Complex Regional Pain Syndromes (CRPS) – Presentation

CLINICAL PRESENTATION

In general, there is a history of a noxious event (may be trivial, or non-existent), usually accompanied by a period of immobilization. It should be remembered that this is a multi-symptom phenomenon that usually involves severe pain, swelling, stiffness, and discoloration of the extremity. The entire symptom complex is rarely observed in one patient.

Signs and Symptoms

Huge constellation of signs and symptoms, often changing as the disease progresses.

Pain (Hyperpathia) – usually burning sensation, but also described as �cutting�, �searing�, �pressure�. However described, the magnitude is out of proportion to the severity of injury. It is usually constant, but is aggravated by attempts to move the limb. Associated with this is allodynia (pain produced by non-noxious stimuli), where light touch is very painful.

Swelling – usually the first and most constant physical sign. Like the pain, it is out of proportion to what would be expected for the injury, and tends to worsen rather than improve, spreading through the extremity. In early RSD, the swelling is soft, but in time becomes hard and brawny. Periarticular swelling is a late finding, and produces tremendous thickening and stiffness of the joints.

Stiffness – again, much worse than would be expected for the injury. Initially, the stiffness is probably due to edema and pain; later, motion becomes limited by the brawny, thick edema, and eventually by intense fibrosis.

Skin Discoloration – this is related to vasomotor instability. Initially, vasodilation takes place and the extremity is erythematous; in later stages, vasoconstriction persists and the hand may appear pale, grayish, or cyanotic. Vasomotor instability is similarly responsible for temperature changes, with the extremity feeling warm during vasodilation, cool during vasoconstriction.

Hyperhidrosis (Excessive Sweating) – in the early stages of disease, this represents excessive adrenergic stimulation of apocrine glands. In advanced stages, diminished sweating or a dry hand is more commonly seen. Sudomotor activity is either increased or decreased, depending on the stage.

Osteoporosis – usually greater in degree than that caused solely by disuse. Demineralization usually starts in the ends of the long bones, but then spreads throughout. Early in the course, demineralization in a speckled, punched-out pattern has been referred to as Sudeck�s atrophy.

Trophic Changes – smooth, shiny skin with disappearance of skin wrinkles, initially due to edema, but later due to atrophy of the skin and subcutaneous tissues; atrophy of fat pads produce �pencil-pointing� fingers.

Palmar Fasciitis – observed in a number of patients, with the formation of acute nodules and thickening of the bands of palmar fascia.