Nonspecific capillary proliferation and vasculopathy indicate skin hypoxia in erythromelalgia.

Objective: To report on the histopathologic findings of affected skin in consecutively collected biopsy specimens from 49 patients with erythromelalgia (EM).

Design: Skin biopsy specimens were obtained from the foot arch and analyzed by light microscopy, immunofluorescence microscopy, and electron microscopy.

Setting: Oslo University Hospital-Gaustad, University of Oslo, Oslo, Norway.

Prostacyclin reduces symptoms and sympathetic dysfunction in erythromelalgia in a double-blind randomized pilot study.

Sympathetic dysfunction and skin microvascular arteriovenous shunting with insufficient nutritive perfusion and tissue hypoxia have been reported in patients with erythromelalgia. The objective of this study was to determine whether iloprost, a synthetic prostacyclin analogue--primarily a vasodilator and inhibitor of platelet activation--improves symptoms and sympathetic function in patients with erythromelalgia. Erythromelalgia is a rare condition, but we managed to collect 12 primary cases for a double-blind, randomized, parallel-group pilot trial evaluating the effect of iloprost (n = 8) and placebo (n = 4).

Impaired neurogenic control of skin perfusion in erythromelalgia.

Erythromelalgia is a clinical diagnosis characterized by erythema, increased temperature and burning pain in acral skin. The pain is relieved by cooling and aggravated by warming. The symptoms have been hypothesized to be caused by skin hypoxia due to increased arteriovenous shunting.