Prospective Observational Study in a Tertiary
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3628-3641-Pruritus in Selected Dermatoses
Eur opean Rev iew for Med ical and Pharmacol ogical Sci ences 2016; 20: 3628-3641 Pruritus in selected dermatoses K. OLEK-HRAB 1, M. HRAB 2, J. SZYFTER-HARRIS 1, Z. ADAMSKI 1 1Department of Dermatology, University of Medical Sciences, Poznan, Poland 2Department of Urology, University of Medical Sciences, Poznan, Poland Abstract. – Pruritus is a natural defence mech - logical self-defence mechanism similar to other anism of the body and creates the scratch reflex skin sensations, such as touch, pain, vibration, as a defensive reaction to potentially dangerous cold or heat, enabling the protection of the skin environmental factors. Together with pain, pruritus from external factors. Pruritus is a frequent is a type of superficial sensory experience. Pruri - symptom associated with dermatoses and various tus is a symptom often experienced both in 1 healthy subjects and in those who have symptoms systemic diseases . Acute pruritus often develops of a disease. In dermatology, pruritus is a frequent simultaneously with urticarial symptoms or as an symptom associated with a number of dermatoses acute undesirable reaction to drugs. The treat - and is sometimes an auxiliary factor in the diag - ment of this form of pruritus is much easier. nostic process. Apart from histamine, the most The chronic pruritus that often develops in pa - popular pruritus mediators include tryptase, en - tients with cholestasis, kidney diseases or skin dothelins, substance P, bradykinin, prostaglandins diseases (e.g. atopic dermatitis) is often more dif - and acetylcholine. The group of atopic diseases is 2,3 characterized by the presence of very persistent ficult to treat . Persistent rubbing, scratching or pruritus. -
Extrafacial Granuloma Faciale
Journal of the American Osteopathic College of Dermatology Volume 11, Number 1 SPONSORS: ',/"!,0!4(/,/'9,!"/2!4/29s-%$)#)3 July 2008 34)%&%,,!"/2!4/2)%3s'!,$%2-! www.aocd.org Journal of the American Osteopathic College of Dermatology Journal of the American Osteopathic College of Dermatology 2007-2008 Officers President: Jay Gottlieb, DO President Elect: Donald Tillman, DO First Vice President: Marc Epstein, DO Second Vice President: Leslie Kramer, DO Third Vice President: Bradley Glick, DO Secretary-Treasurer: Jere Mammino, DO (2007-2010) Immediate Past President: Bill Way, DO Trustees: James Towry, DO (2006-2008) Mark Kuriata, DO (2007-2010) Karen Neubauer, DO (2006-2008) David Grice, DO (2007-2010) Sponsors: Global Pathology Laboratory Editors Stiefel Laboratories Jay S. Gottlieb, D.O., F.O.C.O.O. Medicis Stanley E. Skopit, D.O., F.A.O.C.D. James Q. Del Rosso, D.O., F.A.O.C.D. Galderma Editorial Review Board Ronald Miller, D.O. JAOCD Eugene Conte, D.O. Founding Sponsor Evangelos Poulos, M.D. Stephen Purcell, D.O. Darrel Rigel, M.D. !/#$s%)LLINOISs+IRKSVILLE -/ s&!8 Robert Schwarze, D.O. WWWAOCDORG Andrew Hanly, M.D. #/092)'(4!.$0%2-)33)/.WRITTENPERMISSIONMUSTBEOBTAINED Michael Scott, D.O. FROMTHE*OURNALOFTHE!MERICAN/STEOPATHIC#OLLEGEOF$ERMATOLOGY FORCOPYINGORREPRINTINGTEXTOFMORETHANHALFPAGE TABLESORlGURES Cindy Hoffman, D.O. 0ERMISSIONSARENORMALLYGRANTEDCONTINGENTUPONSIMILARPERMISSION Charles Hughes, D.O. FROMTHEAUTHORS INCLUSIONOFACKNOWLEDGEMENTOFTHEORIGINALSOURCE ANDAPAYMENTOFPERPAGE TABLEORlGUREOFREPRODUCEDMATERIAL Bill Way, D.O. 0ERMISSIONFEESAREWAIVEDFORAUTHORSWISHINGTOREPRODUCETHEIROWN Daniel Hurd, D.O. ARTICLES2EQUESTFORPERMISSIONSHOULDBEDIRECTEDTO*!/#$CO!/#$ 0/"OX+IRKSVILLE -/ Mark Lebwohl, M.D. #OPYRIGHTBYTHE*OURNALOFTHE!MERICAN/STEOPATHIC#OLLEGEOF Edward Yob, D.O. $ERMATOLOGY Jere Mammino, D.O. Printed by: Stoyles Graphics Services, Mason City, IA 50401 Schield M. -
