Dermatol Clin 2017 Jan;35(1):21-38

Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Building 10, Room 6D-47B, 10 Center Drive, Bethesda, MD 20892, USA. Electronic address:

Autoinflammatory disorders are sterile inflammatory conditions characterized by episodes of early-onset fever, rash, and disease-specific patterns of organ inflammation. Gain-of-function mutations in innate danger-sensing pathways, including the inflammasomes and the nucleic acid sensing pathways, play critical roles in the pathogenesis of IL-1 and Type-I IFN-mediated disorders and point to an important role of excessive proinflammatory cytokine signaling, including interleukin (IL)-1b , Type-I interferons, IL-18, TNF and others in causing the organ specific immune dysregulation. The article discusses the concept of targeting proinflammatory cytokines and their signaling pathways with cytokine blocking treatments that have been life changing for some patients. Read More

Actas Dermosifiliogr 2016 Nov 20;107(9):e65-e69. Epub 2016 May 20.

Servicio de Dermatología, Hospital General Universitario de Valencia, Valencia, España; Universidad de Valencia, Valencia, España.

Dermatologic manifestations of leukemia can be both specific and nonspecific (e.g., opportunistic infections, purpura and ecchymosis, Sweet syndrome). Read More

Ther Apher Dial 2016 Oct 16;20(5):433-452. Epub 2016 Sep 16.

Inselklinik Heringsdorf GmbH, 17424, Seeheilbad Heringsdorf, Germany.

The process of curing a patient by removing his illness by extracting blood is a very old one. Many years ago, phlebotomy was practiced to cure illness. Now, this old process, placed on a rational basis with therapeutic apheresis (TA), is being followed in clinical practice. Read More

Semin Cutan Med Surg 2016 Sep;35(3):147-52

Department of Pediatrics, Division of Dermatology, Seattle Children's Hospital/University of Washington School of Medicine, Seattle, Washington, USA.

Kasposiform hemoangioendothelioma (KHE) and tufted angioma (TA) are classifed as vascular tumors with locally aggressive and benign growth potential, respectively, within the classification schema proposed by the International Society for the Study of Vascular Anomalies. A unique feature of these vascular tumors is the risk of Kasabach-Merritt phenomenon (KMP), a severe thrombocytopenia with mild to moderate coagulopathy resulting from intralesional platelet trapping. As with many vascular anomalies, accurate description of clinical course, responses to therapy, and long-term outcomes have been hindered by lesion misidentification, imprecise nomenclature, and lack of prospective, randomized clinical trials to assess therapeutic efficacy. Read More

J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.

Faculté de Médecine, Université de Strasbourg et Clinique Dermatologique, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.

Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided. Read More

Am J Clin Dermatol 2016 Oct;17(5):509-518

Department of Dermatology, University of California San Diego, 10991 Twinleaf Court, San Diego, CA, 92131, USA.

Amyopathic dermatomyositis is a rare, idiopathic, connective tissue disease that presents with dermatologic lesions of classic dermatomyositis but lacks the myopathy of this disease. Cutaneous manifestations may include Gottron's sign, heliotrope rash, and characteristic patterns of poikiloderma. There is a substantial risk for developing interstitial lung disease or malignancy in patients with amyopathic dermatomyositis. Read More

Semin Oncol 2016 Jun 23;43(3):395-400. Epub 2016 Feb 23.

Plasma Cell Disorders Program, Department of Hematologic Oncology and Blood Disorders, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC. Electronic address:

Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a first clue of an underlying neoplasm in the setting of clinical suspicion. In some instances skin lesions result from aggregation of misfolded monoclonal immunoglobulins or their fragments, as in light chain-related systemic amyloidosis. On other occasions the cutaneous lesions result from deposits of malignant plasma cells or monoclonal proteins. Read More

Br J Dermatol 2016 Apr;174(4):e21

Bascom Palmer Eye Institute, Anne Bates Leach Eye Hospital, University of Miami Miller School of Medicine, Miami, FL, 33136, U.S.A.

Clin Lymphoma Myeloma Leuk 2015 Dec 3;15(12):739-47. Epub 2015 Sep 3.

