Erythromelalgia With Subsequent Digital Necrosis, Glomerulonephritis, and Antiphospholipid Antibodies

Abstract

Erythromelalgia is a condition of extremities characterized by redness, increased temperature, and burning pain. We describe the first reported case of erythromelalgia in a young woman with digital necrosis and mesangial nephritis associated with antiphospholipid antibodies. The symptoms and necrosis completely resolved with treatment with corticosteroids. We discuss the differential diagnosis of this entity and highlight the importance of a follow up of these patients, because erythromelalgia may precede a myeloproliferative disorder or systemic lupus erythematosus by months or years.

Coexistence of Erythromelalgia and Raynaud's Phenomenon in a Systemic Lupus Erythematosus Patient

“…Primary EM usually affects younger patients and is more often bilateral. Secondary EM is related to various disorders, such as thrombocythemia, myeloproliferative disorders, arterial hypertension, vasculitis, and SLE [4], and administration of calcium channel blockers or bromocriptine [13,14]. A negative family history, late onset at the age of 59 years, and clinical symptoms of SLE indicated the presence of secondary EM in the patient we reported in our study.…”

“…Primary EM, usually showing symptoms in childhood, is a rare autosomal dominant inherited disease caused by mutations in the SCN9A gene that encodes a voltage-dependent sodium channel α-subunit [3]. Secondary EM is related to various disorders, viz., thrombocythemia, myeloproliferative disorders, arterial hypertension, vasculitis, and systemic lupus erythematosus (SLE) [4]. Compared to EM, the most prominent symptom of Raynaud phenomenon (RP) is whiteness of digits occurring as a result of cold-induced vasoconstriction, sometimes followed by cyanosis and rubor of fingers.…”

Secondary erythromelalgia involving the ears probably preceding lupus erythematosus

“…Eight of these already had a diagnosis of SLE at the time of onset of erythromelalgia. In the other patients, erythromelalgia preceded the onset of SLE by 4 months to 4 years 4,10–13 …”

“…The first symptoms usually develop in childhood, mostly before the age of 10 years 5 . Secondary erythromelalgia can be associated with various disorders, including thrombocythemia, myeloproliferative disorders, arterial hypertension, vasculitis, and systemic lupus erythematosus (SLE) 3,4 …”

“…3 Although there is no generally accepted classification, most authors use the terms primary or secondary erythromelalgia depending on the absence or the occurrence of an associated disease that may precede, coincide with, or follow the clinical onset of erythromelalgia. 4 Primary erythromelalgia (OMIM 603415) is a rare autosomal dominantly inherited disease caused by mutations in the SCN9A gene that encodes a voltage-dependent sodium channel α -subunit. The first symptoms usually develop in childhood, mostly before the age of 10 years.…”