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We present a kindred of 29 persons affected with erythromelalgia (erythermalgia) in 5 generations. This paper updates the family reported by Burbank et al. [1966]. Patients have symptoms of intermittent intense burning limb pain related to increased skin temperature. No successful treatment has been identified, and the pathogenetic mechanism has not been established. Most affected individuals are female.

A 54-year-old man complained of burning pain, warm skin and erythema in his extremities. A diagnosis of primary erythromelalgia was made. Microneurography was used to clarify the role of skin sympathetic nerve activity in the pathophysiology of primary erythromelalgia. The patient showed normal skin sympathetic nerve activity but no vasoconstriction response. Aspirin activated the skin sympathetic nerve activity and improved vasoconstriction producing symptomatic relief. These results suggest that the lack of vasoconstriction following vasoconstrictor activity of the skin sympathetic nerves results in increased skin blood flow and burning pain.

The diagnosis of acrocyanosis and erythromelalgia is done only on clinical grounds. Acrocyanosis is a benign disorder. Erythromelalgia is a very rare syndrome but can be a clue to the early diagnosis of myeloproliferative disorders.

Two cases of cervical myelopathy, which exhibited peculiar vasomotor symptoms ("acro-erythro-cyanosis") on distal regions of the four limbs are reported. Continuous reddening, swelling and skin temperature increase were observed on both hands and feet in case 1, a-44-year-old man, and on both hands in case 2, a-47-year-old man. Cold stimulation resulted in cyanosis and decrease of skin temperature on the affected regions paroxysmally. The condition of skin at room temperature may be caused by arteriectasia of arterioles due to hypotonia of vasomotor fibers, and this was similar to erythromelalgia. On the other hand, cold stimulation may have led to this condition, where the contraction of skin arterioles due to hypertonia of vasomotor fibers was added to the dilation of venule, and this was similar to acrocyanosis. Consequently, we provisionally named the vasomotor symptoms in the present cases as "acro-erythro-cyanosis". The other neurological signs were as follows. Case 1: mild weakness in right upper and lower limbs and left small hand muscles, mild superficial and deep sensory disturbance on bilateral palms and soles and decrease of vibration on bilateral lumbar regions and thereunder. Case 2: mild weakness of right small hand muscles, superficial sensory disturbance on distal regions of bilateral upper and lower limbs and a decrease of joint position sense on right hand. Myelography and metrizamide CT myelography revealed a high-degree deformity of the spinal cord due to the herniated disks between C4 and C5 in case 1 and between C3 and C4 in case 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Erythromelalgia is a vascular acrosyndrome affecting mainly the feet and triggered by elevation of room temperature. It may be primary, or secondary, in particular to a polycythemia, when an increased platelet count plays an essential role, explaining the beneficial effect of aspirin or NSAI agents that inhibit platelet prostaglandin cyclo-oxygenase. The favorable action of beta-blockers could result from the possible existence of an anomaly of cutaneous adrenergic nerves.