RESUMEN
Malignant atrophic papulosis (Degos disease) is an unusual thrombotic microangiopathy of uncertain etiology. The disease characteristically involves the skin and internal organs, with nervous system involvement more common in children. We present a case with diverse neurological manifestations including cranial nerve palsies, gait instability, and urinary incontinence. The patient also developed white papular lesions on her lower extremities and back. Magnetic resonance imaging (MRI) demonstrated progressive intracranial and spinal abnormalities. Despite treatment with numerous biologic agents, the patient had persistent clinical deterioration and expired one month after admission. We highlight the extensive neurologic manifestations of Degos disease correlated with neuroradiological imaging and pathological features. Nervous system involvement in Degos disease requires careful neurologic and dermatologic exam with central nervous system (CNS) magnetic resonance imaging to distinguish it from non-organic etiologies of similar symptoms.
Asunto(s)
Imagen por Resonancia Magnética/métodos , Papulosis Atrófica Maligna/diagnóstico por imagen , Papulosis Atrófica Maligna/patología , Enfermedades del Sistema Nervioso/etiología , Microangiopatías Trombóticas/patología , Adolescente , Anticuerpos Monoclonales Humanizados/uso terapéutico , Factores Biológicos/uso terapéutico , Sistema Nervioso Central/diagnóstico por imagen , Sistema Nervioso Central/patología , Enfermedades de los Nervios Craneales/diagnóstico , Enfermedades de los Nervios Craneales/etiología , Progresión de la Enfermedad , Quimioterapia Combinada , Resultado Fatal , Femenino , Trastornos Neurológicos de la Marcha/diagnóstico , Trastornos Neurológicos de la Marcha/etiología , Humanos , Papulosis Atrófica Maligna/complicaciones , Papulosis Atrófica Maligna/tratamiento farmacológico , Enfermedades del Sistema Nervioso/diagnóstico , Nitrilos , Pirazoles/uso terapéutico , Pirimidinas , Piel/patología , Microangiopatías Trombóticas/etiología , Incontinencia Urinaria/diagnóstico , Incontinencia Urinaria/etiologíaRESUMEN
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that can arise in patients with underlying immune disorders. Others have suggested that tumor necrosis factor (TNF)-α inhibitor therapy for immune disorders increases the risk of HSTCL. To assess for a potential relationship between HSTCL and the use of TNF-α inhibitors, we searched for patients with HSTCL and underlying immune disorders at our institution. We identified 7 patients with a median age of 38 years. Five patients had Crohn disease, 1 ulcerative colitis, and 1 rheumatoid arthritis. In 6 patients, medication history for the immune disorder was available: 6 patients received 6-mercaptopurine or azathioprine, and 2 patients received steroids; no patients received TNF-α inhibitors. In all 7 patients, the histologic, immunophenotypic, and cytogenetic findings were similar to cases of HSTCL that arise in immunocompetent patients. We reviewed the literature and identified 60 patients with immune disorders who subsequently developed HSTCL. These patients were treated with immunosuppressive drugs in 89%, TNF-α inhibitors in 56%, and both therapies in 54%, and 1 (2%) patient was treated with TNF-α inhibitors only. Our cohort and literature review indicates that TNF-α inhibitor therapy is not essential for the development of HSTCL in patients with immunodysregulatory disorders, and implies that immunosuppressive drugs or other factors (eg, genetic predisposition, chronic antigenic stimulation) may be more critical in the pathogenesis in this context. Although these data are observational, they have implications for the use of TNF-α inhibitors in patients with inflammatory bowel disease and other immunodysregulatory disorders.