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HANDBOOK OF PHYSIOLOGY 



CIRCULATION II 



heightened tone. Thus, there are many problems in 

 Raynaud's disease which await clarification. 



Secondary Raynaud's syndrome or phenomenon. A glance 

 at the classification of peripheral vascular disease 

 (see Appendix to this chapter) reveals that Ray- 

 naud's phenomenon occurs as a secondary mani- 

 festation in a number of disease states. The proposed 

 mechanisms invoked in these entities are complex 

 and a description of these is beyond the scope of the 

 present discussion. Further, little definitive work has 

 been done to determine the true physiologic mecha- 

 nisms responsible for the relationship between these 

 diseases and secondary Raynaud's syndrome. In 

 general, what has been said regarding primary 

 Raynaud's phenomenon may be applied to the 

 secondary phenomenon. There are a lew known 

 differences. In the secondary state the associated 

 manifestations of the primary predisposing disease 

 are evident. With obliterative arteriosclerotic endarte- 

 ritis, Buerger's disease, or other obliterative arterial 

 states, the degree of gangrene associated with second- 

 ary Raynaud's phenomenon may be considerably 

 more than in primarv Raynaud's because of the 

 underlying obliterative disease. In the secondary 

 state, exposure to cold or emotional stress may or 

 may not precipitate Raynaud's phenomenon. Lastly, 

 secondary Raynaud's phenomenon is frequently 

 neither bilateral nor symmetrical. 



It is often suggested that the observation of Ray- 

 naud's phenomenon occurring in diseases of the 

 nervous system refutes Lewis' idea of a local arterial 

 defect and that primary Raynaud's disease is a 

 disease of neural origin. Even though this problem 

 is unsettled, it must be remembered that differences 

 between primary and secondary Raynaud's pheno- 

 menon do exist. Further, Raynaud's phenomenon 

 from neural disease still might be due to excessive 

 sympathetic nervous activity in the presence of 

 "normal" digital vessels, whereas primary Raynaud's 

 disease might be due to a local vessel defect in the 

 presence of normal sympathetic nerve activity. 



acrocyanosis. Acrocyanosis is a disorder character- 

 ized bv a persistent cyanotic rubor to the skin of the 

 hands and feet and other acral portions of the bodv 

 associated with a reduced skin temperature. The term 

 "acrocyanosis" was first applied by Crocq (16) in 

 iHi)ti, and Cassirer's description (12) in 191 2 helped 

 clarify this disorder as a distinct clinical entity. Lewis 

 & Landis (50) initiated basic investigations into the 

 pathophysiology of this disease, but only a few con- 



tributions to its mechanisms have been available 

 since. 



The etiology of acrocyanosis is unknown. In his 

 large series of several hundred patients, Stern (94) 

 was unable to detect any constant precursor or accom- 

 paniment of the disorder other than cold, with fre- 

 quent moderate cooling of affected parts, and possibly 

 inactivity, the latter because of the occurrence in 

 lethargic types of mental disorders. It is much more 

 frequent in females and usually present in young or 

 middle-aged individuals. There is frequently a family 

 history of the disorder. It has been described as being 

 rare in the general population but rather common 

 among the inmates of mental institutions. 



The patient usually visits the doctor for cosmetic 

 reasons, complaining of almost constant coldness and 

 bluish discoloration ol the fingers, hands, nose, 

 cheeks, chin, and pinna of several years' duration 

 (fig. 4). The toes and feet may be involved, but usually 

 to a lesser degree than the hands. The changes, 

 though present during the summer, are usually more 

 marked in the winter. The affected parts are usually 

 deeply cyanotic when cold, and bright red when they 

 are very cold or when they are warm. Frequently 

 they present a mixture of the two colors, red and 

 bluish-purple. The deep reddish color (as opposed to 

 cyanosis) produced by a very cold temperature (less 

 than 10 C) is due to arteriolar injury and dilatation 



n 



* 



fig. 4. Acrocyanosis. [Reprinted with the permission of 

 H. K. Lewis & Company, Ltd., London (94).] 



