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Pediatr Allergy Immunol 2019 Jan 27. Epub 2019 Jan 27.

Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran, Iran.

Ataxia-telangiectasia (A-T) is an autosomal recessive primary immunodeficiency (PID) disease that is caused by mutations in ataxia-telangiectasia mutated (ATM) gene encoding a serine/threonine protein kinase. A-T patients represent a broad range of clinical manifestations including progressive cerebellar ataxia, oculocutaneous telangiectasia, variable immunodeficiency, radiosensitivity, susceptibility to malignancies, and increased metabolic diseases. This congenital disorder has phenotypic heterogeneity, and the severity of symptoms varies in different patients based on severity of mutations and disease progression. Read More

Chest 2019 Jan 7;155(1):178-193. Epub 2018 Sep 7.

Respiratory Institute, Cleveland Clinic, Cleveland, OH.

Lung transplant is now an established modality for a broad spectrum of end-stage pulmonary diseases. According to the International Society for Heart and Lung Transplantation Registry, more than 50,000 lung transplants have been performed worldwide, with nearly 11,000 recipients of lung transplants alive in the United States. With the increasing use of lung transplant, pulmonologists must be cognizant of the common as well as the unique posttransplant dermatologic complications. Read More

J Cutan Pathol 2018 Nov 19;45(11):817-823. Epub 2018 Aug 19.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota.

Background: Graft-versus-host disease (GVHD) following solid organ transplantation (SOT) is extremely rare and infrequently described in the dermatologic literature.

Methods: We performed a retrospective clinicopathologic review of our institution's experience with patients diagnosed with SOT-associated GVHD (SOT GVHD) (May 1, 1996 to September 1, 2017).

Results: Of nine patients with SOT GVHD, seven had undergone liver transplantation, while two had undergone lung transplantation. Read More

Eur J Dermatol 2018 Jun;28(3):364-369

Friedrich-Alexander-Universität of Erlangen-Nürnberg, Universitätsklinikum Erlangen, Department of Dermatology, Ulmenweg 18, D-91054 Erlangen, Germany.

Background: Calcitonin gene-related peptide (CGRP) is a vasodilatory neuropeptide that plays an important role in the blood vessels of heart and peripheral circulation, a lack of which may cause vasculopathies.

Objective: In this study, the clinical course of disease, as well as the efficacy, side effects, and patient satisfaction of systemic calcitonin therapy in patients with systemic sclerosis (SSc), was evaluated.

Methods: Forty-nine patients received repetitive intravenous calcitonin infusions as first-line treatment. Read More

Forensic Sci Med Pathol 2018 09 20;14(3):377-380. Epub 2018 Jun 20.

Department of Clinical and Experimental Medicine, Foggia University School of Medicine, Viale L. Pinto, 1, 71121, Foggia, Italy.

Type 1 neurofibromatosis (NF 1), a rare genetic disease with autosomal dominant transmission, has typical dermatologic manifestations with pathognomonic Lisch nodules, and is rarely known for vascular alterations. Among these, aneurysmal dilatation is the most common form. We report a fatal case of massive hemothorax due to a spontaneous rupture of the left pulmonary artery branch micro-aneurysm in a NF 1 patient. Read More

Arthritis Care Res (Hoboken) 2018 Dec 8;70(12):1806-1813. Epub 2018 Nov 8.

University of Washington, Seattle.

Objective: To investigate clinical manifestations of juvenile systemic sclerosis (SSc; scleroderma), including disease characteristics and patient quality of life, using the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry.

Methods: Patients with juvenile SSc were prospectively enrolled between 2010 and 2013. The diagnosis of juvenile SSc was determined by the enrolling pediatric rheumatologist, with the requirement for disease onset prior to age 18 years. Read More

J Am Acad Dermatol 2018 Jun 27;78(6):1097-1101.e1. Epub 2017 Dec 27.

Department of Dermatology, Mayo Clinic, Rochester, Minnesota; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota. Electronic address:

Background: Graft-versus-host-disease (GVHD) after solid organ transplantation (SOT) is extremely rare.

