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Diagnosis and Treatment of Livedo Reticularis on the Legs Document downloaded from http://www.elsevier.es, day 21/06/2016. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. Actas Dermosifiliogr. 2008;99:598-607 PRACTICAL DERMATOLOGY Diagnosis and Treatment of Livedo Reticularis on the Legs C. Herrero, A. Guilabert, and J.M. Mascaró-Galy Dermatology Department, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain Abstract. The term livedo reticularis refers to a reddish-violet reticular discoloration of the skin that mainly affects the limbs. It is caused by an interruption of blood flow in the dermal arteries, either due to spasm, inflammation, or vascular obstruction, and is associated with diseases of varying etiology and severity. To establish the cause of livedo reticularis, it is essential to determine its course (chronic, acute, or fulminant), the presence of other cutaneous signs such as nodules, retiform purpura or necrosis, and the possible association of general symptoms or laboratory findings that suggest a particular systemic process. The aim of this review is to describe the diagnosis and treatment of the disease. Key words: livedo reticularis, retiform purpura, livedo racemosa, vasculitis, cholesterol emboli, calciphylax- is, antiphospholipid antibodies. LIVEDO RETICULARIS DE LAS PIERNAS: METODOLOGÍA DE DIAGNÓSTICO Y TRATA- MIENTO Resumen. El término livedo reticularis describe un retículo cutáneo de coloración rojo-violácea que afecta preferentemente a las extremidades. Su origen es la interrupción del flujo sanguíneo en las arteriolas dérmi- cas, ya sea por espasmo, inflamación u obstrucción intravascular, y se asocia a entidades de diversa etiología y gravedad. Para establecer la causa de una livedo reticularis es fundamental conocer la evolución del cuadro (si la livedo reticularis es o bien crónica o bien aguda o fulminante), la presencia de otros signos cutáneos, como nódulos, púrpura retiforme o necrosis, y la posible asociación con síntomas generales y/o datos analíticos que apunten hacia un determinado proceso sistémico. El objetivo de esta revisión es proporcionar un método diagnóstico y terapéutico para el abordaje de la livedo reticularis. Palabras clave: livedo reticularis, púrpura retiforme, livedo racemosa, vasculitis, émbolos de colesterol, calcifi- laxia, síndrome antifosfolípido. The term livedo reticularis is used to describe a reticular To understand the events associated with livedo reticularis, red-violaceous discoloration of the skin that typically affects it is necessary to be familiar with the anatomy and physiology the limbs, although it can also be generalized. It is secondary of the cutaneous blood vessels. The skin, like any organ, to organic or functional disorders of the dermal arteries or receives its blood supply via the arteries, which branch into arterioles. Because arterioles can be affected by numerous arterioles at the junction between the dermis and the conditions, livedo reticularis has many possible causes (Table subcutaneous cellular tissue to form the deep vascular plexus, 1). The condition has its origins in the reduction or which runs parallel to the epidermis. This plexus is formed interruption of blood flow at certain points in the path of by the feeding artery and a network of arterioles measuring the blood vessels due to spasm, inflammation of the arteriolar 100 µm in diameter; the walls of these vessels consist of wall, or vascular obstruction. Vascular obstruction can, in 2 outer layers of muscle, an elastic lamina, and internally, turn, be caused by thrombosis, embolic events, or vessel the vascular endothelium. Smaller, communicating arterioles wall abnormalities. arise from the deep plexus and run perpendicularly to the epidermis to reach the upper dermis, where they form the superficial vascular plexus, which also runs parallel to the Correspondence: epidermis. The arterioles here are very small (diameter, Carmen Herrero Mateu Servicio de Dermatología, Hospital Clínic 10 µm); their walls only have a single muscle layer and the C/ Villarroel, 170, 08036 Barcelona, Spain internal elastic lamina is absent. The superficial plexus gives [email protected] rise to capillaries that supply blood to the papillae; the blood Manuscript accepted for publication February 2, 2008. then drains through the postcapillary venules into larger 598 Document downloaded from http://www.elsevier.es, day 21/06/2016. