Search Our Scientific Publications & Authors

Orphanet J Rare Dis 2022 04 20;17(1):172. Epub 2022 Apr 20.

Department of Surgery, Henry Ford Health System, Detroit, MI, USA.

Introduction: Kohlmeier-Degos (K-D) disease is a rare obliterative vasculopathy that can present as a benign cutaneous form or with potentially malignant systemic involvement. The gastrointestinal tract is most frequently involved in systemic disease and mortality is often related to bowel perforations. Herein, we provide information to providers and patients regarding gastrointestinal K-D symptomology, pathology, treatment, and diagnosis, with a focus on the importance of timely diagnostic laparoscopy. Read More

J Cardiothorac Surg 2022 Apr 1;17(1):59. Epub 2022 Apr 1.

Department of Cardiovascular Surgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami, Sagamihara, Kanagawa, 252-0374, Japan.

Background: Degos disease, also known as malignant atrophic papulosis, is characterised by cutaneous manifestations due to chronic thrombo-obliterative vasculopathy. There have been reports of the rare late-onset Degos disease complicated by constrictive pericarditis (CP). This study reports a case of CP caused by Degos disease that developed 20 years after diagnosis. Read More

Neurol India 2022 Jan-Feb;70(1):5-10

Albany Medical College, Albany, New York, USA.

Malignant atrophic papulosis (MAP), or systemic Degos disease, is an obliterative vasculopathy of unknown origin, characterized by erythematous papules found on the skin, central nervous system (Neuro-MAP) and gastrointestinal tract. Neurological involvement occurs in approximately 20% of systemic cases, is progressive and largely fatal. It can be described in two forms: 1) the parenchymal presenting with meningoencephalitis and meningomyelitis and 2) the neurovascular presenting with large cerebral infarcts, intracranial and subarachnoid hemorrhage, subdural hematoma and venous sinus thrombosis. Read More

Front Pediatr 2021 10;9:764797. Epub 2021 Dec 10.

Department of Dermatology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.

Malignant atrophic papulosis (MAP) is a life-threatening vasculopathy affecting the skin, gastrointestinal tract, central nervous system, pleural membrane, and pericardium. MAP carries a poor prognosis primarily because of its systemic involvement. It is extremely rare in children. Read More

Pediatr Dermatol 2022 Jan 21;39(1):112-114. Epub 2021 Dec 21.

Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA.

A 17-year-old Caucasian boy presented with progressive left-sided weakness, transient slurred speech, and skin lesions characterized by 3-5 mm, pink, asymptomatic papules with white atrophic centers on his central abdomen, back, and lower extremities. Skin biopsy confirmed the diagnosis of malignant atrophic papulosis, a rare vasculopathy that leads to the occlusion of small- and medium-sized arteries. He was treated with cyclophosphamide, eculizumab, treprostinil, pentoxifylline, heparin, and acetylsalicylic acid. Read More

Australas J Dermatol 2021 Nov 27;62(4):e586-e588. Epub 2021 Sep 27.

Department of Dermatology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.

Malignant atrophic papulosis (Degos disease) is a rare syndrome of multiple-system vascular diseases with unknown etiology. It can affect the skin, gastrointestinal tract and central nervous system. Here, we report a 58-year-old woman with extensive porcelain-white atrophic papules. Read More

Clin Exp Dermatol 2021 Dec 29;46(8):1653-1655. Epub 2021 Jun 29.

Department of Dermatology, University Hospitals of Derby and Burton NHS Foundation Trust, Derby, UK.

Am J Gastroenterol 2021 11;116(11):2164

Key Laboratory of the Ministry of Education for Experimental Teratology, Department of Histology and Embryology, School of Basic Medical Sciences, Cheeloo College of Medicine, Shandong University, Jinan, Shandong, PRC, China.

J Eur Acad Dermatol Venereol 2021 Oct 8;35(10):e672-e674. Epub 2021 Jun 8.

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

Orphanet J Rare Dis 2021 05 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 Aug 16;35(8):1655-1669. Epub 2021 May 16.

Faculty of Medicine, University of Toronto, Toronto, ON, 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

Am J Med Genet A 2021 05 3;185(5):1619-1623. Epub 2021 Mar 3.

Section of Pediatric Dermatology, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.

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

Lupus 2021 Apr 14;30(4):541-548. Epub 2021 Feb 14.

Servicio de Dermatología at Hospital Universitario "Dr. Jose Eleuterio Gonzalez" Universidad Autonoma de Nuevo León, Mexico, México.

Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS. 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 (Engl Ed) 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

Rheumatol Int 2020 Nov 3;40(11):1883-1890. Epub 2020 Jan 3.

Department of Pediatric Rheumatology, Children's Hospital of The King's Daughters, Eastern Virginia Medical School, 601 Children's Lane, Norfolk, VA, 23507, USA.

Dermatomyositis (DM) is a multi-system disease that results in chronic inflammation principally of the skin and striated muscle. Small blood vessel injury in the GI tract has been described in dermatomyositis, manifesting as bleeding, ulceration, pneumatosis intestinalis, and ultimately perforation. Recent histopathological studies have shown deposits in the capillaries of the skin, gastrointestinal tract, and brain of patients with dermatomyositis similar to that found in patients with Degos disease, suggesting these disease processes are closely related or represent varying degrees of severity on the same pathologic spectrum. 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.

Ital J Dermatol Venerol 2021 Dec 12;156(Suppl. 1 to No. 6):71-72. Epub 2019 Jun 12.

Unit of Dermatology, Second University of Naples, Naples, Italy.