Objective: To investigate the dermatologic manifestations and clinical outcomes of SOT GVHD.

Methods: Systematic literature review of SOT GVHD. Read More

Hematology Am Soc Hematol Educ Program 2017 12;2017(1):242-250

Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Bethesda, MD.

The clinical manifestations of inherited susceptibility to leukemia encompass a wide phenotypic range, including patients with certain congenital anomalies or early-onset myelodysplastic syndrome (MDS) and some with no obvious medical problems until they develop leukemia. Leukemia susceptibility syndromes occur as a result of autosomal dominant, autosomal recessive, or X-linked recessive inheritance, or de novo occurrence, of germline pathogenic variants in DNA repair, ribosome biogenesis, telomere biology, hematopoietic transcription factors, tumor suppressors, and other critical cellular processes. Children and adults with cytopenias, MDS, dysmorphic features, notable infectious histories, immunodeficiency, certain dermatologic findings, lymphedema, unusual sensitivity to radiation or chemotherapy, or acute leukemia with a family history of early-onset cancer, pulmonary fibrosis, or alveolar proteinosis should be thoroughly evaluated for a leukemia susceptibility syndrome. Read More

Clin Imaging 2018 May - Jun;49:17-36. Epub 2017 Oct 31.

Department of Radiology, Division of Pediatric Radiology, Montefiore Medical Center, Bronx, NY, United States. Electronic address:

Purpose: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs.

Methods: We review the imaging findings in childhood diseases associated with dermatologic manifestations. Read More

Radiographics 2017 Jul-Aug;37(4):1135-1160. Epub 2017 May 26.

From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.).

Inflammatory bowel disease (IBD) is a chronic, relapsing immune-mediated inflammation of the gastrointestinal tract. IBD includes two major disease entities: Crohn disease and ulcerative colitis. Imaging plays an important role in the diagnosis and surveillance of these complex disorders. Read More

J Immunother 2017 Mar 6. Epub 2017 Mar 6.

Departments of *Pharmacology †Pain and Palliative Care ‡Rheumatology ∥Pneumology, CHU of Caen §Department of General Medicine, Medical School, Normandie University, Caen, France.

The anti-programmed cell-death-1 antibody, nivolumab, has been recently approved for the treatment of advanced non-small cell lung cancer. Although, today, immune-related adverse effects such as dermatologic, digestive, hepatic, and endocrine toxicities are well-known with immune checkpoint inhibitors, rheumatic diseases are less well described. Herein, we report the case of a patient without a history of arthritis who developed polymyalgia rheumatica after 13 cycles of nivolumab used for the treatment of advanced non-small cell lung cancer. Read More

Medwave 2016 Nov 15;16(10):e6609. Epub 2016 Nov 15.

Escuela de Medicina, Universidad de Valparaíso, Viña del Mar, Valparaíso, Chile; Servicio de Cirugía, Hospital Dr. Eduardo Pereira, Valparaíso, Chile.

Dermatomyositis is an autoimmune inflammatory myopathy associated with dermatologic findings. Its association with neoplasms is widely reported and has been shown to be higher in lung cancer, particularly small-cell carcinoma, probably as it originates from neuroendocrine cell precursors, as well as breast and ovarian cancer. The low frequency of dermatomyositis makes it difficult to suspect the underlying lesions among the diagnostic possibilities of systemic diseases with cutaneous manifestations. 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

Am J Med Sci 2016 May 2;351(5):544-5. Epub 2016 Mar 2.

Department of Internal Medicine, University of Arizona, Tucson, Arizona. Electronic address:

Respir Res 2016 Feb 29;17:22. Epub 2016 Feb 29.

Department of Medicine, University of Calgary, Calgary, AB, Canada.

Background: Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant condition characterized by dermatologic lesions, pulmonary manifestations, and renal tumors. The syndrome arises from germline mutations in the folliculin (FLCN) gene. We present findings from the single largest family BHD cohort described to date. Read More

BMC Med 2016 Feb 3;14:18. Epub 2016 Feb 3.

Department of Medicine, Division of Critical Care, McMaster University, 1200 Main St W, L8S 4L8, Hamilton, ON, Canada.

