We describe an 18 year old male who first presented with cutaneous atrophic papulosis but became critically ill from small bowel microperforations. He had an almost immediate and dramatic response to treatment. [ncbi.nlm.nih.gov]

• Pain

  She also was experiencing frequent abdominal pain. Laparoscopy was performed but no serosal lesion suggestive of MAP was seen and her abdominal pain spontaneously abated. [doi.org]

  We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP. [ncbi.nlm.nih.gov]

• Weight Loss

  When the GI tract is involved, it can result in abdominal pain, diarrhea, weight loss, and even intestinal perforation Degos Disease is a complex and progressive condition that has no cure. [dovemed.com]

  Some affected individuals may experience weakness, fatigue and weight loss from malabsorption. The intestinal lesions can tear or rupture (perforate) causing the contents of the intestines to leak into the abdominal cavity. [rarediseases.org]

  0012378 Gastrointestinal hemorrhage Gastrointestinal bleeding 0002239 Gastrointestinal infarctions Death of digestive organ tissue due to poor blood supply 0005244 Intestinal perforation 0031368 Muscle flaccidity 0010547 Nausea and vomiting 0002017 Weight [rarediseases.info.nih.gov]

  He then began to complain of fatigue and experienced weight loss of 15 kg over a period of 2 months. He was admitted due to abdominal pain. [medicaljournals.se]

  Weight loss at an average of 6–10 kg per month is common. A repeat laparotomy is frequently performed because of intestinal occlusion by adherences or acute peritonitis. [dovepress.com]

• Epilepsy

  Nervous system Occlusion of blood vessels in the brain in malignant atrophic papulosis in 20% of patients can lead to strokes, headaches, epilepsy or non-specific neurological symptoms, such as memory loss or altered sensation. [dermnetnz.org]

  Symptoms that may develop from damage to these organs include double vision (diploplia), clouding of lenses of eyes, swelling of the optic disc (papilledema), partial loss of vision, shortness of breath, chest pain, epilepsy, and thickening of pericardium.Someone [en.wikipedia.org]

  When the nervous system is involved the cerebral infarctions manifest as headache, transient neurological defects, multi-infact dementia and epilepsy. [ispub.com]

• Abdominal Pain

  She also was experiencing frequent abdominal pain. Laparoscopy was performed but no serosal lesion suggestive of MAP was seen and her abdominal pain spontaneously abated. [doi.org]

  A 59-year old woman was admitted to our hospital with acute generalized abdominal pain. [ncbi.nlm.nih.gov]

  We report the case of a 60-year-old man who presented with sudden visual loss, a history of postprandial abdominal pain, malabsorption, and skin lesions typical of systemic Degos’ disease. [tandfonline.com]

• Chronic Abdominal Pain

  Jalil et al [17] described a case in a 2-year-old child who presented with chronic abdominal pain of uncertain origin. The benign cutaneous variant of Degos disease can occur in adults. [emedicine.com]

• Abdominal Distension

  Patients experience frequent vomiting and abdominal distension that does not improve after surgery. Weight loss at an average of 6–10 kg per month is common. [dovepress.com]

• Blood in Stool

  […] count Stool guaiac test Antinuclear antibody titer Factor V Leiden, protein C and S, antithrombin III and homocysteine levels Anticardiolipin antibody titer Antiphospholipid antibody titer Endoscopy Laparotomy (Orphanet J Rare Dis 2013;8:10) Laboratory [pathologyoutlines.com]

• Dyspepsia

  […] develop during weeks to years following the onset of skin lesions or even rarely precede the skin lesions. [11] Gastrointestinal tract, predominantly small bowel, is involved in about 50-60% of the patients with clinical manifestations ranging from dyspepsia [e-ijd.org]

• Blurred Vision

  In some patients, history and/or review of systems will reveal signs indicative of extra- cutaneous involvement: abdominal pain, diarrhea, melena, nausea, blurred vision, hemiparesis, paresthesia or any other sign indicative of an ischemic event. [dermaamin.com]

• Papule

  The skin disease shows erythematous papules with characteristic porcelain-white centers and elevated borders. [medical-dictionary.thefreedictionary.com]

  It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. [ncbi.nlm.nih.gov]

  Peristomal atrophic papules. Degos' disease (malignant atrophic papulosis). Archives of Dermatology, 128 (2). DOI: 10.1001/archderm.128.2.256 Bulengo-Ransby, SM, Burns, MK, Taylor, WB, Ellis, CN & Voorhees, JJ 1992, ' Peristomal atrophic papules. [experts.umich.edu]

• Eruptions

  We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. [ncbi.nlm.nih.gov]

  Rash, Myalgia, and Weakness Posted by • June 13th, 2014 In the latest Case Record of the Massachusetts General Hospital, a 32-year-old man was admitted to this hospital because of a violaceous eruption that was followed by muscle pain and weakness, leading [blogs.nejm.org]

