Raynauds Phenomenon

Maurice Raynaud (1834-1881) was a French medical student who noted color changes in the hands of some women while standing outside in the cold waiting for the streetcar during the winter in Paris. Raynaud's phenomenon refers to the typical hand changes he described. Raynaud's disease (primary Raynaud's) refers to the typical hand changes occurring in the absence of any other rheumatic disease. Raynaud's syndrome (secondary Raynaud's) refers to the typical changes occurring in the setting of an underlying rheumatic disease.

Many people experience cold hands whenever it is cool outside. This is not Raynaud's phenomenon. Raynaud's phenomenon results from spasm of the blood vessels in response to a stressor such as exposure to the cold or embarrassment. Many of my patients with significant Raynaud's syndrome describe their worst problems as occurring in the summer when they walk into air-conditioned buildings from the heat outside. The fundamental abnormality is the hyperreactivity of the blood vessels. Raynaud's may be the result of being thin (it is common for tall, thin women to have Raynaud's phenomenon) or of the blood vessels being sensitized by immune complexes or by inflammatory mediators (see Chapter 22) because the individual has an underlying rheumatic disease.

For a diagnosis of Raynaud's phenomenon, there must be a three-phase color change. Initially, the tips of one or more fingers turn white as the blood flow is cut off by spasm of the blood vessels. Once the spasm passes, there is increased reactive blood flow and the fingers turn red, then slowly go back to their normal state of bluish discoloration, with sluggish blood flow. Cold red hands or cold blue hands without the spasmodic white phase do not constitute Raynaud's phenomenon.

The importance of Raynaud's phenomenon lies in its association with a variety of rheumatic diseases. While Raynaud's is common in thin young women, it is often the first manifestation noticed in children with progressive systemic sclerosis (see Chapter 13). Raynaud's is also found frequently in children with other vasculitic diseases, such as lupus, dermatomyositis, and anticardiolipin antibody syndrome. It may occur in children with many other rheumatic conditions.

Since most young women with Raynaud's phenomenon are healthy, it is important to recognize children in whom Raynaud's is a warning of an underlying condition. Every child with Raynaud's should have routine testing done, but boys with Raynaud's are at greater risk of underlying disease than girls, and children less than twelve years of age are at greater risk than older children. Tall, thin girls with a family history of Raynaud's disease tend to have fewer problems with underlying disease than those without a family history. Long-lasting episodes of Raynaud's, a high frequency of episodes, shortness of breath, chest pain when the Raynaud's occurs, and morning stiffness (particularly in the fingers) all increase the probability that an underlying disease is present. When examining the child, the physician should look carefully for nail fold capillary abnormalities (see Fig. 12-1) or distal fingertip pitting (see below). Either of these findings substantially increases the probability of an underlying any finger is significant.

Distal fingertip pitting is another important finding that is also easily detected on routine examination. These pits are little areas where the blood supply has been cut off and the skin and underlying tissues have atrophied. As a result, the areas often feel callused to the examiner. The child will describe decreased feeling in the fingertips, as one would expect from calluses. If you look

FIG 12-1 Dilated nail fold capillaries may be easy to see with a small magnifying glass.

disease being present.

FIG 12-1 Dilated nail fold capillaries may be easy to see with a small magnifying glass.

Many physicians are concerned that they lack the proper equipment for nail fold capillary microscopy, but this can be easily done with an otoscope (the small handheld magnifier used for looking in ears). In a normal individual, the nail fold capillaries cannot be seen, even with an otoscope. If red streaks are seen in the nail folds, it suggests the presence of nail fold capillary abnormalities and the child should be referred for further evaluation. All ten fingers should be examined, as the presence of the abnormality on carefully, you may be able to see the areas of thickened skin, often with little central depressions or scars. However, it is easiest to screen for these by feeling the tips of the fingers. The areas that are scarred are harder than normal skin and feel like little bumps.

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