ABSTRACT
Resumen La eritromelalgia es una enfermedad rara, crónica, poco conocida, de difícil diagnóstico y tratamiento. Caracterizada por enrojecimiento, calor, dolor intenso y ardor en la parte distal de las extremidades. Afecta fundamentalmente manos, pies, nariz y orejas. El rubor, el dolor y el calor de las extremidades aumentan al exponerse a altas temperaturas, así como después de realizar ejercicio. Los síntomas suelen aliviarse mediante la inmersión de la extremidad afectada en agua fría. Se presenta un caso de una mujer de 33 años de edad, blanca, con afectación predominantemente en las manos. A través del interrogatorio minucioso y la realización de los exámenes complementarios realizados se concluyó que la variante etiológica que presentó la paciente era una eritromelalgia primaria o idiopática. Se impuso tratamiento con aspirina y nifedipino. La evolución clínica de la paciente fue favorable (AU).
ASBTRACT Erythromelalgia is a rare, chronic, little known disease, of difficult diagnosis and treatment. It is characterized by redness, heat, and intense pain and burning in the distal part of the extremities. It mainly affects hands, feet, nose and ears. The flushing, pain and warmth of the extremities increase when exposed to high temperatures as well as after exercise. The symptoms are usually relieved by immersing the affected limb in cold water. The authors present the case of a white, 33-years-old woman, with predominantly involved hand. Through a detailed interview and complementary tests they concluded that the etiological variant presented by the patient was a primary or idiopathic erythromelalgia. Treatment with aspirin and nifedipine was prescribed; the clinical progress of the patient was successful (AU).
Subject(s)
Humans , Female , Signs and Symptoms , Erythromelalgia/epidemiology , Clinical Diagnosis , Clinical Evolution , Erythromelalgia/diagnosis , Erythromelalgia/drug therapyABSTRACT
Abstract Erythromelalgia is a neuropathic pain syndrome due to an autosomal dominant gene, characterized by erythema, increased skin temperature and burning pain in hands and feet, whose treatment is often unsatisfactory. In this paper, we report a case of a 9 years old female patient whose first episode of burning pain, erythema and edema of the hands, without triggering factors, had instant relief after immersion in cold water. She presented with systemic arterial hypertension and had seizures. The patient was treated with gabapentin (150 mg.8 h−1) and amitriptyline (12.5 mg) orally, intravenous lidocaine infusion (120 mg), without relieving pain complaints. Due to the lack of response to the proposed treatment, it was decided to gradually reduce these medications and to introduce carbamazepine (200 mg) orally and, after 4 days of treatment, there was complete relief of the manifestations.
Resumo Eritromelalgia é uma síndrome dolorosa neuropática decorrente de gene autossômico dominante, caracterizada por eritema, aumento da temperatura da pele e dor em queimação, em mãos e pés, e o tratamento é muitas vezes insatisfatório. Neste caso, está o relato de uma paciente do sexo feminino, com nove anos e primeiro episódio de dor em queimação, eritema e edema em mãos, sem fatores desencadeantes, com alívio instantâneo após imersão em água fria. Apresentava hipertensão arterial sistêmica e teve crises convulsivas. Foi tratada com gabapentina (150 mg.8 h-1) e amitriptilina (12,5 mg) via oral, lidocaína (120 mg) venosa em infusão, sem alívio das queixas álgicas. Devido à ausência de resposta ao tratamento proposto, decidiu-se redução gradativa dessas medicações e introdução de carbamazepina (200 mg) via oral e após quatro dias de tratamento houve alívio completo das manifestações.