Background: Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease associated with high morbidity and mortality. Effective treatments for IPF are limited. Several recent studies have investigated novel therapeutic agents for IPF, but very few have addressed their comparative benefits and harms. 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

Respir Med 2015 Apr 14;109(4):443-50. Epub 2015 Jan 14.

Department of Respiratory Medicine and Allergology, Aarhus University Hospital, Denmark.

Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatologic disease that occasionally is accompanied by extracutaneous manifestations, amongst these is pulmonary involvement. The etiology is unknown. More than 50% of PG cases are associated with an underlying systemic disease such as inflammatory bowel disease, rheumatoid arthritis, hematological disorder or malignancy. Read More

Adv Parasitol 2014 ;84:27-149

Departamento de Parasitologia, Facultad de Farmacia, Universidad de Valencia, Valencia, Spain.

Fascioliasis is a food-borne parasitic disease caused by the trematode species Fasciola hepatica, distributed worldwide, and Fasciola gigantica, restricted to given regions of Africa and Asia. This disease in humans shows an increasing importance, which relies on its recent widespread emergence related to climate and global changes and also on its pathogenicity in the invasive, biliary, and advanced chronic phases in the human endemic areas, mainly of developing countries. In spite of the large neurological affection capacity of Fasciola, this important pathogenic aspect of the disease has been pronouncedly overlooked in the past decades and has not even appear within the numerous reviews on the parasitic diseases of the central nervous system. Read More

J Cell Biochem 2013 Dec;114(12):2673-6

School and Division of Allergy and Clinical Immunology, Department of Human Pathology, University Policlinic "G. Martino", via Consolare Valeria 1, Messina, 90125, Italy; Institute of Biomedicine and Molecular Immunology "A. Monroy" (IBIM)-Consiglio Nazionale delle Ricerche (CNR), via Ugo la Malfa 153, Palermo, 90146, Italy.

The dermatologic side effects are the most common adverse effects associated with Epidermal Growth Factor Receptor tyrosine kinase inhibitors. Although the mechanisms underlying the development of the skin toxicity remain unclear, immunological mechanisms are considered to be involved. A possible correlation between plasma levels of certain cytokines and development of skin toxicity has been reported. 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

Adv Clin Exp Med 2012 Jan-Feb;21(1):43-6

Department of Gastroenterology and Hepatology, Wroclaw Medical University, Poland.

Background: The etiopathogenesis of inflammatory bowel disease (IBD), encompassing Crohn's disease (CD) and ulcerative colitis (UC), is still not fully elucidated and seems to be multifactorial. It has been suggested that genetic, immunological and environmental factors participate in IBD development. IBD extraintestinal manifestations include rheumatic, metabolic, dermatologic, ophthalmologic, hepatobiliary, pancreatic, urologic, pulmonary, neurological, hematological and thromboembolic complications. 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

Dis Esophagus 2013 Feb-Mar;26(2):197-203. Epub 2012 May 16.

Section of Gastroenterology, Department of Medicine, Temple University School of Medicine, Philadelphia, Pennsylvania 19140, USA.

Scleroderma esophagus is characterized by ineffective peristalsis and reduced esophageal sphincter pressure. Esophageal disease in scleroderma can precede cutaneous manifestations and has been associated with Raynaud's phenomenon (RP) and pulmonary fibrosis (PF). The objective of the study is to evaluate the impact of cutaneous findings, RP, and PF on demographics, symptoms, and esophageal motility in patients with scleroderma. Read More

Pediatr Clin North Am 2012 Apr 6;59(2):381-405. Epub 2012 Apr 6.

Division of Rheumatology, Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, Pittsburgh, PA 15224, USA.

Pediatric scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a common pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of patients showing extracutaneous disease manifestations such as arthritis and uveitis. Read More

Am J Dermatopathol 2012 Apr;34(2):220-5

Department of Pathology, Wake Forest University School of Medicine, Winston Salem, NC, USA.

