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Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis). The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological back-ground. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

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Polycythemia Vera (PV) is a Philadelphia-negative chronic myeloproliferative neoplasm (MPN) mainly characterized by erythrocytosis. In this report we describe a case of severe cutaneous toxicity in patients with PV treated with hydroxyurea. A 72-year-old woman diagnosed with PV with V617F mutation of JAK2 performed more than 10 years before and treated with hydroxyurea plus phlebotomies and low-dose ASA for about 7 years addressed our center for the appearance of serious dermatitis at the face symptomatic for severe itch. The patient underwent a dermatology visit with diagnosis of desquamative dermatitis due to iatrogenic cause related to the use of hydroxyurea. HU was stopped for a month with no improvement after a month of wash-out. Ruxolitinib was prescribed at a dose of 20 mg per day, in order to control hypercytosis and considering the severe intolerance to hydroxyurea. Ruxolitinib allowed not only to reduce the haematocrit, reaching the target value of 45%, and control thrombocytosis, but also to switch off the severe itch and to completely resolve skin toxicity.

Introducción: el lupus es una enfermedad compleja y varias veces de difícil abordaje. Alcanzar la remisión es uno de los objetivos, incorporando opciones terapéuticas. Objetivos: describir las características generales de los pacientes según el estado de la enfermedad y el uso de belimumab. Materiales y métodos: estudio de corte transversal, registro RELESSAR. Se definió el estado de la enfermedad como: remisión: SLEDAI=0 y sin corticoides; baja actividad de la enfermedad: SLEDAI >0 y ≤4 y sin corticoides; control no óptimo: SLEDAI >4 y cualquier dosis de corticoides. Resultados: se incluyeron 1.277 pacientes, 23,4% en remisión, 12,6% en baja actividad y 63,8% con control no óptimo. En este último grupo eran más jóvenes y con menor duración de la enfermedad; presentaban mayores índices de actividad y cronicidad, y mayor empleo de inmunosupresores. Solo el 22,3% de los pacientes con criterio potencial de uso de belimumab (lupus eritematoso sistémico activo a pesar del tratamiento estándar) lo recibía en ese momento. Las variables asociadas a hospitalizaciones fueron: terapia con corticoides, ciclofosfamida y mayor SLICC. Conclusiones: se refleja la complejidad del manejo de estos pacientes y se visualizan aspectos estructurales como la desigualdad. El uso del belimumab resultaría beneficioso en los pacientes seleccionados.