Diffuse atrophic papules and plaques, intermittent abdominal pain, paresthesias, and cardiac abnormalities in a 55-year-old woman
Reference: Journal of the American Academy of Dermatology, Volume 75, Issue 6, 2016 Dec
Case summary History A 55-year-old Caucasian woman was referred to the National Institutes of Health (NIHacrnm1) for evaluation of widespread white papules and plaques, intermittent abdominal pain, cardiac abnormalities, and paresthesias. Symptoms…
Degos disease mimicking primary vasculitis of the CNS
Reference: Neurol Neuroimmunol Neuroinflamm April 2016 vol. 3 no. 2 e206
A 4-year-old boy developed a headache. Initial evaluation revealed a normal neurologic examination and a right subdural hygroma on CT. Worsening headaches led to hospitalization at an outside institution. MRI showed leptomeningeal enhancement; magnetic resonance angiography (MRA) was normal. Infectious, rheumatologic, hematologic, and CSF studies were unrevealing. He then developed a left-sided hemiparesis. Imaging showed increased leptomeningeal enhancement with punctate infarcts in the right hemisphere.
Bowel perforation in Dego’s disease: a lethal surgical scenario
Reference: Int Surg J. 2016; 3(1): 361-363doi: 10.18203/2349-2902.isj20151489
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.
Aspirin in dermatology: Revisited
Department of Dermatology, Sri Ramachandra University, Chennai, Tamil Nadu, India
Reference: Indian Dermatol Online J [serial online] 2015 [cited 2015 Nov 24];6:428-35.
Aspirin has been one of the oldest drugs in the field of medicine, with a wide range of applications. In dermatology, aspirin has shown benefit in a variety of disorders. Recently, reduction of melanoma risk with aspirin has been demonstrated. Although an analgesic to begin with, aspirin has come a long way; after cardiology, it is now found to be useful even in dermatology.
Acquired neurocutaneous disorders.
1Department of Neurology, Hartford Hospital - University of Connecticut, Hartford CT, USA. 2Section of Dermatopathology, Department of Pathology, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA. 3Department of Neurology, Stritch School of Medicine, Loyola University Chicago, Maywood, IL, USA. Electronic address: firstname.lastname@example.org.
Reference: Handb Clin Neurol. 2015;132:29-73. doi: 10.1016/B978-0-444-62702-5.00003-2.
A variety of neurologic diseases have cutaneous manifestations. These may precede, coincide with, or follow the neurologic findings. An array of autoimmune, genetic, and environmental factors play a role in expression and severity of the neurologic burden in these conditions. This chapter emphasizes congenital and genetic disorders, but we also discuss the pathophysiology and manifestation of various acquired neurocutaneous disorders with an emphasis Behcet’s disease, dermatomyositis, Sjögren’s syndrome, systemic lupus erythematosus, scleroderma, Parry-Romberg syndrome and Degos disease.
Title: Anti-C5 antibodies having improved pharmacokinetics Document Type and Number: United States Patent 9079949
The disclosure provides antibodies that are useful for, among other things, inhibiting terminal complement (e.g., the assembly and/or activity of the C5b-9 TCC) and C5a anaphylatoxin-mediated inflammation and, thus, treating complement-associated disorders. The antibodies have a number of improved properties relative to eculizumab, including, e.g., increased serum half-life in a human.
Title: COMPOSITIONS COMPRISING A COMPLEMENT INHIBITOR AND AN INTERFERON ALPHA INHIBITOR Document Type and Number: United States Patent Application 20150174243 Kind Code: A1
The present disclosure is based, at least in part, on the discovery by the inventor that an inhibitor of complement, namely the humanized anti-C5 antibody eculizumab, was highly efficacious in the treatment of a patient afflicted with the systemic form of Degos’ disease.
Title: Methods for Treating Conditions Associated with MASP-2 Dependent Complement Activation Document Type and Number: United States Patent Application 20150166675 Kind Code: A1
Reference: Application Number: 14/517750 International Classes: C07K16/40; A61K39/395
In one aspect, the invention provides methods of inhibiting the effects of MASP-2-dependent complement activation in a living subject suffering from, or at risk for developing a thrombotic microangiopathy (TMA). The methods comprise the step of administering, to a subject in need thereof, an amount of a MASP-2 inhibitory agent effective to inhibit MASP-2-dependent complement activation.
Malign atrofik papüloz (Köhlmeier-Degos hastalığı)
Degos disease – malignant atrophic papulosis or cutaneointestinal lethal syndrome: rarity of the disease
Spaulding Rehabilitation Network Research Laboratory, Harvard Medical School, Boston, MA, USA; 2University of São Paulo, Sirio Libanes Hospital, São Paulo, Brazil; Sirio Libanes Hospital, São Paulo, Brazil
Reference: Journal of Clinical and Experimental Gastroenterology, April 2015 Volume 2015:8 Pages 141—147
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.
