RESUMEN
Scleromyxedema is a rare idiopathic fibromucinous disorder characterized by a generalized papular and sclerodermoid cutaneous eruption. Patients often have praraproteinemia and extracutaneous, even lethal, manifestations. Yet the prognostic and therapeutic features of scleromyxedema are poorly documented. High-dose intravenous immunoglobulin (IVIG), used either alone or in conjunction with systemic steroids and/or thalidomide, has been suggested as a first-line treatment. We report the case of a 45-year-old woman diagnosed with scleromyxedema with paraproteinemia that initially did not respond to systemic steroids, retinoids, and thalidomide but greatly improvement in terms of systemic and cutaneous symptoms after treatment with IVIG.
Asunto(s)
Exantema , Paraproteinemias , Escleromixedema , Femenino , Humanos , Persona de Mediana Edad , Escleromixedema/diagnóstico , Escleromixedema/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Talidomida/uso terapéutico , Enfermedades Raras , Paraproteinemias/complicaciones , Paraproteinemias/diagnóstico , Paraproteinemias/tratamiento farmacológicoAsunto(s)
Corticoesteroides/administración & dosificación , Terapia PUVA/métodos , Retinoides/administración & dosificación , Escleromixedema/diagnóstico , Escleromixedema/tratamiento farmacológico , Talidomida/uso terapéutico , Adolescente , Adulto , Terapia Combinada/métodos , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Resultado del TratamientoRESUMEN
In this report, we review 5 sclerodermiform cutaneous conditions: eosinophilic fasciitis, systemic nephrogenic fibrosis, scleredema, scleromyxedema, and toxic oil syndrome. We emphasize the morphological differences between the conditions and some morphological clues that are important to differential diagnosis.