Pediatrics 2018 Apr;141(Suppl 5):S481-S484

Departments of Pediatrics and

Malignant atrophic papulosis (MAP), also known as Degos disease, is an extremely rare disease that is characterized by its unique skin presentation (namely, central, porcelain-white atrophic lesions with a telangiectatic rim). MAP has the following 2 variants: cutaneous MAP is manifested in the skin alone, whereas systemic MAP affects the gastrointestinal tract, central nervous system, lungs, and other internal organs. Some patients who presented with only cutaneous symptoms at first may develop systemic symptoms several years later. Read More

J Dermatol 2018 Mar 8. Epub 2018 Mar 8.

Department of Critical Care Medicine, Chinese People's Liberation Army General Hospital, Beijing, China.

Malignant atrophic papulosis (MAP) is a rare type of obliterating vasculopathy that can present as pure cutaneous lesions or a systemic entity affecting multiple organs. Systemic disease, such as gastrointestinal or central nervous system involvement, may predispose the patients to poorer or even fatal outcomes. We present a 30-year-old female patient with systemic manifestation of MAP 10 days after delivery of a full-term pregnancy who subsequently developed motor aphasia and intestinal perforation. Read More

J Clin Neurosci 2018 Feb 26;48:114-117. Epub 2017 Nov 26.

Department of Radiology, the Royal Melbourne Hospital, Parkville, VIC, Australia; Department of Radiology, the University of Melbourne, Parkville, VIC, Australia.

Köhlmeier-Degos disease is rare idiopathic vasculopathy, the exact pathogenesis of which remains unclear. Here, we review pertinent literatutre and present a case of a Köhlmeier-Degos disease with central nervous system involvement followed-up over 11 years with various neuroimaging modalities. Evolution of neurovascular and neuropathological changes over an extended time period has not been previously described. Read More

Clin Exp Dermatol 2017 Oct 10;42(7):774-776. Epub 2017 Jul 10.

Department of Dermatology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge Biomedical Campus, Cambridge, UK.

Systemic lupus erythematosus (SLE) is an autoimmune disease. Its incidence in the UK is approximately 1 per 10 000. Cutaneous involvement, encompassing acute, subacute and chronic disease, occurs in over two-thirds of cases, and can often be the first clue to diagnosis. Read More

J Eur Acad Dermatol Venereol 2017 Oct 20;31(10):1753-1756. Epub 2017 Jun 20.

Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, NY, USA.

Background: Atrophic papulosis is a rare thrombo-occlusive disease, characterized by the appearance of multiple atrophic porcelain-white skin papules, with a surrounding erythematous rim, which are histologically consisting of wedge-shaped necrosis of the dermis.

Objective: It consists of two variants: (i) the benign atrophic papulosis (BAP) only involving the skin and (ii) the malignant atrophic papulosis (MAP) also involving several internal organs with a cumulative five-year survival rate of approx. 55%. Read More

J Neuroophthalmol 2017 09;37(3):303-308

Departments of Ophthalmology (AAT, JJC, JAL), Radiology (LJE), and Pathology (DRS), Mayo Clinic, Rochester, Minnesota.

Ann Dermatol 2017 Apr 24;29(2):215-218. Epub 2017 Mar 24.

Department of Dermatology, Kosin University College of Medicine, Busan, Korea.

Degos disease, also referred to as malignant atrophic papulosis, was first described in 1941 by Köhlmeier and was independently described by Degos in 1942. Degos disease is characterized by diffuse, papular skin eruptions with porcelain-white centers and slightly raised erythematous telangiectatic rims associated with bowel infarction. Although the etiology of Degos disease is unknown, autoimmune diseases, coagulation disorders, and vasculitis have all been considered as underlying pathogenic mechanisms. Read More

Pediatr Dermatol 2017 May 20;34(3):e109-e115. Epub 2017 Mar 20.

Department of Dermatology, Instituto Nacional de Salud del Niño, Lima, Perú.

