Primary meningococcal septic arthritis associated with joint calcium oxalate crystals: A case report and review of the literature.

Primary meningococcal septic arthritis (PMSA) is an extremely rare local infection by Neisseria meningitidis in the absence of meningitis or meningococcaemia syndrome. A 30-year-old healthy, immunocompetent man presented with arthralgia, fever, chest rash, and significant swelling of the right knee. On admission, a disseminated maculopapular and purpuric rash, oligoarthritis, neutrophilia, and elevated acute phase reactants were documented. Following arthrocentesis of the right knee, isolation of N. meningitidis and the presence of calcium oxalate crystals in the synovial fluid were reported. The diagnosis of PMSA was made. Histological analysis of the skin lesion showed leucocytoclastic vasculitis. He was treated with intravenous ceftriaxone plus open surgical drainage and ambulatory cefixime with adequate response. After 1 month, he presented resolution of the pathological process. We performed an extensive review of the literature, finding that the key elements supporting the diagnosis of PMSA are prodromal upper respiratory tract symptoms and skin involvement prior to or synchronous with the arthritis. Also, the most frequently involved joint is the knee. This report is the first case of a patient presenting with PMSA associated with calcium oxalate crystals in the synovial fluid. Herein, we discuss the most frequent clinical manifestations, the unusual histological features, the recommended treatment, and the reported prognosis of this rare entity.

Acquired hemophilia: clinical case of hemorrhage caused by inhibitors against factor VIII. / Hemofilia adquirida: caso clínico de hemorragia por inhibidores contra el factor VIII.

BACKGROUND: Acquired hemophilia is caused by antibodies against coagulation factors, especially against factor VIII. As it is a low prevalence disease, our objective is to present an idiopathic case of acquired hemophilia to facilitate decision-making in clinical practice for others, exposing the therapeutic modality used. CASE REPORT: 65 years old woman, started with non-trauma hematomas, with asthenia, adynamia and dyspnea. Laboratory studies showed anemia (hemoglobin 7.1 g/dl, hematocrit 21%, mean corpuscular volume 91 fl, mean corpuscular hemoglobin 29.6 pg) and long clotting times (activated partial thromboplastin time 100 seconds) and with prothrombin time and International Normalized Index (17 seconds and 1.2 seconds respectively). So, plasma dilution tests were performed with plasma, without aPTT correction (1:2, 76.6 seconds; 1: 4, 60 seconds; 1: 8, 45 seconds) evidencing the presence of the inhibitor. The diagnosis was confirmed by the decrease in factor VIII (1%) and the presence of the factor VIII inhibitor (256 UB). The treatment started with prednisone and cyclophosphamide. Due to the presence of severe bleeding, treatment with activated prothrombin complex concentrate (CCpa) was required. Subsequently, Rituximab was prescribed, with clinical improvement and increased levels of hgb and reduced aPTT from the first week of treatment. CONCLUSIONS: The approach of acquired hemophilia should include control of the bleeding, the search for the etiology ruling out of the main causes of malignant neoplasms according to age and gender, and the immediate initiation of immunosuppression to decrease inhibitor levels.

INTRODUCCIÓN: La hemofilia adquirida es ocasionada por anticuerpos contra los factores de la coagulación, en especial contra el factor VIII. Por ser de baja prevalencia, presentamos un caso de hemofilia adquirida idiopática exponiendo la modalidad terapéutica utilizada para facilitar a otros la toma de decisiones en la práctica clínica. CASO CLÍNICO: Mujer de 65 años que inició con hematomas no asociados a traumatismo, con astenia, adinamia y disnea. Los estudios de laboratorio mostraron anemia (hemoglobina 7.1 g/dl, hematocrito 21%, volumen corpuscular medio 91 fl, hemoglobina corpuscular media 29.6 pg) y tiempos de coagulación prolongados (tiempo de tromboplastina parcial activado [TTPa] 100 segundos), con tiempo de protrombina e INR (International Normalized Ratio) dentro de parámetros normales (17 y 1.2 segundos, respectivamente), por lo que se realizaron pruebas de dilución con plasma sin corrección del TTPa (1:2, 76.6 segundos; 1:4, 60 segundos; 1:8, 45 segundos), evidenciando la presencia del inhibidor. El diagnóstico se confirmó con la disminución del factor VIII (1%) y la presencia de inhibidor del factor VIII (256 UB). Se inició tratamiento con prednisona y ciclofosfamida. Por presentar hemorragia grave requirió tratamiento con concentrado de complejo de protrombina activado y posteriormente se indicó rituximab, con mejoría clínica, incremento en los niveles de hemoglobina y reducción del TTPa desde la primera semana de tratamiento. CONCLUSIONES: El abordaje de la hemofilia adquirida debe incluir el control de la hemorragia, la búsqueda de la etiología descartando las principales causas de neoplasias malignas de acuerdo con la edad y el sexo, y el inicio inmediato de inmunosupresión para disminuir los niveles de inhibidor.