• Skin Rash

  The patient had had a symptomless, pathognomonic skin rash for 6 months and thereafter also multiple intestinal infarctions which caused perforations, peritonitis, and finally death. [ncbi.nlm.nih.gov]

  More or less, the first manifestation of DD is skin rash and in about 15% of patients, disease remains limited to skin (benign form) whereas in others progresses to systemic involvement (malignant form). [e-ijd.org]

  Degos' disease: acute abdomen with skin rash. Indian J Gastroenterol. 2006 Jul-Aug. 25(4):187. [Medline]. Kim DW, Kang SB, Lee KH, Choe GY, Park SY, Nicholay M. [emedicine.com]

• Stroke

  Roach chaired the AHA panel that produced the consensus guidelines on childhood stroke and Drs. Lo and Heyer have published extensively on stroke and related topics [books.google.com]

  Blecic and Julien Bogousslavsky, Chapter 8 Unusual Causes of Stroke, Prevention and Treatment of Ischemic Stroke, 10.1016/S1877-3419(09)70083-2, (139-174), (2004). Elizabeth Ball, Amy Newburger and A. [doi.org]

• Burning Sensation

  The lumps are usually painless but occasionally may have a slight burning sensation. [dermcoll.edu.au]

  His medical history was notable for skin lesions persisting for over 3 years, first appeared as pink/reddish rash accompanied by a slight burning sensation then resolved within a few weeks leaving white depressed lesions. [e-ijd.org]

Treprostinil may offer a second effective treatment approach to individuals with MAP or "rescue therapy" to those in whom eculizumab treatment has failed to maintain suppression of disease activity. [ncbi.nlm.nih.gov]

Conclusions Treprostinil may offer a second effective treatment approach to individuals with MAP or “rescue therapy” to those in whom eculizumab treatment has failed to maintain suppression of disease activity. [doi.org]

Malignant atrophic papulosis (MAP) is a rare disease with an extremely grim prognosis, death being due to gastrointestinal perforation and neurological disorders. We report a severe case of MAP in a 37-year old woman. [ncbi.nlm.nih.gov]

Prognosis Due to the markedly different prognosis between the apparently idiopathic cutaneous disease and MAP with systemic involvement, the first variant - in contrast to the latter "malignant" one - has been termed "benign atrophic papulosis" by some [doi.org]

The first chapter stresses the etiology and pathophysiology of childhood stroke as the basis for evaluations and treatment. [books.google.com]

The etiology and pathophysiology are unknown.,NCI: A rare disorder of unknown etiology. It may present as a cutaneous disorder with a benign clinical course or a systemic disorder with a grim prognosis. [linkedlifedata.com]

Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. [ncbi.nlm.nih.gov]

The etiology and pathophysiology are unknown. [purl.bioontology.org]

▪ EPIDEMIOLOGY Classic Degos disease is rare, with less than 200 reported cases. It almost always occurs in Caucasians, but cases have been observed in African American patients and in Japan. [dermaamin.com]

This finding supports the theory that Degos disease is perhaps, in part, an endothelial disease. [13] Epidemiology Frequency Degos disease is rare. About 200 cases have been reported in the world literature. [emedicine.com]

Asymmetric periflexural exanthem of childhood: a clinical, pathologic, and epidemiologic prospective study. Arch Dermatol 1999; 135 (7): 799-803. [scielo.conicyt.cl]

Etiology / Pathophysiology 14 ● Familial cases: Autosomal dominant mode of inheritance (?) ● Are familial cases more benign than sporadic ones ? 32 cases in 10 families: 4 cases with malignant course (12.5%) ! [slideplayer.com]

Gain fresh perspectives and up-to-date insights from the world’s leading authorities on the pathophysiology, diagnosis, and management of stroke. [books.google.com]

Our review of the reported intrathoracic involvement in the more than 60 reported cases of Degos' disease suggests that pleuritis and pericarditis are manifestations of the underlying pathophysiologic process. [ncbi.nlm.nih.gov]

The etiology and pathophysiology are unknown. [purl.bioontology.org]

Prevention of coronary heart disease with pravastatin in men with hypercholesterolemia. ‏ [books.google.com]

Despite this very intensive anticoagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement. [ncbi.nlm.nih.gov]

Blecic and Julien Bogousslavsky, Chapter 8 Unusual Causes of Stroke, Prevention and Treatment of Ischemic Stroke, 10.1016/S1877-3419(09)70083-2, (139-174), (2004). Elizabeth Ball, Amy Newburger and A. [doi.org]

In most cases of familial inheritance, the benign variant of the disease has been present.Due to the lack of knowledge of the pathomechanism for this condition prevention strategies are not known. [en.wikipedia.org]