Di. S. Sal. Section of Dermatology, IRCCS Azienda Ospedaliera Universitaria, San Martino-IST, Genoa, Italy.

Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by an increase in blood platelets and dominated by a predisposition to vascular events. Cutaneous manifestations can complicate its course. itching has been the most common symptom reported; however, the percentage has ranged from 3% to 46%, depending on the survey. Read More

Iran J Kidney Dis 2015 Sep;9(5):339-53

Division of General Internal Medicine, Toronto General Hospital, University of Toronto, Toronto, Canada.

End-stage renal disease (ESRD) is a rapidly growing global health problem within the past decades due to increased life expectancy, diabetes mellitus, hypertension, and vascular diseases. Since ESRD is not curable definitively, patients suffering from ESRD have a very low quality of life; therefore, symptomatic management is the cornerstone of medical treatment. Uremia affects almost all body organs, such as skin, through different mechanisms including biochemical, vascular, neurologic, immunologic, hematologic, endocrine, and electrolyte and volume balance disturbances. Read More

Turk J Haematol 2015 Jun;32(2):168-71

University of Messina Faculty of Medicine, Department of General Surgery and Oncology, Division of Hematology, Messina, Italy Phone: 0039 090 221 23 64 E-mail:

Imatinib mesylate is a small-molecule tyrosine kinase inhibitor (TKi) designed to target c-ABL and BCR-ABL, approved for the treatment of chronic myeloid leukemia and gastrointestinal stromal tumors. Adverse cutaneous reactions induced by imatinib are frequent, generally moderate, and dose-dependent. The aim of this work was to investigate the possible contribution of interleukin (IL)-33 and IL-31, cytokines involved in disorders associated with itching, in the pathogenesis of pruritus in a patient undergoing imatinib mesylate treatment. Read More

Immunol Allergy Clin North Am 2015 Aug 17;35(3):403-11. Epub 2015 Jun 17.

Division of Allergy and Inflammation, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA; Division of Infectious Diseases, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston, MA 02215, USA.

Eosinophil-associated disorders can affect practically all tissues and organs in the body, either individually or in combination. This article provides an overview of end-organ manifestations of eosinophilia and discusses selected organ systems, including the upper and lower respiratory, cardiovascular, gastrointestinal, nervous, dermatologic, and renal systems. Mechanisms by which eosinophilia leads to end-organ damage are also considered. Read More

Perm J 2015 ;19(3):77-8

Senior Emergency Physician at the Santa Clara Medical Center, and Clinical Assistant Professor of Emergency Medicine (Surgery) at Stanford University. He is the Medical Director for the Foothill College Paramedic Program in Los Altos, CA.

Leukocytoclastic vasculitis (LCV), also termed hypersensitivity vasculitis, is a small-vessel vasculitis. The skin is the organ most commonly involved in LCV. Typical presentation is a painful, burning rash predominantly in the lower extremities. Read More

Clin Toxicol (Phila) 2015 Jun 10;53(5):489-92. Epub 2015 Apr 10.

Department of Intensive Care, Cliniques St-Luc, Université catholique de Louvain , Brussels , Belgium.

Objective: While methemoglobinemia is a possible complication of chronic dapsone therapy or of acute overdose, serious adverse manifestations related to methemoglobin formation remain rare. We present an unusual case with severe ischemic retinal injury.

Case Report: A 30-year-old African woman presented with a sudden decrease of visual acuity secondary to retinal ischemia. Read More

J Am Acad Dermatol 2015 Jan;72(1):1-19; quiz 19-20

Cooper Medical School of Rowan University, Camden, New Jersey; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.

Epstein-Barr virus (EBV) is a ubiquitous virus that has been implicated in a wide range of human diseases, many of which have mucocutaneous manifestations. As a member of the herpesviridae family, EBV causes lifelong infection by establishing latency in B lymphocytes. An intact immune response is critical in preventing progression of EBV disease, and the clinical manifestations of infection are dependent on the intricate relationship between virus and host immune system. Read More

Ann Dermatol 2014 Dec 26;26(6):722-6. Epub 2014 Nov 26.

