Necrotizing Livedo Related to Neoplastic Microvascular Embolism

Abstract

Sir, A 64-year-old woman was admitted for evaluation of a necrotic livedo of the perineal area. She had developed fluctuating erosive lesions of the buttocks within the past 3 months, initially considered as sacral herpes zoster and treated with oral aciclovir without significant results. Her medical history included multi-complicated type 2 diabetes mellitus, elevated blood pressure, atrial flutter, inter-atrial communication, and chronic exposure to tobacco (40 packs (7300 cigarettes) per year). At initial evaluation, she displayed erythematous, exquisitely painful, necrotic and livedoid lesions of the buttocks, lumbar areas, vulva and perineum. No other skin lesions were noticed, especially on the extremities. Physical examination also showed a reduction in intensity of pulses on lower limbs, effort-related claudication, a mitral heart murmur and a mild alteration of the intellectual capabilities. Biological explorations disclosed increased inflammatory parameters but no significant abnormality regarding standard and coagulation tests (absence of anti-phospholipid antibodies or cryoglobulinaemia; serum levels of protein C and S, anti-thrombin III and homocysteine within normal limits; no resistance to activated C protein). Echocardiography was normal except for the inter-atrial communication already known. Skin biopsies revealed obstructions of the dermal vessels by clusters of neoplastic cells further identified by immuno-histological methods as adenocarcinoma cells with positive results for cytokeratins AE1-AE3 and 7 and no staining with cytokeratin 20 and CA-125. However, renal, abdominal and pelvic ultrasound, endoscopic exploration of upper and lower digestive tract, total-body scan and mammography failed to identify any primary neoplastic lesion. Instead, the scanner disclosed an aortic sub-renal thrombosis with complete luminal occlusion along with a highly reduced perfusion in the hypogastric and iliac territories contrasting with the development of a collateral circulation directed to the lower limbs. Anticoagulant therapy was then introduced without any significant clinical improvement and the livedo slowly spread during the following month, resulting in severe necrotic lesions while small, painful ulcerations of the legs and weakness of the lower limbs appeared as well. In the meantime, intellectual alterations quickly worsened with increasing negligence without any evidence of brain metastasis, cerebral infarct, neoplastic, viral or bacterial meningitis or meningo-encephalitis according to comprehensive investigations including cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI). Two courses of gemcitabine were eventually performed due to the undetermined origin of the causal neoplasia, but the patient died 2 months after admission and 5 months after the occurrence of the first cutaneous signs. No post-mortem examination was performed.