Erythromelalgia precipitated by acral erythema in the setting of thrombocytopenia

Erythromelalgia is a rare syndrome that is characterized by episodic attacks of burning pain in the distal extremities, which last from minutes to days and are precipitated by exercise, warmth, or limb dependency. There is a primary or idiopathic form and a secondary form that occur with myeloproliferative or other diseases. All previous reports about erythromelalgia that is the result of a myeloproliferative process have documented associated thrombocytosis. We describe a 40-year-old woman with myelodysplastic syndrome who experienced erythromelalgia in the setting of acral erythema and thrombocytopenia, first induced by chemotherapy and recurring after a radiation- and chemotherapy-based myeloablative regimen that was administered before a T-cell-depleted allogeneic bone marrow transplantation.