RESUMEN
Scleromyxedema is a rare systemic disorder characterized by cutaneous sclerosis and papulosis, accompanied by deposition of mucin in the skin and other organs. We describe a case of scleromyxedema in a 62-year-old man. The cutaneous symptoms of the disorder were preceded by episodes of acute central nervous system dysfunction that included mental confusion, hemiparesis, tremor, and migraine. As the cutaneous symptoms progressed, the patient experienced persistent confusion and difficulty concentrating. Therapy with melphalan and plasmapheresis led to complete resolution of the cutaneous symptoms as well as near-resolution of the neurologic symptoms. This is the first report to describe the successful treatment of the cutaneous symptoms of scleromyxedema accompanied by reversal of chronic neurologic dysfunction.
Asunto(s)
Enfermedades del Sistema Nervioso Central/terapia , Erupciones Liquenoides/terapia , Mixedema/terapia , Esclerodermia Sistémica/terapia , Enfermedades del Sistema Nervioso Central/complicaciones , Enfermedades del Sistema Nervioso Central/diagnóstico , Humanos , Erupciones Liquenoides/complicaciones , Erupciones Liquenoides/diagnóstico , Masculino , Persona de Mediana Edad , Mixedema/complicaciones , Mixedema/diagnóstico , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/diagnósticoRESUMEN
Skin infection secondary to gram-negative organisms is uncommon and is typically limited to persons who are immunocompromised. When these do occur, they are acute, progressive, and severe. Here we report 2 cases of painful red nodules that presented with a waxing and waning course over a long period. One case is that of a 45-year-old healthy white man who developed Serratia marcescens infection in 1 leg. The other case is that of a 78-year-old man with chronic lymphocytic leukemia treated with prednisone who developed infection of the leg secondary to Pseudomonas aeruginosa. In the first case, symptoms were present for 2 years before definitive diagnosis and treatment. In the second case, 4 months elapsed. Ultimately, both patients responded to antibiotic therapy and recovered. These cases illustrate an unusual presentation of chronic red painful nodules of the leg secondary to infection with gram-negative organisms and underscore the importance of culture even when infection seems unlikely.