We present the case of a 17-year-old male kidney transplant recipient who presented initially with dermatologic symptoms and was found to have histologic changes in the skin that were consistent with mycosis fungoides. Shortly after this diagnosis was made, imaging studies demonstrated multifocal interstitial and airspace consolidation in both lungs. Physical examination revealed no lymphadenopathy or hepatosplenomegaly, but an open lung biopsy revealed an Epstein-Barr virus (EBV)-negative monomorphic T-cell posttransplant lymphoproliferative disorder (PTLD) with a concomitant EBV-positive B-cell PTLD involving the same lesion of the lung. Read More

Gastroenterol Hepatol (N Y) 2011 Apr;7(4):235-41

Dr. Levine is an Associate Physician in the Division of Gastroenterology, Hepatology, and Endoscopy at Brigham and Women's Hospital and an Instructor of Medicine at Harvard Medical School, both in Boston, Massachusetts.

Extraintestinal manifestations of inflammatory bowel disease are prevalent in both ulcerative colitis and Crohn's disease. The most common manifestations involve the musculoskeletal and dermatologic systems. Other manifestations involve the hepatopan-creatobiliary system (eg, primary sclerosing cholangitis) as well as the ocular, renal, and pulmonary systems. 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

J Crohns Colitis 2010 Oct;4(4):390-7

Department of Gastroenterology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.

Inflammatory bowel disease (IBD) is associated with a number of extraintestinal manifestations that may involve most organ systems. Extraintestinal manifestations are more common in Crohn disease (CD) and may include rheumatologic, ocular, dermatologic, biliary and pulmonary manifestations. The most common pulmonary manifestations of IBD are drug-induced lung disease. Read More

Emerg Med Clin North Am 2010 May;28(2):393-407, Table of Contents

Positive Health Program at San Francisco General Hospital, University of California San Francisco, Building 80, Ward 84, 995 Potrero Avenue, San Francisco, CA 94110, USA.

Immune reconstitution inflammatory syndrome (IRIS) must be considered in the differential diagnosis for any patient infected with HIV who has begun ART in the preceding months. Distinguishing between manifestations of IRIS and active infection is of paramount importance and poses a diagnostic challenge to the provider in the acute care setting. Presentations of IRIS are often atypical for the precipitating pathogen, and novel presentations are likely. Read More

Dermatol Online J 2010 Apr 15;16(4). Epub 2010 Apr 15.

University of California Davis, Sacramento, California, USA.

An 81-year-old woman with a history of renal cell carcinoma and years of slowly, progressively enlarging pulmonary nodules of uncertain etiology presented with several weeks of painful lower extremity nodules. A biopsy revealed changes consistent with nodular vasculitis. A purified protein derivative and QuantiFERON test were positive, favoring the diagnosis of erythema induratum of Bazin. 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

Clin Breast Cancer 2010 Feb;10(1):E3-5

Johns Hopkins University and Sinai Hospital Program in Internal Medicine, Baltimore, MD, USA.

Prostate adenocarcinoma can manifest as a fairly indolent tumor or as a very aggressive cancer with significant invasive and metastatic potential. Common metastatic sites include bone, liver, lymph nodes, and adrenal glands. Dermatologic manifestations are rare. Read More

Arch Pathol Lab Med 2009 Dec;133(12):1943-8

Department of Pathology, James Homer Wright Pathology Laboratories, Massachusetts General Hospital and Harvard Medical School, Boston, USA.

Context: -Nephrogenic systemic fibrosis (NSF) is a rare but serious disorder initially described as a purely dermatologic process. Isolated autopsy reports have described multiorgan involvement by this disease.

Objective: -To further illustrate the varied and systemic involvement of NSF by describing the autopsy experience at the Massachusetts General Hospital. Read More

J Med Toxicol 2009 Dec;5(4):191-5

Department of Clinical Pharmacy, Faculty of Pharmacy, Shiraz University of Medical Science, Shiraz, Iran.

Introduction: Sulphur mustard (SM) is an alkylating chemical warfare agent that was widely used during the Iran-Iraq conflict(1980-1988). Delayed complications of SM in different organs were evaluated in this study.