Pediatric Degos disease is rare, with only 36 cases reported in the medical literature. Classically the diagnosis has been established according to pathognomonic histopathologic findings, but when these features are not present, there may be a delay in diagnosis. We report the second congenital case of Degos disease, highlighting the clinical and dermoscopic findings. Read More

Am J Dermatopathol 2018 Apr;40(4):272-274

Department of Dermatology, University of Florida College of Medicine, Gainesville, FL.

Degos disease manifests as 2 distinct clinical variants, malignant atrophic papulosis and benign atrophic papulosis, which are distinguished by the presence or absence of systemic disease. Both forms feature cutaneous involvement typified by erythematous papules with scar-like centers, and the classic histologic picture is described as wedge-shaped dermal necrosis overlying thrombotic vasculopathy. However, the histopathology of early lesions is distinct and more variable. Read More

Clin Exp Dermatol 2016 Dec 26;41(8):948-950. Epub 2016 Oct 26.

Department of Dermatology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

J Am Acad Dermatol 2016 Dec 4;75(6):1274-1277. Epub 2016 Oct 4.

Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland. Electronic address:

KEY TEACHING POINTS. Read More

J Dtsch Dermatol Ges 2016 Aug 9;14(8):839-40. Epub 2016 Aug 9.

Department of Dermatology, University Hospital Jena, Jena, Germany.

Zhonghua Yi Xue Za Zhi 2015 Dec;95(46):3750-2

Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing 210042, China.

Objective: To investigate the clinical presentation, histopathological features, progression, and treatment of lymphomatoid papulosis (LyP).

Methods: A retrospective review was performed on clinicopathological data of 22 patients diagnosed with LyP from June 2010 to March 2015 in Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College.

Result: The mean age of the 22 LyP patients was 39 years (range: 7-83 years). Read More

BMC Gastroenterol 2015 Nov 2;15:156. Epub 2015 Nov 2.

Gastroenterology Consultants, Albany, NY, USA.

Background: The malignant form of atrophic papulosis (Köhlmeier-Degos disease) is a rare thrombo-occlusive vasculopathy that can affect multiple organ systems. Patients typically present with distinctive skin lesions reflective of vascular drop out. The small bowel is the most common internal organ involved, resulting in considerable morbidity and mortality attributable to ischemic microperforations. Read More

J Am Acad Dermatol 2015 Nov;73(5):894-6

Tampa, Florida.

Dermatol Online J 2015 Sep 17;21(9). Epub 2015 Sep 17.

Weill Cornell College of Cornell University.

Background: In 2012, a nephrologist reported the development of a multiorgan thrombotic syndromic complex resembling thrombotic thrombocytopenic purpura (TTP) in patients who were abusing long acting oxymorphone hydrochloride; all patients had hemolytic anemia and thrombocytopenia.

Objective: Herein, we report another case involving a 31-year-old woman who self intravenously administered dissolved oral oxymorphone resulting in thrombotic sequelae resembling Degos disease.

Methods: Formalin-fixed and paraffin embedded skin biopsies were prepared according to standard protocols for H&E and immunohistochemistry. Read More

An Bras Dermatol 2015 May-Jun;90(3 Suppl 1):19-21

Jinling Hospital, Medical School, Nanjing University, Nanjing, CN.

Malignant atrophic papulosisis is a rare, multisystem obliterative vasculopathy of unknown etiology, occasionally involving the cranial nerve. We describe the first case of malignant atrophic papulosisis with cranial nerve and peripheral nerve involvement in China. A 47-year-old woman presented to our hospital with atrophic porcelain white papules over the trunk and extremities, numbness in the right calf, vision decrease and impaired movement of the right eye. Read More

Clin Exp Gastroenterol 2015 16;8:141-7. Epub 2015 Apr 16.

Sirio Libanes Hospital, São Paulo, Brazil.