Title: Methods for treating multiorgan, systemic degos' disease with a complement inhibitor Document Type and Number: United States Patent 8999340
This is an application for the drug Eculizumab (Soliris), made by Alexion, to be patented for use in treating Degos disease.
The present disclosure relates to, inter alia, compositions containing an inhibitor of human complement and/or an inhibitor of interferon alpha, and the use of the compositions in methods for treating or preventing Degos’ disease in a subject. In some embodiments, the inhibitor is an antibody, or antigen-binding fragment thereof, that binds to a human complement component C5 protein or to a biologically-active fragment of C5 such as C5a or C5b.
Degos Disease Associated with Behçet's Disease
Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea.
Reference: Ann Dermatol. 2015 Apr;27(2):235-236. English.
Degos disease (DD) is a rare, thrombo-occlusive vasculopathy that primarily affect the skin, gastrointestinal tract, and central nervous system1. The cutaneous lesions present as characteristic papules with porcelain-white central atrophy and an erythematous raised border. Behçet’s disease (BD) is a systemic vasculitis characterized clinically by recurrent oral aphthous and genital ulcers, cutaneous lesions, and iridocyclitis/posterior uveitis2. DD can be found as an apparent idiopathic disease or as a surrogate clinical finding in some connective tissue diseases.
Degos disease: report of a case and review of the literature.
Dermatology Section, Department of Clinical Medicine and Immunological Sciences, University of Siena, Siena, Italy - .
Reference: G Ital Dermatol Venereol. 2015 Feb;150(1):123-129.
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. On this basis, we diagnosed malignant atrophic papulosis. Laboratory tests and instrumental investigation did not reveal any systemic involvement.
Skin in Rheumatic Disease
Reference: Clinical Basis of Rheumatic Disease
Skin is involved frequently in rheumatic diseases.
Skin manifestations can be hallmark features in some rheumatic
diseases, especially lupus erythematosus, dermatomyositis, and systemic
Skin changes in rheumatic diseases are specific when characterized by a
distinct clinical and histopathologic picture.
Nonspecific skin changes occur in a variety of diseases but not
exclusively in a distinct rheumatic disorder.
Skin involvement can alert the clinician to a different course, therapeutic
approach, and prognostic outcome in a patient with rheumatic disease.
Challenging Mimickers of Primary Systemic Vasculitis.
1Rheumatology Unit, Massachusetts General Hospital, Yawkey 2, 55 Fruit Street, Boston, MA 02114, USA.
Reference: Rheum Dis Clin North Am. 2015;41(1):141-160. doi: 10.1016/j.rdc.2014.09.011
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.
Non-atherosclerotic Causes of Mesenteric Arterial Disease
4. Division of Vascular and Endovascular Surgery, Mayo Clinic, Rochester, MN, USA
Reference: Mesenteric Vascular Disease 2015, pp 123-144
The most common cause of occlusive mesenteric artery disease is ostial atherosclerosis. Non-atherosclerotic causes account for 5–10 % of all cases of chronic mesenteric ischemia (CMI). The vasculitides consist in a varied group of conditions characterized by an inflammatory response of vessel wall, with or without associated necrosis and granulomas, affecting 20 individuals per million a year. These diseases have different etiologies and pathogenic mechanisms, albeit most of them are not completely understood.
Renal involvement in Malignant Atrophic Papulosis (Degos Disease)
Nephrology-Dialysis Department, Civico and Di Cristina Hospital – Palermo, Italy.
Reference: An Bras Dermatol. 2015 Mar-Apr; 90(2): 285.
Anais Brasileiros de Dermatologia
Sociedade Brasileira de Dermatologia
We read with interest the article of Dr. Lima on Malignant Atrophic Papulosis (MAP) or Degos Disease (DD) 1. This rare disease is an occlusive vasculopathy characterized by skin lesions and systemic involvement. DD is a potentially life-threatening disease, mainly the involvement of inner organs (bowel perforation and peritonitis, massive cerebral haemorrhage, thrombosis of the cerebral arteries, encephalitis, meningitis).
Malignant Atrophic Papulosis Is Challenging to Diagnose, Treat
Steffens Scleroderma Center in Albany and Saratoga Springs, N.Y and Albany College of Pharmacy and Health Sciences in Albany, N.Y
Reference: The Rheumatologist - An official publication of the ACR and the ARHP serving rheumatologists and rheumatology health professionals
In December 2009, we encountered a 17-year-old male who was dying from a recently diagnosed catastrophic disease. He was previously healthy and athletic. He was a patient in the intensive care unit (ICU) with decompensated vital signs, following an exploratory laparotomy for polybacterial peritonitis and sepsis from gastrointestinal (GI) microperforations. He was deteriorating despite treatment with immune intravenous globulin (IVIG) and broad-spectrum antibiotics.
