Original article
Efficacy of intravenous immunoglobulins in livedoid vasculopathy: Long-term follow-up of 11 patients

https://doi.org/10.1016/j.jaad.2014.05.039Get rights and content

Background

Evidence for the efficacy of various therapies of livedoid vasculopathy (LV) is limited.

Objective

We sought to determine efficacy and tolerability of 2 g/kg of intravenous immunoglobulins (IVIG) every 4 weeks in patients with LV.

Methods

This was a long-term follow-up study of 11 patients with LV treated with 2 g/kg of IVIG assessing the clinical characteristics, disease course, and quality of life.

Results

The treatment regimen led to complete remission of ulcerations and pain in 17 of 29 disease episodes (59%) after 3 cycles and in 25 of 29 episodes (86%) after 6 cycles. Two disease episodes showed remission after 7 and 8 cycles, resulting in a total number of remissions of 27 (93%). Subscore analysis showed resolution of pain in 80% after 2 IVIG cycles. Disease severity and quality of life were significantly improved after 6 cycles. Median duration of remissions was 26.7 months after initial and 7.5 months after subsequent disease episodes.

Limitations

This was a retrospective study that did not include the comparison of IVIG efficacy and its impact on quality of life with treatment options.

Conclusions

In our patients with LV, high-dose IVIG led to fast and complete resolution of pain and ulcerations and to substantial improvement in quality of life.

Section snippets

Demographic data, clinical and laboratory findings

This study was approved by our institutional review board. From 2002 until 2012, 11 consecutive patients with LV and primary and recurrent disease flares received IVIG at a dose of 2 g/kg body weight over 2 or 3 consecutive days every 4 weeks over 6 months. Each case was diagnosed on basis of clinical presentation (livedo reticularis, atrophie blanche, pain, ± ulceration) and by histopathology. Analyzed data were taken from the medical records and missing data were obtained by interviewing

Demographic data, clinical and laboratory findings

The mean age at diagnosis was 34 ± 12 years and the median disease history before diagnosis was 8.5 years (range 0.5-25). Cranial magnetic resonance imaging scan results (8 patients) were unremarkable in 6 and showed nonspecific isolated foci of gliosis in 2 cases. Peripheral arterial disease and chronic venous insufficiency were ruled out in all patients. Trigger factors were seasonal worsening in summer (5 patients) and in winter (1 patient) along with long-standing and mental distress. Six

Discussion

This long-term study demonstrates the significant reduction of disease activity in patients with LV after 6 IVIG cycles, measured by LVAS and DLQI score. Administration of 2 g/kg of IVIG over 2 to 3 consecutive days every 4 weeks induced complete remission of ulcerations and pain in 17 disease episodes (59%) after 3 cycles and in 25 of 29 disease episodes (86%) after 6 cycles. In addition, 2 disease episodes showed remission after 7 and 8 cycles, thereby increasing the total number of

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    Funding sources: None.

    Conflicts of interest: None declared.

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