Síndrome del dedo azul como manifestación de feocromocitoma / Blue toe syndrome as a clinical finding of pheochromocytoma

Los feocromocitomas son tumores que proceden de las células cromafines del sistema nervioso simpático y actúan sintetizando y liberando catecolaminas. Suelen presentarse entre la cuarta y quinta década de la vida y tienen presentaciones clínicas muy diversas. Ocurren solamente en 0.1-0.2% de la población hipertensa, constituyen una causa tratable y curable de hipertensión arterial, así como de otras manifestaciones derivadas de la liberación incontrolada de catecolaminas. La isquemia arterial periférica secundaria a la liberación masiva de aminas por un feocromocitoma es muy infrecuente. Aquí se presenta un caso clínico de feocromocitoma manifestado como síndrome del dedo azul en un paciente con pulsos distales conservados y el antecedente de mal control tensional a pesar de tratamiento con dos fármacos.

Pheochromocytomas are tumors that arise from chromaffin cells of the sympathetic nervous system and act by synthesizing and releasing catecholamines. They usually occur between the fourth and fifth decade of life and have a very wide clinical presentation. They occur only in 0.1-0.2% of the hypertensive population and represent a treatable and curable cause of arterial hypertension, as well as other symptoms derived from the uncontrolled secretion of catecholamines. Peripheral arterial ischemia secondary to massive amines release by a pheochromocytoma is a very uncommon condition. Here we report a case of pheochromocytoma manifested as blue finger syndrome in a patient with palpable distal pulses and history of poor blood pressure control despite treatment with two drugs.

Síndrome RACAND: presentación de un caso y diagnósticos diferenciales / RACAND syndrome: a case report and differential diagnoses

Se describe una mujer de 57 años, tabaquista, con necrosis digital, fenómeno de Raynaud y anticuerpo anticentrómero en ausencia de esclerodactilia, esclerodermia proximal y distal y trastornos esofágicos. Fueron descartadas patologías neoplásicas, otras enfermedades del tejido conectivo y enfermedades vasculares, las cuales no fueron desarrolladas durante el tiempo de seguimiento de esta paciente. Se concluye que la paciente tuvo un síndrome RACAND, el cual no evolucionó a otra enfermedad del tejido conectivo hasta el fallecimiento de la misma.

We described a 57-year old woman smoker, with digital necrosis, Raynaud phenomenon and positive anticentromere antibody. Shedidn’t show sclerodactily, proximal or distal scleroderma or esophagealdisorders. Malignancies, other connective tissues diseases andvascular diseases were ruled out and were not developed during thefollow up. We conclude that this patient had a RACAND syndrome,which do not evolved into other connective disease until the deathof the patient.

Necrose digital em paciente com lúpus eritematoso sistêmico e esclerose sistêmica tratada com inibidores da fosfodiesterase / Digital necrosis treated with phosphodiesterase inhibitors in a patient with connective tissue disease

Os inibidores da fosfodiesterase têm sido introduzidos, nos últimos anos, como novos agentes farmacológicos no tratamento dos pacientes com fenômeno de Raynaud e isquemia digital. Será descrito o caso de uma paciente com lúpus eritematoso sistêmico e esclerose sistêmica limitada apresentando fenômeno de Raynaud grave e necrose digital refratária à terapia. A paciente obteve excelente resposta à associação de imunossupressão e sildenafil.

The phosphodiesterase inhibitors have been used recently for the treatment of Raynaud's phenomenon and digital ischaemia. We report the case of a patient affected by systemic lupus erythematosus and limited systemic sclerosis who presented severe Raynaud's phenomenon with digital necrosis despite treatment. The patient presented an excellent response to the association of immunosuppressant therapy and sildenafil.

Hypereosinophilic Syndrome Accompanied by Digital Necrosis

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders with the common feature of prolonged eosinophilia of unknown cause and associated dysfunctions of organ systems, including the hematologic, cardiovascular, cutaneous, neurologic and respiratory systems, as well as the spleen, liver, eye, and gastrointestinal tract. Bone marrow is involved in almost all subjects, and the most severe complications involve the heart and the central nervous systems. We report a case of HES observed in a 37-year-old man with digital necrosis and persistent eosinophilia lasting for more than 6 months. This patient also showed signs of peripheral neuropathy, cutaneous manifestations, and eosinophilic hyperplasia on the bone marrow biopsy.