Background: Degos disease is a very rare syndrome with a rare type of multisystem vasculopathy of unknown cause that affects the skin, gastrointestinal tract, and central nervous system. Other organs such as the kidneys, lungs, pleura, liver, heart, and eyes, can also be involved.

Objective: To highlight the incidence of Degos disease with regard to age and sex, discuss the necessity of its accurate and early diagnosis, and demonstrate the most current techniques for its diagnosis; to discuss whether early therapeutic intervention can impact patient prognosis; and to present a literature review about this disease. Read More

An Bras Dermatol 2015 Mar-Apr;90(2):285

Nephrology-Dialysis Department, Civico and Di Cristina Hospital, Palermo, Italy.

G Ital Dermatol Venereol 2015 Feb;150(1):123-6

Dermatology Section, Department of Clinical Medicine and Immunological Sciences, University of Siena, Siena, Italy -

We report the case of a 20-year-old woman with one-year history of asymptomatic pink papules on the abdomen, with central atrophy. Fever and symptoms suggesting involvement of other organs were absent. Histological examination revealed wedge-shaped area of cutaneous ischemia extending into the deep dermis with superficial and deep perivascular lymphocytic infiltrate. Read More

Rheum Dis Clin North Am 2015 ;41(1):141-60, ix

Rheumatology Unit, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, USA.

The need to distinguish true primary systemic vasculitis from its multiple potential mimickers is one of the most challenging diagnostic conundrums in clinical medicine. This article reviews 9 challenging vasculitis mimickers: fibromuscular dysplasia, calciphylaxis, segmental arterial mediolysis, antiphospholipid syndrome, hypereosinophilic syndrome, lymphomatoid granulomatosis, malignant atrophic papulosis, livedoid vasculopathy, and immunoglobulin G4-related disease. Read More

N Engl J Med 2014 Oct;371(14):1361

N Engl J Med 2014 Oct;371(14):1361-2

J Eur Acad Dermatol Venereol 2016 Jan 1;30(1):126-8. Epub 2014 Jul 1.

Insitute of Dermatology and Venereology, Ospedale Maggiore di Trieste, University of Trieste, Trieste, Italy.

An Bras Dermatol 2014 May-Jun;89(3):521-2

Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, RJ, Brazil.

Degos disease, also known as malignant atrophic papulosis, is a rare occlusive vasculopathy of unknown etiology characterized by infarcts in the dermis, gastrointestinal tract, central nervous system, and other organs. It is characterized by papules, which become umbilicated and evolve with a depressed porcelain-white central area, with an erythematous halo with telangiectasias. Histological findings include wedge-shaped dermoepidermal necrosis and blood vessel thrombosis. Read More

N Engl J Med 2014 Jun;370(24):2327-37

Br J Dermatol 2014 Apr;170(4):992-4

Department of Dermatology, Lyon 1 University - Centre Hospitalier Lyon Sud, 69495, PIERRE BENITE Cedex, France.

Int J Dermatol 2014 May 14;53(5):631-4. Epub 2014 Feb 14.

Department of Dermatology, China-Japan Union Hospital of Jilin University, Changchun, Jilin, China.

Mod Rheumatol 2015 May 7;25(3):480-3. Epub 2014 Feb 7.

Division of Rheumatology, Department of Medicine, Showa University School of Medicine , Tokyo , Japan.

A 68-year-old Japanese male presented with atrophic erythematous white lesions with peripheral dark reddish rims on his back. Multiple ulcers were detected from his stomach to his large intestine using endoscopy. Although the patient was given high doses of a steroid, aspirin, dipyridamole, and intravenous immunoglobulin therapy, he died of gastrointestinal hemorrhage, perforation and septic shock. Read More

CNS Neurosci Ther 2014 Jan;20(1):88-91

Department of Dermatology, Xinhua Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Eur J Dermatol 2013 Sep-Oct;23(5):742-3

Clinic of Dermatovenerology and Allergology, Military Medical Academy Sofia, Bulgaria. 3, Gueorgui Sofiiski Str., 1606, Sofia, Bulgaria.

