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Periungual capillaroscopy is a simple noninvasive exploration contributing to the clinical examination. It is highly contributive to the etiological diagnosis of Raynaud's phenomenon and is the simplest means of detecting scleroderma early. Capillaroscopy has also largely contributed to better understanding of the pathogenesis of vascular disorders encountered in acrosyndromes. Experience has shown however that clinical indications should be more precise and results interpreted with more scientific rigor (J Mal Vasc 1999; 24: 357-362).

The authors report sixteen consecutive cases of erythromelalgia, an infrequent disease in which local heat, redness, and pain develop in the hands and/or feet in recurrent attacks. The disease was essential in nine patients; in the remaining seven, the cause was a myeloproliferative syndrome (polycythemia vera in 3 cases and thrombocythemia in 1 case) or a drug (bromocriptine, nicardipine, and nifedipine, one case each). Acetylsalicylic acid was effective in only six of the nine essential cases. Intravascular platelet activation and aggregation with plugging of the arterioles has been suggested as the mechanism of erythromelalgia in patients with myeloproliferative disorders. Other, as yet unelucidated pathophysiologic events underlie the juvenile-onset forms, which usually fail to respond to acetylsalicylic acid.

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.

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.