Medium-pressure hyperbaric oxygen therapy for livedoid vasculopathy / Terapia de oxigenación hiperbárica de media presión en vasculopatía livedoide

Abstract Livedoid vasculopathy (LV) is a cutaneous manifestation of several diseases that lead to non-inflammatory thrombosis of dermal vessels. We report the case of a 26-year-old female with a 4 years and 8 months-old history of diagnosis of LV and a non-healing ulcer of more than a year of evolution. Because of refractory response to standard care, low-pressure hyperbaric oxygen (LPHBOT) was added to the therapeutic scheme (azathiopine 2.5 mg/kg, folic acid and acetylsalicylic acid). After 12 sessions of LHBOT (60 min, 1.45 ATA ≈100% O2), ulcers achieved complete healing with significant pain relief and no recurrence was present over 6 months. More studies are necessary to determine the effectiveness of HBOT for LV treatment.

Resumen La vasculopatía livedoide (VL) es una manifestación cutánea de varias enfermedades que conducen a una trom bosis no inflamatoria de los vasos dérmicos. Se presenta el caso de una mujer de 26 años con antecedente de diagnóstico de vasculopatía livedoide de 4 años y 8 meses, además de una úlcera no cicatrizante de más de un año de evolución. Debido a la respuesta refractaria a la atención estándar, se añadió oxígeno hiperbárico a baja presión (LPHBOT) al esquema terapéutico (azatriopina 2.5 mg/kg, ácido fólico y ácido acetilsalicílico). Después de 12 sesiones de LHBOT (60 min, 1,45 ATA ≈100% O2), las úlceras tuvieron una curación completa con un alivio significativo del dolor y no hubo recurrencia durante 16 meses. Se necesitan más estudios para determinar la eficacia de TOHB para el tratamiento del VL.

Vasculopatía livedoide: reporte de dos casos / Livedoid vasculopathy: a two case report

Vasculopatía livedoide es una enfermedad poco frecuente que se presenta principalmente en mujeres de edad media de la vida, antes de los cuarenta años. Es llamado también atrofia blanca, livedo reticular con ulceración de verano, vasculitis hialinizante segmentaria, o PURPLE (úlceras purpúricas dolorosas con patrón reticular de extremidades inferiores). Generalmente afecta los tobillos y se exacerba en primavera y verano. Su etiología es aún desconocida, pero se puede clasificar en formas primarias (sin ninguna patología asociada) y secundarias, ambos con un componente trombótico. A continuación se describen dos casos de vasculopatía livedoide: una mujer de 22 años con úlcera en pierna izquierda y púrpura retiforme en ambas extremidades. El estudio demostró que el cuadro estaba asociado o síndrome antifosfolípidos. El segundo caso es de una mujer de 47 años con uno vasculopatía livedoide secundaria o lupus eritematoso sistémico.

Livedoid vasculitis is a rare disease that occurs mainly in women under 40 years of age. It has also be ennamed atrofia blanche, hyaline segmentary vasculitis or PURPLE (purpuric ulcers with reticular pattem in low legs). It usually affects ankles and has an exacerbation during Spring and Summer seasons. It has an unknown etiology, and it has been classified as primary (or idiopathic) or secondary (associated to another disease). We describe two cases of livedoid vasculopathy: the first case is o 22-year-old women with ulcers and retiform purpura associated to antiphospholipid syndrome. The second case is o 47-year-old women with livedoid vasculopathy associated to systemic lupus erythematosus.

Drug-associated livedoid vasculopathy

Livedoid vasculopathy is reported in a 38-year-old white female, who first presented with spontaneous skin lesions in the left ankle, at 17 years old. For the last fifteen years she used low-dose oral contraceptive (gestodene and ethinylestradiol) and persisted asymptomatic for a long period. Nevertheless, painful red papules and dark spots reappeared in the same area and progressed to an intensely tender and irregular shallow ulcer, during summer. Skin biopsy samples showed dermal vessels with subintimal fibrinoid necrosis and intraluminal thrombosis, without clear inflammation. Cutaneous lesions have improved faster after topical betamethasone was added to treatment. Data from the present case suggests a drug-associated livedoid vasculopathy.

Relata-se caso de vasculopatia livedóide em uma mulher branca de 38 anos, que inicialmente apresentou lesões cutâneas espontâneas no tornozelo esquerdo aos 17 anos de idade. Durante os últimos quinze anos, fez uso de contraceptivos orais (gestodene e etinilestradiol). Permaneceu assintomática por longo período; entretanto, no último verão, pápulas avermelhadas dolorosas e manchas escuras reapareceram na mesma área e formaram uma úlcera rasa irregular intensamente dolorosa. O exame das amostras da biópsia de pele mostrou vasos dérmicos com necrose fibrinóide e trombose intraluminar, sem reação inflamatória. As lesões cutâneas apresentaram cicatrização mais rápida após acréscimo de betametasona tópica ao esquema terapêutico. Os dados do presente caso sugerem vasculopatia livedóide associada ao uso de droga.