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. Herrero C et al. Diagnosis and Treatment of Livedo Reticularis on the Legs Table 1. Causes of Livedo Reticularis Table 2. Clinical Associations of Livedo Reticularis With Purpura, Necrosis, and/or Nodules Arteriolar spasm Response to cold Isolated Livedo Livedo Reticularis with Retiform Drug-induced Reticularis (Caused Purpura, Necrosis, Ulcers, Nodules (ergotamine, cocaine) by Spasm) (Caused by Vascular Obstruction) Vessel Inflammation Cold Noninfectious (vasculitis) Systemic polyarteritis nodosa Drugs Cutaneous polyarteritis nodosa Wegener granulomatosis SLE, RA Churg–Strauss syndrome Polyarteritis Nodosa Polyarteritis Nodosa Microscopic polyangiitis Wegener granulomatosis, Drug-induced vasculitis (thiouracil) Churg–Strauss syndrome, Vasculitis associated with microscopy polyangiitis, SLE, RA SLE or RA Drug-induced vasculitis (thiouracil) Lucio’s phenomenon Infectious Lucio’s phenomenon Sneddon syndrome Vascular Obstruction Without Inflammation Antiphospholipid Antiphospholipid syndrome Thrombosis Antiphospholipid syndrome syndrome Livedoid vasculopathy Sneddon syndrome Disseminated intravascular Livedoid vasculopathy coagulation SLE, RA Coumarin-induced necrosis Coumarin-induced necrosis Type I cryoglobulinemia Disseminated intravascular Thrombophilia Sickle cell anemia coagulation Drugs Dysproteinemia Atrial myxoma (type I cryoglobulinemia) Cholesterol Procoagulant genetic factors Calciphylaxis (factor V) Primary hyperoxaluria Sickle cell anemia Drugs Abbreviations: RA, rheumatoid arthritis; SLE, systemic lupus erythematosus. Embolization Atrial myxoma Cholesterol Vessel wall disorders Calciphylaxis Hyperoxaluria Diagnosis Abbreviations: RA, rheumatoid arthritis; SLE, systemic lupus erythematosus. The diagnosis in a patient with livedo reticularis on the legs requires a search for associated subcutaneous nodules, retiform purpura, necrosis and secondary ulceration (Table 2). Some venules, which follow a path that is parallel but inverse authors distinguish between livedo reticularis (network to the afferent vessels.1 This entire structure forms a pattern with complete, regular rings) and livedo racemosa 3-dimensional network that acts as a single unit. Nonetheless, (network pattern with incomplete, irregular rings) and the clinical repercussions of vascular lesions vary according consider that the latter is always secondary to organic rather to the characteristics of the affected vessels and their location than functional disorders (Figure 1).2 A detailed history within the layers of the skin. can provide invaluable information and should include While a reduction of blood flow in the arterioles can drugs (coumarins, sedatives, ergotamine), known associated cause livedo reticularis, a complete interruption of this flow diseases (such as renal failure, arteriosclerosis, systemic due to blockage of the lumen can cause hemorrhagic infarcts. autoimmune diseases, and monoclonal gammopathy), recent Infarcted areas initially present as reticular purpuric lesions surgery (catheterization, angioplasty), and history of (retiform purpura) that can become visibly ecchymotic and spontaneous abortion. It is also essential to determine the eventually form more or less extensive areas of necrosis and course of the disease (chronic, acute, or fulminant), explore secondary ulceration. Accordingly, necrotic lesions and associated symptoms (fever, dyspnea, arthralgia), and analyze ulcers caused by arterial or arteriolar occlusion tend to have clinical findings (such as murmurs, signs of pneumonitis, reticular, stellate margins. Intense inflammatory reactions and neck stiffness) however associated fever may suggest of the vascular wall and perivascular dermis cause painful the presence of an infectious process, it can also be due to subcutaneous nodules, which are often located either in the a generalized inflammatory process—almost always same area as the reticular lesions or along the path of the serious—such as an exacerbation of systemic lupus vessel. erythematosus or systemic vasculitis. Actas Dermosifiliogr. 2008;99:598-607 599 Document downloaded from http://www.elsevier.es, day 21/06/2016. This copy is for personal use. Any transmission of this document by any media or format is strictly prohibited. Herrero C et al. Diagnosis and Treatment of Livedo Reticularis on the Legs Figure 1. Schematic Livedo reticularis Livedo racemosa Retiform purpura representation of livedo reticularis, livedo racemosa (functional) (organic) and retiform purpura. Adapted from Sepp.35 Laboratory tests should include a complete blood count, as the reponse to cold in an attempt to maintain body coagulation studies, evaluation of kidney function, urinary temperature, or may be a response to certain drugs. It is sediment, proteinuria, antinuclear antibodies, complement well known that ergotamine and other alkaloids derived levels, antineutrophil cytoplasmic antibodies (ANCAs), from rye ergot—once used to induce postpartum uterine cryoglobulin and cryofibrinogen levels,
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