Orphanet J Rare Dis 2013 Nov 26;8:185. Epub 2013 Nov 26.

Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, Box 58, Room F-309, 1300 York Avenue, New York, New York 10065, USA.

Background: Degos disease is a frequently fatal and incurable occlusive vasculopathy most commonly affecting the skin, gastrointestinal tract and brain. Vascular C5b-9 deposition and a type I interferon (IFN) rich microenvironment are held to be pathogenetically important in the evolution of the vascular changes. We recently discovered the use of eculizumab as a salvage drug in the treatment of near fatal Malignant atrophic papulosis (MAP). Read More

Br J Dermatol 2014 Jan;170(1):110-5

Departments of Dermatology, Venereology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, 06847, Dessau, Germany; Freiburg Vein Center, Zähringer Straße 14, 79108, Freiburg, Germany.

Background: Atrophic papulosis (Köhlmeier-Degos disease) is a rare disease of unknown aetiology. The cutaneous signs--papular skin lesions with central porcelain-white atrophy and surrounding telangiectatic rim--are almost pathognomonic. Extracutaneous, systemic involvement includes multiple limited infarcts of the gastrointestinal system, central nervous system and other organs. Read More

JAMA Dermatol 2014 Jan;150(1):96-7

Department of Dermatology, University Hospital of Nice, Nice, France3INSERM U1065 C3M Team 1, Nice, France4Cliniacal Research Center, University Hospital of Nice, Nice, France.

Int J Dermatol 2015 Jun 20;54(6):699-703. Epub 2013 Jun 20.

Center for Translational Medicine and Jiangsu Key Laboratory of Molecular Medicine, Medical School of Nanjing Universityg, Nanjing, China.

Orphanet J Rare Dis 2013 Apr 4;8:52. Epub 2013 Apr 4.

Steffens Scleroderma Center, Saratoga Springs, NY, USA.

Background: Malignant atrophic papulosis (Köhlmeier-Degos disease; MAP) is an uncommon endotheliopathy with pathological findings similar to the vascular lesions of systemic sclerosis. These two disorders can overlap. When associated with visceral lesions, MAP has been considered almost universally and rapidly fatal. Read More

Hong Kong Med J 2013 Apr;19(2):174-7

Department of Diagnostic Radiology, Princess Margaret Hospital, Laichikok, Hong Kong.

We describe a 50-year-old man who first presented with multiple skin lesions which were characteristic of Degos' syndrome. The patient developed multiple episodes of abdominal pain. Some episodes resolved with conservative management, for others he underwent urgent operations for bowel perforations. Read More

J Child Neurol 2014 Apr 11;29(4):550-4. Epub 2013 Feb 11.

1Department of Pediatric Neurology, Dokuz Eylul University Medical School, Izmir, Turkey.

A 14-year-old male presented with paresthesias on the right upper and lower extremities, headache, and vomiting. In addition to worsening paresthesia and weakness on the right side of his body, blurred vision, fever, and skin lesions developed. He also had skin lesions characterized with 3-10 mm papules with a white atrophic center surrounded by pink rim mostly on the trunk and lower extremities. Read More

J Am Acad Dermatol 2013 Feb;68(2):211.e1-33; quiz 244-6

Division of Dermatology, Department of Internal Medicine, Baylor University Medical Center, Dallas, Texas 75246, USA.

The gastrointestinal (GI) and cutaneous organ systems are closely linked. In part I of this continuing medical education article, the intricacies of this relationship were explored as they pertained to hereditary polyposis disorders, hamartomatous disorders, and paraneoplastic disease. Part II focuses on the cutaneous system's links to inflammatory bowel disease and vascular disorders. Read More

Orphanet J Rare Dis 2013 Jan 14;8:10. Epub 2013 Jan 14.

Departments of Dermatology, Venerology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, Dessau, 06847, Germany.

Unlabelled: DEFINITION OF THE DISEASE: Malignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.

