title: [Wiskott Aldrich syndrome associated to vasculitis: a therapeutic challenge].
abstract: Wiskott Aldrich syndrome (WAS) is an X-linked primary immunodeficiency, associated with WASP gene mutation that causes severe immunological abnormalities and alterations in platelet function. A seven year old male patient with WASP, began with acute abdominal pain, fever and knee swelling. The diagnosis of septic arthritis was made, and he was treated with broad-spectrum antibiotics and human gammaglobulin. During treatment he presented digestive tract bleeding with hypovolemic shock; after 72 hours palpable purpura in upper and lower extremities appeared. Then Henoch-Schönlein purpura with abdominal vasculitis was suspected, and later confirmed by histopathology. Methylprednisolone pulses were initiated, showing improvement within 24 hours. The patient had a severe inflammatory reaction, caused by a serious infectious disease, but the clinical evolution suggested an autoimmune disease such as Henoch-Schönlein purpura. Up to 20% of patients with WAS have autoimmune manifestations of vasculitis. An early diagnosis of autoimmunity in WAS is important for a favorable clinical outcome.
authors.affiliation: Departamento de EducaciÛn MÈdica e InvestigaciÛn, Unidad MÈdica de Alta Especialidad 25, IMSS, Avenida Lincoln y Fidel Vel·zquez, colonia Nuevo Morelos, Monterrey 64180, NL, MÈxico.
authors.forename: Aidé Tamara
authors.initials: AT
authors.lastname: Staines-Boon
authors.forename: Luis Alfredo
authors.initials: LA
authors.lastname: Domínguez-Sansores
authors.forename: Luz María
authors.initials: LM
authors.lastname: Sánchez-Sánchez
authors.forename: Mario
authors.initials: M
authors.lastname: Amaya-Guerra
authors.forename: Diana
authors.initials: D
authors.lastname: González-Cabello
authors.forename: Silvia
authors.initials: S
authors.lastname: Danielian
mesh_terms: D015746:Abdominal Pain; D006801:Humans; D011695:IgA Vasculitis; D014657:Vasculitis; D014923:Wiskott-Aldrich Syndrome; D053632:X-Linked Combined Immunodeficiency Diseases