Primary cutaneous granulomatous phlebitis with visceral involvement: unmasking of a masked villain.

Abstract

Primary cutaneous granulomatous phlebitis (PCGP) is distinctly unusual. The entity was first described in 1954 and, to date, the only four known reported cases of PCGP occurred in two men and two women, all under 40 years of age. The arm and/or leg veins were affected in three patients and mesenteric veins in one; all were diagnosed by means of excisional biopsy specimens. The first three patients presented with a febrile illness, and two of them had elevated erythrocyte sedimentation rates but little else indicative of a systemic disease. Three of the four patients received no immunosuppressive drug treatment. The fourth patient presented with a segmental infarction of the ileum that required a bowel resection. We now describe four new cases of PCGP, in women aged 26, 62, 76, and 38 years, one black, one Hispanic, and two white. The diagnosis of PCGP was made by means of biopsy specimens in two patients, at autopsy in one, and from a below-knee amputation specimen in one. Despite the obvious limited global experience of this rare form of phlebitis, there is ground for uneasiness that PCGP may not be as innocuous a curiosity in surgical pathology as was thought at first sight but a more sinister, little-known villain among the vasculitides that is only now beginning to show its true color.