title: Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review.
abstract: 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.
authors.affiliation: Department of Dermatology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Eun Jee
authors.initials: EJ
authors.lastname: Kim
authors.affiliation: Clean-up Dermatologic Clinic, Seoul, Korea.
authors.forename: So Young
authors.initials: SY
authors.lastname: Yoon
authors.affiliation: Department of Dermatology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Hyun Sun
authors.initials: HS
authors.lastname: Park
authors.affiliation: Department of Dermatology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Hyun-Sun
authors.initials: HS
authors.lastname: Yoon
authors.affiliation: Department of Dermatology, Seoul National University Boramae Hospital, Seoul, Korea.
authors.forename: Soyun
authors.initials: S
authors.lastname: Cho
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D054068:Livedo Reticularis / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D008297:Male; D020551:Pulse Therapy, Drug; D012008:Recurrence; D019233:Retreatment; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
keywords: intravenous immunoglobulin; livedoid vasculopathy