Department of Dermatology, Institute of Hair and Cosmetic Medicine, Yonsei University Wonju College of Medicine, Wonju, Korea.

Background: Alopecia areata (AA) is a common dermatologic condition with a broad spectrum of clinical features and age of onset, classically characterized by nonscarring patches of hair loss. In the past, early-onset (before adolescence) AA has been associated with various autoimmune diseases, especially atopic diseases and lupus erythematosus and demonstrates a worse prognosis compared with late onset AA.

Objective: To evaluate the differences in the comorbidity profile of AA with regard to age at onset. Read More

Medicine (Baltimore) 2014 Oct;93(17):255-66

Institut d'Immunologie, Réseau Eosinophile, EA2686 (GL, FL, DL, ML, JT, JEK), Service d'Immunologie Clinique et de Médecine Interne, Centre de Référence Maladies Auto-Immunes Systémiques (GL, DL, LT, NLG, PYH), Institut de Pathologie (MCC), Service de Dermatologie (DSS), Service des Maladies du Sang (FM), Institut de Génétique Médicale, U837 Inserm (CRL), Réseau Eosinophile, U995 Inserm (MC), CHRU de Lille, Université Lille Nord de France, Lille; Service de Dermatologie (MAA, CL), CHU d'Angers, L'UNAM Université, Angers; Service de Dermatologie et Dermatologie Pédiatrique, Centre de Référence pour les Maladies Rares de la Peau, U1035 (AT), CHU de Bordeaux, Bordeaux; Service d'Hématologie (GS) and Service de Radiologie, CREATIS, UMR5220 CNRS, U1044 Inserm (FC), Hôpital Sud, Service de Pneumologie, Centre de Référence pour les Maladies Pulmonaires Rares (CK), Hospices Civils de Lyon, Université Claude Bernard Lyon I, Lyon; Service d'Onco-Hématologie (HM), Centre Hospitalier de La Roche-Sur-Yon, La Roche-Sur-Yon; Service d'Onco-Hématologie (KG), Centre Hospitalier de Beauvais, Beauvais; Service de Médecine Interne (FA), Hôpital Bicêtre, APHP, Université Paris Sud XI, Le Kremlin-Bicêtre; Service d'Hématologie et Immunologie Pédiatrique (AB), Hôpital Robert Debré, APHP, Université Paris Diderot and Service de Pneumologie A, Centre de Compétence Maladies Pulmonaires Rares (RB), Hôpital Bichat, APHP, Paris; Service de Médecine Interne (CMH), Centre Hospitalier d'Annecy, Annecy; Service de Médecine Interne, Hôpital Foch, Suresne; EA4340, Biomarqueurs en Cancérologie et en Onco-Hématologie, Université Versailles-Saint-Quentin-en-Yvelines (JEK), France.

The CD3-CD4+ aberrant T-cell phenotype is the most described in the lymphoid variant of hypereosinophilic syndrome (L-HES), a rare form of HES. Only a few cases have been reported, and data for these patients are scarce. To describe characteristics and outcome of CD3-CD4+ L-HES patients, we conducted a national multicentric retrospective study in the French Eosinophil Network. Read More

Curr Allergy Asthma Rep 2014 Dec;14(12):480

Division of Allergy/Immunology/Pediatric Rheumatology, Department of Pediatrics, Women and Children's Hospital of Buffalo, SUNY at Buffalo School of Medicine and Biomedical Sciences, 219 Bryant Street, Buffalo, NY, 14222, USA.

The skin is the largest organ of our body; it consists of the epidermis, dermis, hair follicles, sweat glands, blood vessels, and connective tissue matrix. Its main function is to act as a barrier to the outside world and protect us from infections. Any component of the skin is subject to insults from the environment and/or from within the body. Read More

World J Gastroenterol 2014 Sep;20(34):11950-61

Frank Wong, Erin Rayner-Hartley, Michael F Byrne, Division of Gastroenterology, University of British Columbia, Vancouver, BC V5Z 1M9, Canada.

