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

Itzinger-Monshi, Babak; Posch, Christian; Vujic, Igor; Sesti, Alma; Sobótka, S; Rappersberger, Klemens

doi:10.1016/j.jaad.2014.05.039

Cited by 51 publications

(62 citation statements)

References 23 publications

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“…Both facts are similar to data published by Renner et al . while others had described a female‐to‐male ratio of 3 : 1 and a clearly younger population with a mean age of 32 or 37 years . The average BMI in our study population was only slightly lower (25.6 kg/m²) compared to an age‐matched German population with a BMI of 26.4 kg/m² although BMI was elevated in 40% of LV patients.…”

Section: Discussionsupporting

confidence: 91%

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Weishaupt

Strölin

Kahle³

et al. 2019

Acad Dermatol Venereol

105

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Background Livedoid vasculopathy (LV) is a rare cutaneous thrombotic disease. It is characterized by occlusion of dermal vessels resulting in livedo racemosa, ulceration and atrophie blanche. Clear guidelines for diagnosis and treatment are missing. Objective The purpose of this study was to better characterize epidemiology, clinical appearance and treatment reality of LV in a well‐defined patient cohort. Methods The cohort was allocated within a prospective, multicentre, phase IIa trial that investigated the effect of rivaroxaban in LV. Results Analysis of 27 patients revealed that LV patients had an increased Body Mass Index (BMI; 11/27), hypertension (19/27) and increased levels of lipoprotein (a) (5/12) and homocysteine (10/12) in the blood. The female‐to‐male ratio was 2.1 : 1, and the median age was 53.0 years [interquartile range (IQR) 40.5–68]. Investigation of the clinical appearance found that 82% of patients had livedo racemosa, and the ankle region was most likely to be affected by ulceration (56–70%). The analysis of patient treatment history showed that heparin was most effective (12/17), while anti‐inflammatory regimens were, although often used (17/24), not effective (0/17). Conclusion We add clinical clues for a data supported diagnosis of LV, and we provide evidence that anticoagulants should be administered in monotherapy first line (EudraCT number 2012‐000108‐13‐DE).

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Section: Discussionsupporting

confidence: 91%

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Weishaupt

Strölin

Kahle³

et al. 2019

Acad Dermatol Venereol

105

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“…There have been three case series of LV treated with IVIg thus far . The results of our series are similar to the previous reports in that three cycles of monthly IVIg was effective and improvement in pain was achieved faster and was more significant compared with erythema and ulceration ( p = 0.041).…”

Section: Discussionsupporting

confidence: 87%

Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review

Kim

Yoon

Park

et al. 2015

Dermatologic Therapy

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Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports demonstrated successful use of intravenous immunoglobulins (IVIg) in LV. We assessed the efficacy and tolerability of 2 g/kg IVIg therapy every month for 2∼3 cycles in patients with refractory LV. We analyzed the efficacy, side effects and recurrence after long-term follow-up (51.9 ± 14.0 months) in seven patients with LV treated with 2 g/kg of IVIg. Mean clinical score of sum of erythema, ulceration and pain index (each: 0-3) was 5.7 ± 0.9 before the therapy and significantly lower after therapy (1.1 ± 0.5) (p = 0.001). Even after just one cycle of IVIg, the score decreased significantly from 5.7 ± 0.9 to 3.7 ± 0.9 (p = 0.002), especially the pain score. In one patient, LV has not recurred for over 7 years; six patients experienced recurrence after a mean of 12.7 ± 2.8 months. Out of the six patients, two patients were re-administered IVIg whereas the others were well controlled by conventional therapy. We propose that IVIg is a rapid, effective, and safe therapeutic option in LV refractory to other treatment modalities.

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“…Numerous case studies have reported on the successful use of IVIg in therapy‐resistant livedoid vasculopathy . Although no general recommendation can be given at this point, the amount of evidence for a positive effect of IVIg is increasing, justifying their use in desperate cases.…”

Section: Dermatomyositismentioning

confidence: 99%

European Guidelines (S1) on the use of high‐dose intravenous immunoglobulin in dermatology

Enk

Hadaschik

Eming

et al. 2016

Acad Dermatol Venereol

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

Cited by 51 publications

References 23 publications

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review

European Guidelines (S1) on the use of high‐dose intravenous immunoglobulin in dermatology

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