunts of the skin of the digits. This is manifested clinically as pallor and cyanosis of the digits followed by reactive hyperemia after rewarming. Unlike episodes of uncomplicated primary Raynaud's phenomenon, attacks of Raynaud phenomenon in patient with sclerodenria are often painful and frequently lead to digital ulcerations, gangrenes or amputation. Also know Diseases associated with Raynaud's phenomenon : Raynaud's phenomenon is associated with several connective tissue disease. Systemic sclerosis (scleroderma) Systemic lupus erythematosus Rheumatoid ahritis Sjogren syndrome Dermatomyositis Polymyositis Vasculitis All patients with history of Raynaud's phenomenon should be asked about symptoms suggestive of autoimmune disease such as ahritis, dry eyes or dry mouth, myalgias, fever, skin rash or cardiomyopathy abnormalities. If underlying autohnmune disease is suspected, the patient should be evaluated for presence of specific autoantibodies. Antinuclear antibody (ANA) assays are highly sensitive for the type of connective tissue disorders that are often associated with Raynaud's phenomenon. However, positive ANA, are quite nonspecific and therefore should be followed by testing for autoantibodies with higher positive predictive values for such conditions.
Systemic lupus erythematosis
Scleroderma
Rheumatoid ahritis
Histiocytosis
Scleroderma