Helicobacter pylori (H. pylori) infection has been clearly linked to peptic ulcer disease and some gastrointestinal malignancies. Increasing evidence demonstrates possible associations to disease states in other organ systems, known as the extraintestinal manifestations of H. Read More

G Ital Dermatol Venereol 2014 Jun;149(3):291-300

Department of Clinical‑Surgical, Diagnostic and Pediatric Sciences Dermatology Section, University of Pavia Foundation IRCCS Policlinico San Matteo, Pavia, Italy -

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is characterized by an heterogeneous group of severe dermatologic manifestations and systemic involvement, due to several groups of medicaments. A series of 9 consecutive cases, observed from 2008 to 2013 in the Department of Dermatology, University of Pavia, is reported, all satisfying the clinical, hematological and systemic diagnostic criteria of DRESS. Clinically, 4 out of 9 patients had an urticarial and papular eruption, 2 an erythema-multiforme-like (EM-like) pattern, 2 erythroderma and 1 had an erythematous and macular reaction. Read More

J Am Acad Dermatol 2014 May;70(5):821.e1-19

Baylor University Medical Center, Dallas, Texas. Electronic address:

There are a multitude of diseases that commonly affect both the skin and the eye. Part II of this 2-part series reviews the oculocutaneous manifestations of neoplasms, both benign and malignant, and adverse drug reactions affecting the skin and the eye. Though rare, a number of neoplasms that primarily involve the skin, such as melanoma and basal cell carcinoma, can metastasize to the eye, leading to permanent damage if not properly treated. Read More

G Ital Dermatol Venereol 2013 Oct;148(5):453-63

Department of Medical Sciences Dermatologic Division, University of Turin, Turin, Italy -

Aim: Objective of the study was to determine the most common cutaneous lesions in patients with haematologic malignancies observed at dermatologic consultation and to identify the impact parameters related to the haematologic condition, like disease type/duration, remission, chemotherapy and transplantation, have on skin manifestations.

Methods: A total of 101 consecutive patients with onco-haematological malignancies referred for dermatological consultation over a two-year period were included in this prospective single-centre observational cohort study.

Results: The most common finding was infection (19. Read More

Int J Dermatol 2013 Nov 4;52(11):1349-56. Epub 2013 Apr 4.

Department of Dermatology, Mayo Clinic, RochesterDepartment of Laboratory Medicine and Pathology, Mayo Clinic, RochesterDivision of Hematology, Mayo Clinic, RochesterDivision of Clinical Biochemistry and Immunology, Mayo Clinic, RochesterDivision of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA.

Background: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome is a rare multisystem paraneoplastic condition associated with plasma cell dyscrasia.

Methods: From our institution's dysproteinemia database, 107 patients met criteria for polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome between January 1, 2000, and October 1, 2009. Medical records were reviewed for documented syndrome features at diagnosis. 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

J Clin Oncol 2012 Nov 8;30(33):4091-7. Epub 2012 Oct 8.

Department of Dermatology, University Hospital of Zurich, Gloriastrasse 31, 8091 Zurich, Switzerland.

Purpose: Mycosis fungoides (MF) is the most common primary cutaneous T-cell lymphoma. There is a need for multicenter trials involving defined patient populations using rigorous assessment criteria. We have investigated pegylated liposomal doxorubicin (PLD) in a clearly defined patient population with advanced MF. Read More

J Clin Pediatr Dent 2012 ;36(4):393-400

Department of Pedodontics, Istanbul University, Faculty of Dentistry, Istanbul, Turkey.

Objective: Kabuki syndrome is a rare autosomal dominant trait with variable clinical expression. Common dental manifestations of Kabuki syndrome are high-arched palate, micrognathia, malocclusion, microdontia, small dental arches, hypodontia, severe maxillary recession and mid-facial hypoplasia.

Study Design: This report includes the oral manifestations of a Turkish patient with Kabuki syndrome with consideration of the long-term craniofacial prognosis for this patient based on the physical, clinical and radiological findings in 9 years follow-up period. Read More

Int Wound J 2013 Oct 21;10(5):590-6. Epub 2012 Jun 21.