Methods: This cross-sectional study was performed from March 2005 to June 2006. Read More

J Am Acad Dermatol 2009 Jun;60(6):897-925; quiz 926-8

Department of Dermatology at Federal University of the State of Rio de Janeiro, Rio de Janeiro, Brazil.

Unlabelled: Protozoan infections are very common among tropical countries and have an important impact on public health. Leishmaniasis is the most widely disseminated protozoan infection in the world, while the trypanosomiases are widespread in both Africa and South America. Amebiasis, a less common protozoal infection, is a cause of significant morbidity in some regions. 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

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 Sep 20;59(3):385-96. Epub 2008 Jun 20.

Department of Dermatology, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611-2997, USA.

Background: Morphea is an autoimmune inflammatory sclerosing disorder that may cause permanent functional disability and disfigurement.

Objectives: We sought to determine the clinical features of morphea in a large pediatric cohort.

Methods: We conducted a retrospective chart review of 136 pediatric patients with morphea from one center, 1989 to 2006. Read More

Pediatr Dermatol 2008 Mar-Apr;25(2):150-7

Division of Dermatology, University of Massachusetts Medical School, Worcester, Massachusetts, USA.

Cystic fibrosis is an autosomal recessive disease reported in 1 in 2500 live births in Northern American and Northern European Caucasian populations. Classic disease findings include chronic bacterial infection of airways and sinuses, malabsorption of fat, infertility in men, and elevated concentrations of chloride in sweat. Less well-recognized findings associated with cystic fibrosis include cutaneous findings, which can be primary or secondary manifestations of the disease process. Read More

J Clin Gastroenterol 2007 Mar;41(3):329-32

Departments of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.

Primary biliary cirrhosis (PBC) and systemic sarcoidosis are granulomatous diseases of unknown etiology whose hepatic manifestations may infrequently be imitative of one another. Described herein is the first reported case in the medical literature of systemic sarcoidosis developing after liver transplantation for PBC. The presented patient, who suffered from typical clinical, laboratory, and pathologic manifestations of PBC, developed decompensated liver cirrhosis within a course of 8 years, necessitating orthotopic liver transplantation. Read More

J Am Dent Assoc 2007 Mar;138(3):338-48; quiz 396, 398

Department of Oral and Maxillofacial Surgery and Diagnostic Sciences, University of Florida, College of Dentistry, Gainesville, FL 32610, USA.

Background: Hyperplastic granular gingivitis or "strawberry gingivitis" is a rare manifestation of Wegener's granulomatosis (WG), but it is nearly pathognomonic for this multisystem autoimmune vasculitis. The dentist may be the first health care professional to see patients with symptoms and findings of this condition. Early diagnosis and treatment is the most important factor in the management of this potentially fatal disease. Read More

J Am Acad Dermatol 2005 Dec;53(6):931-51, quiz 952-4

Department of Medical Clinics (Dermatology), Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

Unlabelled: Fungal infections are common in tropical countries and can have an important impact on public health. Lobomycosis is a common fungal infection in the tropical rain forest of South America, and paracoccidioidomycosis (South American blastomycosis) is a widespread and sometimes severe illness. Penicilliosis marneffei is an opportunistic infection of AIDS patients in southeast Asia. Read More

Skinmed 2005 Sep-Oct;4(5):320-2

Department of Dermatology, Dr. Manuel Gea González, General Hospital, Mexico City, Mexico.

Case 1: A 33-year-old man with a 14-year history of localized skin disease on the face and scalp was evaluated at the department of dermatology. The physical examination revealed plaques with papules, pustules, and a golden yellow crusting on the forehead, cheeks, upper lip, and chin (Figure 1). The scalp presented fine, whitish scales. Read More

Biol Blood Marrow Transplant 2005 Oct;11(10):773-80

Department of Pediatrics, Children's Memorial Hospital, Chicago, Illinois, USA.

Graft-versus-host disease (GVHD) is a major cause of morbidity and mortality after bone marrow transplantation. Well-documented manifestations of GVHD include dermatologic, gastrointestinal, hepatic, pulmonary, musculoskeletal, and hematologic manifestations and sicca syndrome. To date, the heart has only rarely been reported to be a target of GVHD. Read More