International Surgery Journal | January-March 2016 | Vol 3 | Issue 1 Page 361 International Surgery Journal Vagholkar K et al. Int Surg J. 2016 Feb;3(1):361-363 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 Case Report Bowel perforation in Dego’s disease: a lethal surgical scenario Ketan Vagholkar*, Madhavan Iyengar, Suvarna Vagholkar INTRODUCTION Dego’s disease also described as malignant atrophic papulosis (MAP) is an extremely uncommon condition of the skin associated with multisystem morbid complications. The condition was first described by KohlMeier in 1941 and later documented as a separate entity by Robert Degos. 1 The patho- physiology of the disease continues to be obscure with less than 200 cases reported in world literature. Since multisystem involvement of the disease more so of the gastrointestinal tract is associated with high mortality, awareness of this condition is extremely important for a general surgeon. A case of Dego’s disease manifesting as severe perforative peritonitis is reported along with a brief review of literature. CASE REPORT A 27 year old male presented with history of severe pain in abdomen of 4 hours duration accompanied with vomiting and distention of abdomen. He sought treatment from his family physician. But as the severity of the symptoms increased, he was referred to our surgical unit. On examination he was febrile with a pulse of 110 /min and a B.P of 110/70 mm of Hg. Patient was dehydrated but didn’t have icterus. Examination of the trunk revealed multiple white cutaneous lesions which were papillary in nature (Figure 1). Per abdominal examination revealed board like rigidity. Genitalia were unremarkable. Patient was administered aggressive resuscitation. Laboratory investigations revealed neutrophilic leukocytosis. Rest of the laboratory reports including coagulation profile were within the normal range. Chest x ray revealed free gas under the diaphragm. Ultrasound of the abdomen revealed distended bowel loops and significant amount of free fluid. Patient underwent exploratory laparotomy. At laparotomy there was around 2 liters of purulent fluid and a perforation in the small bowel approximately 1.5 feet distal to the duodenojejunal junction. There was another lesion approximately 1 foot proximal to the IC junction, which showed excessive thinning of the bowel wall .The edges of the perforation were freshened and sutured. For the serosal lesions reinforcing interrupted seromuscular sutures were taken. Drains were placed in the peritoneal ABSTRACT Malignant atrophic papulosis or Dego’s disease is a type of cutaneous disease with involvement of the gastrointestinal and central nervous system. Involvement of the gastrointestinal system by way of perforation invariably leads to a fatal outcome. Awareness of this condition will help in providing a high index of suspicion while managing unexplained multiple intestinal perforations or while dealing with rapidly developing complications in perforative peritonitis. Keywords: Kohlmeier, Dego’s disease, Manifestation, Treatment Department of Surgery, D.Y. Patil University School of Medicine, Navi Mumbai, Maharashtra, India Received: 28 November 2015 Accepted: 07 December 2015 *Correspondence: Dr. Ketan Vagholkar, E-mail: kvagholkar@yahoo.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: http://dx.doi.org/10.18203/2349-2902.isj20151489