A 14 yrs old girl on exposure to cold has pallor of extremities followed by pain and cyanosis. In later ages of life she is prone to develp?
Ans. is 'b' i.e., Scleroderma Pallor of extremities on exposure to cold that is followed by pain and cyanosis suggests Raynaud9s phenomenon. A Female patient with Raynaud9s 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 precedes other symptoms in 70% of cases Q.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 important to keep scleroderma in mind while dealing with Raynaud's phenomenon.More on Scleroderma : - Scleroderma is primarily a disease of the female Q (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 Q.Microvascular disease is consistently present early in the course of systemic sclerosis. Intimal proliferation is evident in 100% of digital arteries 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 arteries, arterioles and arteriovenous 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 scleroderma are often painful and frequently lead to digital ulcerations, gangrenes or amputation.Also knowDiseases associated with Raynaud's phenomenon :Raynaud's phenomenon is associated with several connective tissue disease.Systemic sclerosis (scleroderma)Systemic lupus erythematosusRheumatoid arthritisSjogren syndromeDermatomyositisPolymyositisVasculitisAll patients with history of Raynaud's phenomenon should be asked about symptoms suggestive of autoimmune disease such as arthritis, dry eyes or dry mouth, myalgias, fever, skin rash or cardiomyopathy abnormalities.If underlying autoimmune 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 arthritis
Hisiocytosis
SLE