Division of Cardiology, Howard University Hospital, Washington, DC, USA Division of Rheumatology, Immunology and Allergy, Georgetown University Hospital, Washington, DC, USA Division of Rheumatology, Veterans Affairs Hospital, Washington, DC, USA Department of Internal Medicine, Howard University Hospital, Washington, DC, USA.

We present three cases describing the various skin manifestations of presumed levamisole-contaminated cocaine use. Antibody-mediated vasculitis and neutropenia were consistent findings in these cases and repeat exposure resulted in distinct dermatologic complications. This phenomenon of levamisole-induced vasculitis and neutropenia is being increasingly described and has characteristic wound manifestations that must be recognised and treated early. Read More

Arch Iran Med 2012 Jan;15(1):43-8

Department of Immunology, School of Public Health, Tehran University of Medical Sciences, Iran.

The skin could be a main target organ for extrahepatic manifestations in hepatitis C virus- (HCV) infected patients and research data suggest that interferon treatment may be associated with immune-mediated skin lesions. However, case reports propose that the response of dermatologic extrahepatic manifestations to interferon in patients with chronic HCV is greatly different. The objective of this study is to summarize currently available data on dermatologic conditions associated with chronic HCV infection. Read More

Pediatr Dermatol 2012 May-Jun;29(3):264-9. Epub 2011 Nov 2.

Department of Dermatology, Hospital Universitario Dr. Jose E. Gonzalez, Universidad Autonoma de Nuevo Leon, Monterrey, Mexico.

Chemotherapy used in the treatment of malignancies produces multiple mucocutaneous adverse reactions that may be clinically challenging. These mucocutaneous reactions are common and sometimes not diagnosed. The objective of this study was to determine the clinical patterns of the mucocutaneous manifestations during and after chemotherapy in children with a hematologic malignancy and to determine whether nutritional status influences the clinical presentation. Read More

Clin Dermatol 2011 Sep-Oct;29(5):531-40

Department of Dermatology, University of Athens, Andreas Sygros Hospital, 5th Ionos Dragoumi St, 16121 Athens, Greece.

The skin should not be considered as an isolated organ but rather as a definite functioning system that communicates with the internal environment. Skin signs of systemic diseases occur frequently and sometimes feature the first symptoms of an internal disease; furthermore, these manifestations may be the sole expressions of otherwise asymptomatic systemic disorders. A number of dermatologic signs, symptoms, and disorders can be invaluable as markers of systemic disease. Read More

Dermatol Online J 2011 May 15;17(5). Epub 2011 May 15.

University of Toronto, Sunnybrook Health Sciences Center, Department of Dentistry.

Dermatologic manifestations from therapy with imatinib are well known and frequently include hypopigmentation, and less commonly, hyperpigmentation. There have been few reports of oral hyperpigmentation. We present a case of palatal melanosis related to imatinib therapy for chronic myelogenous leukemia. Read More

Arch Dermatol 2011 Mar;147(3):331-5

Mayo Medical School, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Background: Granulomatous dermatitis has rarely been reported as a manifestation of leukemia or myelodysplastic syndrome (MDS). We describe a case of widespread granulomatous dermatitis that preceded the diagnosis of MDS by 2 years.

Observations: A 71-year-old man developed a generalized, mildly pruritic eruption that slowly progressed over a 2-year period. Read More

Hepat Mon 2010 1;10(4):258-69. Epub 2010 Dec 1.

Department of Internal Medicine, Minia University, Minia, Egypt.

Hepatitis C virus infection is an emerging disease and a public health problem in the world. There are accumulating data regarding extra hepatic manifestation of HCV, such as rheumatologic manifestations, endocrine, hematologic, dermatologic, renal, neurologic, and systemic manifestations. The therapy of them needs more attention to some exacerbations of extra hepatic manifestation and in some situation it needs different approaches. Read More

Asian Pac J Allergy Immunol 2009 Dec;27(4):233-6

Department of Dermatology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand.