Degos Disease A C5b-9/Interferon-α–Mediated Endotheliopathy Syndrome
Division of Dermatopathology, Department of Pathology and Laboratory Medicine, Weill Cornell College of Cornell University, New York, NY; Department of Immunology, Duke University Medical Center, Durham, NC; New York University, New York; Department of Rheumatology, Albany Medical College, Albany, NY; Pathology, Ohio State University, Columbus; Division of Rheumatology, Hospital for Special Surgery, New York, NY; and Genetic Medicine, University of Manchester, Manchester Academic Health Science Centre, Central Manchester Foundation Trust University Hospitals, Manchester, England
Reference: American Society for Clinical Pathology
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. All had characteristic skin lesions with gastrointestinal involvement; other affected organs included brain in one and pericardium and pleura in another.
Case 18-2014 — A 32-Year-Old Man with a Rash, Myalgia, and Weakness
The Massachusetts General Hospital, Boston
Reference: N Engl J Med 2014; 370:2327-2337June 12, 2014DOI: 10.1056/NEJMcpc1304161
A 32-year-old man was admitted to this hospital because of a violaceous eruption that was followed by muscle pain and weakness, leading to respiratory failure. New papules with white centers developed. A diagnostic procedure was performed.
This case was presented at Medical Grand Rounds.
Severe Neurologic Involvement of Degos Disease in a Pediatric Patient
Reference: J Child Neurol April 2014 vol. 29 no. 4 550-554
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. Brain magnetic resonance imaging showed chronic subdural effusion and encephalomalacia of the left cerebral hemisphere. Cerebrospinal fluid (CSF) examination revealed increased protein levels.
Degos Disease: an update
Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease): systemic involvement determines the prognosis.
Reference: Br J Dermatol. 2014 Jan;170(1):110-5. doi: 10.1111/bjd.12642.
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.
Degos Hastalığı (Malign Atrofik Papülozis)
Romatoloji BD, İstanbul Üniversitesi İstanbul Tıp Fakültesi, İstanbul
Reference: Turkiye Klinikleri J Rheumatol-Special Topics 2014;7(1):33-7 Makale Dili: TR Ücretsiz Erişim
Malign atrofik papülozis (MAP), diğer adıyla Degos hastalığı, patogenezi aydınlatılamamış, küçük ve orta boy damarların oklüzyonuna bağlı, deri, gastrointestinal sistem, nörolojik sistem ve birçok organda multipl infarktlarla seyreden, nadir görülen, kronik, ilerleyici bir hastalıktır. MAP tüm yaş gruplarını ve her iki cinsiyeti etkileyebilir. MAP, merkezinde porselen beyazlığında, atrofik skar dokusu, çevresinde telenjiektazili eritem alanı bulunan papüler cilt lezyonları ile karakterizedir.
The effects of Eculizumab on the pathology of malignant atrophic papulosis
Cynthia M Magro,1 Xuan Wang,1 Francine Garrett-Bakelman,2 Jeffrey Laurence,2 Lee S Shapiro,3 and Maria T DeSancho2 1Department 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 2Division of Hematology/Medical Oncology, Department of Medicine, Weill Medical College of Cornell University, New York, New York 10065, USA 3The Center for Rheumatology, LLP, 1367 Washington Ave., Suite 101, Albany, NY 12206, USA
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).
Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review
1Departments of Dermatology, Venerology, Allergology and Immunology, Dessau Medical Center, Auenweg 38, Dessau 06847, Germany
Reference: Theodoridis et Al, Orphanet Journal of Rare Diseases 2013 8.10
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. The first manifestation of MAP usually occurs between the 20th and 50th year of life.
Clinical description: The cutaneous clinical picture is almost pathognomonic.
Effective treatment of malignant atrophic papulosis (Köhlmeier-Degos disease) with treprostinil – early experience
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.
Effective Treatment of Degos Disea with Treprostinil-Early Experience (abstract).
2nd Systemic Sclerosis World Congress Madrid, Spain
fatal case of Degos’ disease which presented with recurrent intestinal perforation
Reference: World J Gastrointest Surg. 2011 Oct 27; 3(10): 156–158. Published online 2011 Oct 27. doi: 10.4240/wjgs.v3.i10.156 PMCID: PMC3220729
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.
LETHAL SYSTEMIC DEGOS DISEASE WITH PROMINENT CARDIO-PULMONARY INVOLVEMENT
From the Department of Surgery, Sina Hospital, Tehran, Iran 1Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran 2Department of Pathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran
Reference: Indian J Dermatol. 2011 Sep-Oct; 56(5): 564–567. doi: 10.4103/0019-5154.87157 PMCID: PMC3221225
A 48-year-old man presented with acute abdominal pain underwent laparotomy that revealed two perforated ulcers in jejunum. He had skin lesions with porcelain white atrophic scar which were ignored for 3 years, whereas the disease revealed own malignant nature and progressed to nervous, gastrointestinal, and cardiopulmonary systems. The diagnosis of Degos disease was established on the basis of clinical and histopathological features. He expired due to cardio-pulmonary insufficiency after 5 months from the onset of systemic involvement.