Parry-Romberg, a neurocutaneous syndrome: presentation of three cases. / Parry-Romberg, un síndrome neurocutáneo: presentación de tres casos.

BACKGROUND: Parry-Romberg syndrome is characterized by progressive hemiatrophy of the skin, subcutaneous tissue, muscle and bones of the skull. Its incidence is low, with a progressive and slow course. Its etiology is unknown, but it has been associated with several factors. Its clinical presentation involves dermatological, musculoskeletal and neuropsychiatric manifestations. The treatment consists of medical and surgical strategies. The use of steroids, alone or in combination with immunomodulators, has the objective of slowing down progression. The surgical treatment lies in facial reconstruction or volumetric regeneration, to correct the appearance and function of facial structures. The objective is to show three cases of Parry-Romberg syndrome with the representative characteristics of the disease. CLINICAL CASEs: 1) A 41-year-old woman with atrophy and right supraciliary hypochromia associated with seizures and headache. 2) A 43-year-old woman with parietal deformation and right supraciliary atrophy, associated with facial paralysis, depression and headache. 3) A 36-year-old woman with right hemifacial atrophy associated with ocular involvement and headache. The indicated treatment was based on a systemic steroid plus a cytostatic agent. The surgical treatment was evaluated according to the affection of facial structures. CONCLUSIONS: Parry-Romberg syndrome is a rare disease, characterized by progressive atrophy. Clinical manifestations determine the diagnosis and early start of medical and surgical treatment.

INTRODUCCIÓN: El síndrome de Parry-Romberg se caracteriza por hemiatrofia progresiva de la piel, el tejido subcutáneo, los músculos y los huesos del cráneo. Su incidencia es baja, y su curso es progresivo y lento. Se desconoce su etiología, pero se ha asociado a diversos factores. Su presentación clínica involucra manifestaciones dermatológicas, musculoesqueléticas y neuropsiquiátricas. El tratamiento consiste en estrategias médicas y quirúrgicas. El uso de esteroides, solos o en combinación con inmunomoduladores, tiene el objetivo de frenar la progresión. El tratamiento quirúrgico consiste en reconstrucción facial o regeneración volumétrica, a fin de corregir la apariencia y la función de las estructuras faciales. Se presentan tres casos de síndrome de Parry-Romberg con las características representativas de la enfermedad. CASOS CLÍNICOS: 1) Mujer de 41 años, con atrofia e hipocromía supraciliar derecha, asociadas a crisis convulsivas y cefalea. 2) Mujer de 43 años, con deformación parietal y atrofia supraciliar derecha, asociadas a parálisis facial, depresión y cefalea. 3) Mujer de 36 años, con atrofia hemifacial derecha asociada a afección ocular y cefalea. El tratamiento se basó en esteroide sistémico más un agente citostático. El tratamiento quirúrgico se valoró de acuerdo con la afectación de la estructuras faciales. CONCLUSIONES: El síndrome de Parry-Romberg es una enfermedad rara caracterizada por una atrofia progresiva. Las manifestaciones clínicas determinan el diagnóstico y el inicio temprano del tratamiento médico y quirúrgico.