Hyper-IgE syndrome (HIES) is a rare idiopathic primary immunodeficiency. It is characterized by a triad of findings, including high levels of serum IgE, recurrent skin abscesses and pneumonia and leads to pneumatocele formation. The diagnosis of HIES is complicated by a diversity of clinical and immunological spectrums and a heterogeneous set of genetic defects. Read More

Ann Med 2010 Mar;42(2):97-114

Department of Gastroenterology, Medical Section, Herlev Hospital, University of Copenhagen, Denmark.

Abstract Extraintestinal manifestations occur rather frequently in inflammatory bowel disease (IBD), e.g. ulcerative colitis (UC) and Crohn's disease (CD). Read More

G Ital Dermatol Venereol 2009 Dec;144(6):725-8

Dermatologic Clinic, University of Catania, University Hospital G. Rodolico Policlinic, Catania, Italy.

Syphilis is a sexually transmitted disease, caused by Treponema pallidum subspecies pallidum, its incidence, in the last decade, has significantly increased both in Western World and in developing countries. It represents a global health problem: it is estimated that each year the new cases of syphilis account for about 12 millions. The diagnosis is not always easy, especially in secondary syphilis in which the cutaneous manifestations are quite variable and should be considered in the differential diagnosis. Read More

Aust Fam Physician 2009 Jul;38(7):498-505

St Vincent's Hospital and Monash Medical Centre, Victoria.

Background: Dermatologic complaints are a common reason for presentation to a general practitioner. In some cases, one needs to determine if the complaint may be a manifestation of a more serious underlying systemic disease.

Objective: This article aims to highlight common dermatologic presentations where further assessment is needed to exclude an underlying systemic disease, to discuss classic cutaneous features of specific systemic diseases, and to outline rare cutaneous paraneoplastic syndromes. Read More

Hautarzt 2009 May;60(5):378-88

Universitätsklinik für Dermatologie, Paracelsus Medizinische Privatuniversität (PMU), Salzburg.

Hereditary epidermolysis bullosa (EB) is a term for a heterogeneous group of rare genetic disorders characterized by marked fragility of the skin and mucous membranes following minor trauma. Significant progress has been made in understanding the molecular basis of EB, which has far-reaching implications for an improved classification with consequences for prognosis, genetic counseling, DNA-based prenatal and preimplantation testing, and the development of future treatments including gene therapy. Besides mucocutaneous changes, EB leads to a number of systemic manifestations whose management requires multidisciplinary access. Read More

Hum Pathol 2009 Sep 22;40(9):1269-77. Epub 2009 Apr 22.

Department of Diagnostic Pathology, Kyoto University Hospital, Kyoto 606-8507, Japan.

Cutaneous plasmacytosis is a rare condition affecting middle-aged individuals, characterized by multiple red-brown papules and plaques over the trunk. It has been reported mainly in Japan. The condition is accompanied by polyclonal hypergammaglobulinemia and superficial lymphadenopathy. Read More

An Sist Sanit Navar 2008 ;31 Suppl 3:155-70

Departamento de oftalmología, Clínica Universitaria de Navarra, Pamplona 31008, Spain.

Systemic diseases affecting the cornea have a wide range of manifestations. The detailed study of all pathologies that cause corneal alteration is unapproachable, so we have centered our interest in the most prevalent or characteristic of them. In this paper we have divided these pathologies in sections to facilitate their study. Read More

J Cardiovasc Med (Hagerstown) 2009 Feb;10(2):188-91

Section of Cardiovascular Medicine, Yale University School of Medicine, New Haven, USA.

Idiopathic hypereosinophilic syndrome (IHES) is a rare systemic disease with multiple clinical presentations including cardiac, pulmonary and dermatologic manifestations. Little is known about the pathophysiology and potential triggers of this condition. Approximately 40-70% of these cases have cardiac involvement, which is the major cause of morbidity and mortality. Read More

Auris Nasus Larynx 2009 Oct 27;36(5):513-20. Epub 2008 Dec 27.

Department of Surgical Sciences, Maxillofacial Surgery Clinic, University of Udine, Italy.

