Incontinentia pigmenti

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the lines of Blaschko: First phase - vesicles on an erythematous base; second phase - verrucous hyperkeratotic lesions; third phase - hyperchromic spots and fourth phase - hypochromic atrophic lesions.

O valor da ultra-sonografia no diagnóstico da apendicite aguda / The role of the ultra-sonography in the diagnosis of acute appendicitis

Apendicite aguda é a causa mais comum de abdome agudo e o principal motivo de cirurgia abdominal de emergência. É diagnosticada com base na avaliação clínica, contagem de leucócitos e exames de imagem. O objetivo do trabalho é avaliar a ultra-sonografia como método complementar em casos suspeitos de apendicite aguda, comparando-a com o resultado do anatomopatológico. Revisamos prontuários dos 320 pacientes submetidos a apendicectomias, no Complexo Hospitalar Santa Casa de Porto Alegre, entre janeiro de 2005 e dezembro de 2006, analisando-se a impressão diagnóstica definida pelo ultra-sonografista e o resultado do exame anatomopatológico. No total, 251 pacientes (78,4%) foram submetidos ao ultra-som no pré-operatório. Destes, 216 pacientes foram diagnosticados com apendicite aguda, pelo exame anatomopatológico. Em 170 pacientes (78,70%) a ecografia abdominal sugeriu o diagnóstico de apendicite aguda, em 21 casos (9,72%) o exame não foi sugestivo e em 25 casos (11,57%) houve dúvida diagnóstica. A sensibilidade verificada do ultra-som foi de 78%, enquanto que a especificidade foi de 42%. O valor preditivo positivo foi de 89,47% e o negativo de 24,59%. A acurácia calculada foi de 73,70%. Excluindo a dúvida, sensibilidade da ecografia foi de 89%, a especificidade de 35%. Valores preditivos positivo e negativo foram, respectivamente, de 89,47% e 34,37%. A acurácia foi de 81,53%. Concluímos que a ultra-sonografia mostra-se como uma importante ferramenta no diagnóstico da apendicite aguda e um excelente método para o diagnóstico diferencial dos quadros sindrômicos da fossa ilíaca direita. Contudo, não se devem substituir as rotinas de anamnese e exame físico para estabelecer o diagnóstico

Acute appendicitis is the most common cause of acute abdominal inflammatory disease and it is the main reason for emergency laparotomy. The diagnosis is based in the clinical evaluation, white blood cels count and diagnostic imaging exams. The aym of the study is to evaluate ultrasonography as a complemental diagnosis method in cases of acute appendicitis comparing with pathologycal exam. We reviewed promptuaries of 320 patients submited to appendicectomies in Santa Casa Hospital of Porto Alegre between January 2005 and December 2006 to analyse the impression of the ultrasonographist and the pathologycal examination of the surgical material. Of those, 251 patients (78,4 %) were submitet to ultrasonography in the pre-operatory. 216 of those had diagnosticaded acute appendicitis in the pathologycal examination. In 170 patients (78,7%), ultrasonography suggested diagnosis of acute appendicitis. In 21 cases (9,72 %) the exam did not suggest inflammatory process and in 25 patients (11,57%) the ultrasonographist had doubt about the diagnosis. The sensibility verificated was 78% and the specificity was 35%. The positive and negative predictive values were 89,47% and 24,59%, respectively. The acuracy was 73,7%. If we eliminate the doubt, the sensibility was 89%, specificity 35%, positive and negative predictive values were 89,47% and 34,37%, respectively. The acuracy was 81,53%. We concluded that ultrasonography is an important diagnostic tool to the appendicitis and an excelent method to discriminate other acute diseases of the right inferior quadrant. However, the exam should not substitute anamnesis and physical examination to establish the diagnosis of acute appendicitis