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Orphanet J Rare Dis 2021 May 6;16(1):203. Epub 2021 May 6.

Department of Dermatology, The Hospital for Sick Children, Toronto, ON, Canada.

Background: Degos disease is a rare vascular disorder with a cutaneous-limited form, benign atrophic papulosis (BAP), and a systemic variant, malignant atrophic papulosis (MAP). Despite the poor prognosis of MAP, no study has established features associated with systemic disease.

Objectives: The aims of this systematic review were to: (1) summarize clinical features and treatments implemented for patients with MAP and BAP (2) identify clinical and laboratory factors associated with the development of MAP, compared to BAP. Read More

J Eur Acad Dermatol Venereol 2021 Apr 29. Epub 2021 Apr 29.

Faculty of Medicine, University of Toronto, Canada.

Degos disease (atrophic papulosis) is a rare vasculopathy with cutaneous and systemic manifestations. Although potentially fatal, the characteristics of and treatments for Degos disease variants are not adequately described. We conducted a systematic review to summarize cutaneous and systemic presentations, treatments, and outcomes of malignant (MAP) and benign (BAP) variants of Degos disease. Read More

Cureus 2021 Jan 13;13(1):e12677. Epub 2021 Jan 13.

Pathology and Dermatology, University of Rochester School of Medicine and Dentistry, Rochester, USA.

Degos disease is a rare vasculopathy characterized by skin papules with central porcelain white atrophy and a surrounding telangiectatic rim. Etiology of this condition is unknown. There are benign and systemic forms of the disease, and the latter may lead to fatality. Read More

Proc (Bayl Univ Med Cent) 2020 Sep 14;34(1):111-113. Epub 2020 Sep 14.

Department of General Surgery, Baylor Scott and White Medical Center - Round Rock, Texas.

We present the case of a 57-year-old man with known cutaneous manifestations of malignant atrophic papulosis, also known as Köhlmeier-Degos disease, who developed an almost-fatal small bowel perforation following a parathyroidectomy. He required two surgical interventions during his initial hospitalization and was started on eculizumab. Despite these therapies, the patient developed recurrent bowel perforations and ultimately died. Read More

J Fam Pract 2020 12;69(10):E11-E13

Department of Family Medicine and Community Health, University of Wisconsin School of Medicine and Public Health, Madison, USA.

A diagnosis was arrived at by doing something that the patient's other doctors hadn't: perform a biopsy. Read More

J Dermatol 2021 Apr 11;48(4):533-536. Epub 2020 Dec 11.

Division of Dermatology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

Cytomegalovirus causes a myriad of clinical features, potentially affecting any organ system, significantly increasing morbidity and even mortality. Vascular endothelial cell infection by cytomegalovirus has been implicated in the development of vasculopathy, possibly accounting for the clinical association between cytomegalovirus and vascular thrombosis. In contrast with visceral organ involvement, the cutaneous manifestations of cytomegalovirus are variable and rarely described. Read More

J Med Case Rep 2020 Oct 29;14(1):204. Epub 2020 Oct 29.

Department of Pathology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India.

Background: Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. Read More

Actas Dermosifiliogr 2021 Feb 16;112(2):103-117. Epub 2020 Oct 16.

Servicio de Anatomía Patológica, Hospital Universitario de Cruces, Barakaldo, Vizcaya, España.

Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in partI of this review. In this second part, we look at additional causes of vascular occlusion. Read More

Ann Diagn Pathol 2020 Aug 30;47:151545. Epub 2020 May 30.

Baylor College of Medicine, Texas Children's Hospital, Houston, TX, United States of America.

Malignant atrophic papulosis (Degos disease) is an unusual thrombotic microangiopathy of uncertain etiology. The disease characteristically involves the skin and internal organs, with nervous system involvement more common in children. We present a case with diverse neurological manifestations including cranial nerve palsies, gait instability, and urinary incontinence. Read More

Clin Dermatol 2019 Sep - Oct;37(5):561-579. Epub 2019 Jul 17.

Department of Dermatology, Temple University Lewis Katz School of Medicine, Philadelphia, Pennsylvania, USA.

White diseases are a heterogenous group characterized by hypopigmentation or depigmentation. Skin and eye color are determined by the number and size of melanosomes present. Melanin is produced by melanosomes in the melanocytes present within the epidermis of the skin, uvea, and retinal pigmented epithelium (RPE). Read More

JAMA Dermatol 2020 02;156(2):218-220

Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico-Milano, Milano, Italy.

BMJ Case Rep 2019 Dec 8;12(12). Epub 2019 Dec 8.

Pulmonary and Critical Care Medicine, Albany Medical Center Hospital, Albany, New York, USA.

This case demonstrates chronic fibrosing pleuritis, as a rare pulmonary aetiology for mortality in patients with Degos disease or malignant atrophic papulosis (MAP). Knowledge of this unusual complication will help physicians identify this entity early and provide appropriate treatment.Patients with MAP die from gastrointestinal and brain involvement within 2-3 years of diagnosis. Read More

JAMA Dermatol 2020 02;156(2):204

Department of Dermatology, University Hospital of Besançon, Besançon, France.

Ann Diagn Pathol 2020 Feb 23;44:151414. Epub 2019 Oct 23.

Weill Cornell Medicine, New York, United States of America.

The small guanosine triphosphatase Rho and its target Rho kinase are involved in a heterogeneous spectrum of cellular activities, many of which are integral to cytoskeletal organization. Furthermore, the Rho kinases result in NF kappa beta activation and hence the induction of various pro-inflammatory cytokines including TNF-alpha, IL-1B and IL-6. ROCK2 is a downstream protein, whose expression is indicative of Rho Kinase activation. Read More

J Dermatol 2020 Jan 20;47(1):e22-e23. Epub 2019 Oct 20.

Department of Internal Medicine, Besancon University Hospital, Besancon, France.

Australas J Dermatol 2020 Feb 20;61(1):e114-e116. Epub 2019 Aug 20.

Department of Dermatology, Cleveland Clinic, Cleveland, Ohio, USA.

Neurol Sci 2019 Oct 10;40(10):2201-2203. Epub 2019 Jun 10.

Department of Advanced Medical and Surgical Sciences, University of Campania "Luigi Vanvitelli", Caserta, Italy.

G Ital Dermatol Venereol 2020 Apr 29;155(2):237-239. Epub 2018 Oct 29.

Dermatology Unit, Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Bologna, Italy.

Hautarzt 2018 Oct;69(Suppl 1):34-36

Klinik und Poliklinik für Dermatologie und Allergologie, Ludwig-Maximilians-Universität München, Frauenlobstr. 9-11, 80337, München, Deutschland.

J Echocardiogr 2018 12 4;16(4):192-193. Epub 2018 Jul 4.

Division of Cardiology, Department of Medicine, Albany Medical College, Albany, NY, 12208, USA.

Pediatrics 2018 04;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 Jun 8;45(6):723-726. 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

Pathology 2017 Dec 31;49(7):798-801. Epub 2017 Oct 31.

Department of Nephrology, Monash Health, Melbourne, Australia; Centre for Inflammatory Diseases, Department of Medicine, Monash University, Clayton, Vic, Australia.

Gastroenterology 2018 06 1;154(8):e1-e2. Epub 2017 Sep 1.

Department of Nuclear Medicine, Changhai Hospital, Second Military Medical University, Shanghai, China.

Am J Dermatopathol 2017 Sep;39(9):e137

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

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

JAMA Dermatol 2017 11;153(11):1183-1184

Dermatology Service, Guillermo Grant Benavente Clinical Hospital, Concepción, Chile.

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