Epidemiology: Less than 200 cases have been described in the literature. Read More

J Dtsch Dermatol Ges 2013 Jan 26;11(1):86-8. Epub 2012 Nov 26.

Department of Dermatology, Venereology, and Allergology, University of Leipzig Hospital, Germany.

J Am Acad Dermatol 2013 Jan 27;68(1):138-43. Epub 2012 Aug 27.

Department of Dermatology, Jichi Medical University, Tochigi, Japan.

Background: Degos disease or malignant atrophic papulosis is a rare occlusive vasculopathic disease characterized by pathognomonic cutaneous lesions and frequently fatal systemic involvement. The etiology of malignant atrophic papulosis remains unclear, and there is currently no effective treatment for malignant atrophic papulosis. Several chemokines can potentiate and expand the platelet response to increase thrombus formation. Read More

World J Gastrointest Surg 2011 Oct;3(10):156-8

Mona Ahmadi, Sevil Agabalaey Rafi, Zhale Faham, Ramin Azhough, Samad Beheshty Rooy, Omid Rahmani, Department of General Surgery and Pathology, Imam Reza Hospital, Azadi Street, Tabriz 5156975389, Iran.

Degos' disease, otherwise known as "malignant atrophic papulosis" is a rare vasculopathy with an unknown etiology characterized by typical cutaneous lesions. Involvement of the gastrointestinal (GI) tract is observed in approximately half of patients and small infarctions in the mucosa can cause perforation and resulting peritonitis, the leading cause of death. We present a fatal case of Degos' disease with skin and GI involvement, manifesting as recurrent intestinal perforations and peritonitis, in a 15-year-old Iranian boy. Read More

Dermatol Online J 2011 Aug 15;17(8). Epub 2011 Aug 15.

Columbia University, New York City, NY, USA.

Magro et al in April of 2011 published a new article in the American Journal of Clinical Pathology on the etiology and treatment of Degos Disease (DD), and importantly, its fatal variant malignant atrophic papulosis (MAP). Specifically, Magro noted that MAP is a disease involving the complement cascade that can be treated effectively with eculizumab. DD has two variants, a benign variant confined to the skin and a malignant (heretofore fatal) variant that involves the skin and systemic organs. Read More

J Am Acad Dermatol 2011 Aug;65(2):e49-50

Int J Dermatol 2011 Jun;50(6):726-9

Department of Health, Social Hygiene Service, Centre for Health Protection, Hong Kong, China.

J Cutan Med Surg 2011 May-Jun;15(3):162-6

Department of Dermatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Background: Degos disease is a type of vasculopathy characterized by progressive occlusion of small-sized blood vessels primarily involving the skin, gastrointestinal system, and central nervous system as well as various other systems. Owing to the rarity of the condition, the diagnosis is often a challenge; consequently, management is even more difficult owing to the paucity of experience and literature for the effective treatment of this entity.

Case Presentation: We report a case of a 50-year-old male patient with classic skin lesions and rapidly progressive fatal clinical course involving multiple organs associated with dermatomyositis-like features. Read More

Int J Dermatol 2011 May;50(5):604-6

Dermatology Department, Hospital Clinic of Barcelona, University of Barcelona, Spain.

Am J Clin Pathol 2011 Apr;135(4):599-610

Division of Dermatopathology, Dept of Pathology and Laboratory Medicine, Weill Cornell College of Cornell University, New York, NY 10065, USA.

Degos disease is a lethal small vessel angiopathy targeting the skin, gastrointestinal tract, and central nervous system, potentially developing in the setting of known autoimmune disease, although forme fruste primary variants exist. Its pathogenetic basis is unknown. Four cases of Degos disease were encountered in archival material, representing 2 men, ages 38 and 43 years, and 2 females, ages 48 and 2 years; 3 patients died of disease. Read More

Indian J Dermatol Venereol Leprol 2011 Mar-Apr;77(2):219-21