Paraneoplastic syndromes (PNS) represent the clinical manifestation of the remote and indirect effects produced by tumor metabolites or other products. Paraneoplastic effects are not directly mediated by tumor invasion of normal tissue, or by the disruption of normal function of the involved organ, or by distant metastases. More than 260 cases of nasopharyngeal carcinoma (NPC) associated with PNS have been reported in the literature. Read More

Braz J Otorhinolaryngol 2008 May-Jun;74(3):452-7

Faculdade de Medicina de Marília, Brazil.

Hereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease is a rare fibrovascular dysplasia that makes vascular walls vulnerable to trauma and rupture, causing skin and mucosa bleeding. It is of dominant autosomal inheritance, characterized by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations and positive family history. Epistaxis is often the first and foremost manifestation. Read More

J Am Acad Dermatol 2008 Apr;58(4):703-6

Department of Dermatology, St Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.

Extramedullary hematopoiesis (EMH) is a relatively rare, but well-documented, manifestation of chronic myeloproliferative disorders. Microscopically, foci of EMH consist of erythroid and myeloid precursors intermixed with megakaryocytes. It typically occurs in the spleen and liver, but very occasionally manifests as cutaneous EMH. Read More

Actas Dermosifiliogr 2008 Jan;99 Suppl 1:18-28

Servicio de Dermatología, Hospital Universitario la Princesa, Madrid, España.

Psoriasis is a chronic disease that has a very negative impact on the quality of life of those suffering it. Therefore, treatments that make it possible to satisfactorily control the disease in the long term and maintain a good safety profile are needed. Efalizumab fulfills both requirements. Read More

Dermatol Clin 2008 Jan;26(1):31-43, vii

Department of Dermatology, Dermatopathology Division, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

The concept that noncutaneous malignancies may induce paraneoplastic inflammatory reactions and neoplastic or non-neoplastic proliferations in the skin is well known. Previous work on this subject primarily provides lists and descriptions of dermatologic entities that are exclusively or occasionally associated with specific or varied internal cancers or precancerous states. This review seeks to provide a different perspective to this subject by emphasizing components of the skin (the dermis and subcutis) as focal points of paraneoplastic phenomena, with the intent of broadening thinking and differential diagnoses when the findings described are encountered in dermatology clinics and dermatopathology laboratories. Read More

Immunol Allergy Clin North Am 2007 Aug;27(3):415-41

Department of Dermatology, 4B454 School of Medicine, University of Utah Health Sciences Center, University of Utah, 30 North 1900 East, Salt Lake City, UT 84132-2409, USA.

Skin is a commonly affected organ in hypereosinophilic syndromes (HES). Cutaneous lesions may be an important presenting sign, may be extremely debilitating, and often reflect disease activity in HES. Recognition of dermatologic manifestations is important in approaching diagnosis and treatment of HES. Read More

Clin Gastroenterol Hepatol 2007 Sep;5(9):1010-7; quiz 1005-6

Department of Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts.

The skin and the gastrointestinal tract may be affected concurrently by the same diseases. Pathogenetically, these conditions may be primarily dermatologic diseases involving the gastrointestinal (GI) tract or systemic diseases involving the skin, GI tract, and liver simultaneously. The correct diagnosis of such conditions relies on the ability of the gastroenterologist to recognize the underlying dermatologic disorder. Read More

Ann Otol Rhinol Laryngol 2007 Jul;116(7):502-13

Department of Surgical Sciences, ENT Clinic, University of Udine, Udine, Italy.

Paraneoplastic syndromes may be the first sign of a malignancy. They are systemic, nonmetastatic manifestations associated with a variety of malignant neoplasms and occurring in a minority of cancer patients. These associations of symptoms and signs are not directly related to the site or local manifestations of a malignant tumor or its metastases, but their recognition may facilitate the detection of malignancies or recurrences. Read More

Dermatol Surg 2007 Apr;33(4):403-9

Laser and Skin Surgery Center of Indiana, Carmel, Indiana 46032, USA.

Background: Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting an estimated 0.1% to 1% of the population. It is caused by a qualitative or quantitative defect of von Willebrand factor. Read More