Intravenous Immunoglobulin Therapy in Livedoid Vasculopathy: Retrospective Observation of Clinical Outcome and Patient's Activity Level.

Katrin Kofler,Vanessa Geiger,Anke Strölin,Lukas Kofler

doi:10.1177/12034754211003525

Katrin Kofler, Vanessa Geiger + Show 2 more

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https://doi.org/10.1177/12034754211003525

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Abstract

BackgroundLivedoid vasculopathy (LV) is a rare disease characterized by livedo racemosa, atrophie blanche, ulcerations, and severe pain. Low molecular weight heparins and rivaroxaban can be used in LV-patients. In addition, intravenous immunoglobulins (IVIG) have been described as treatment-option.ObjectivesObjective was to investigate the therapeutic effect of IVIG on ulcer, pain and restrictions in daily life.MethodsThirty-two LV-patients who received IVIG at the Department of Dermatology Tübingen between 01/2014 and 06/2019 were identified. Twenty-five of these patients were available for further follow up and were included in the study. Patients were interviewed using a questionnaire focusing on the course of the disease, symptoms, and subjective response to IVIG-treatment.ResultsTwenty-five patients were included in the study (mean follow up: 28.9 months). Patients received an average of 6.8 cycles (range 1-45) of IVIG during the observed period.Significant improvements were seen regarding skin findings, pain, and limitation of daily activities. Complete remission of symptoms was observed in 68% of patients. Good tolerability of IVIG was shown in 92%.ConclusionsA good therapy response regarding ulceration, pain, and daily life restrictions with good tolerability was demonstrated for IVIG (2 g/kg bodyweight over 5 days).

Highlights

Livedoid vasculopathy (LV) is a rare disease with recurrent thrombotic occlusion of cutaneous vessels of the lower extremity
Case reports and studies with smaller case numbers indicate a good response for intravenous immunoglobulins (IVIG) in the treatment of LV.[10,11,12,13]
This study shows a significant improvement in the symptoms of LV in terms of pain, ulceration and restrictions in everyday life under therapy with IVIG

Summary

Introduction

Livedoid vasculopathy (LV) is a rare disease with recurrent thrombotic occlusion of cutaneous vessels of the lower extremity. Women are more often affected than men; in a recent study a gender ratio of 2.1:1 has been reported.[4,5] It has been shown, that the symptoms of the disease and its consequences considerably reduce the quality of life.[6] As patients experience severe pain, especially due to local ischemia, extensive ulceration and rapid irreversible scarring, quick and efficient treatment options are essential. Most experience in Germany is available for low molecular weight heparins.[4,7,8] Recent study results showed effective pain reduction using rivaroxaban.[9] Antithrombotic therapy is intended to prevent or positively influence microcirculatory thrombotic events. Conclusions: A good therapy response regarding ulceration, pain, and daily life restrictions with good tolerability was demonstrated for IVIG (2 g/kg bodyweight over 5 days)

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