Received 28 September 2022. Accepted 15 February 2024
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Table 1. Differential diagnosis of systemic manifestations of scleromyxedema and systemic sclerosis.
Abstract
Scleromyxoedema is a cutaneous fibromucinosis of unknown aetiology. It is associated with haematological dyscrasias and quite diverse manifestations. Pulmonary vascular involvement is rare and requires a differential diagnosis approach with systemic sclerosis. The case of a patient with scleromyxoedema with an extracutaneous pulmonary manifestation is described.
Keywords:
Scleromyxoedema
Pulmonary hypertension
Monoclonal gammopathy of undetermined significance
Resumen
El escleromixedema es una fibromucinosis cutánea de etiología desconocida. Se asocia a discrasias hematológicas y a manifestaciones sistémicas muy diversas. El compromiso vascular pulmonar es poco frecuente y requiere un abordaje de diagnóstico diferencial con la esclerosis sistémica. Se describe el caso de un paciente con escleromixedema con manifestación extracutánea pulmonar.
Palabras clave:
Escleromixedema
Hipertensión pulmonar
Gammapatía monoclonal de significado inciertoribed
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