J.M. Azaña Defez, M.L. Martínez Martínez Doctors in Medicine and Surgery
Acne J.M. Azaña Defez, M.L. Martínez Martínez Doctors in Medicine and Surgery. Consultant Physicians at the Dermatology Service. Pediatric Dermatology Unit. University Hospital Complex of Albacete Abstract Resumen Acne is a chronic inflammatory skin disease of El acné es una enfermedad cutánea inflamatoria the pilosebaceous unit of multifactorial etiology crónica del folículo pilosebáceo, de origen characterized by increased sebaceous secretion, multifactorial, caracterizada por: aumento de la comedone formation, inflammatory lesions and secreción sebácea, formación de comedones, lesiones risk of scarring sequelae. It is undoubtedly one inflamatorias y riesgo de secuelas cicatrizales. of the most frequent dermatological processes Es, sin duda, uno de los procesos dermatológicos in the daily clinical practice, especially in más frecuentes en la práctica clínica diaria, adolescence, although it can also appear in especialmente en la adolescencia, aunque también childhood and persist into adulthood. Adequate puede aparecer en niños y persistir en la edad management of this pathology is relevant, adulta. Es importante un manejo adecuado de esta as it can cause lower self-esteem and social patología, que puede producir una disminución de la dysfunction in patients, with the subsequent autoestima y disfunción social de los pacientes, con impact on quality of life. el consiguiente impacto en la calidad de vida. Key words: Cutibacterium acnes; Grading and classification of acne; Acne management; Isotretinoin. Palabras clave: Acné; Cutibacterium acnes; Graduación y clasificación del acné; Manejo del acné; Isotretinoina. Introduction Etiopathogenesis acne among the Spanish population Acne is a frequent inflammatory skin aged 12 to 18 years is 74%, without Acne is a multifactorial disease, produced disease of chronic course and polymor- significant differences regarding sex by: increased sebaceous secretion, follicu- phous in its clinical expression. -
04 Rampazzo 04 Rampazzo
Heart International / Vol. 2 no. 1, 2006 / pp. 17-26 © Wichtig Editore, 2006 Genetic bases of arrhythmogenic right ventricular cardiomyopathy ALESSANDRA RAMPAZZO Department of Biology, University of Padova - Italy ABSTRACT: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease in which the pathological substrate is a fibro-fatty replacement of the right ventricular myocardi- um. The major clinical features are different types of arrhythmias with a left branch block pattern. ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms were also described, although in association with skin disorders. Ten genetic loci have been discovered so far and mutations were reported in five different genes. ARVD1 was associated with regulatory mutations of transforming growth factor beta-3 (TGFβ3), whereas ARVD2, characterized by effort-induced polymorphic arrhythmias, was associated with mutations in cardiac ryanodine receptor-2 (RYR2). All other mutations identified to date have been detected in genes encoding desmosomal proteins: plakoglobin (JUP) which causes Naxos disease (a recessive form of ARVC associated with palmoplantar keratosis and woolly hair); desmoplakin (DSP) which causes the autosomal dominant ARVD8 and plakophilin-2 (PKP2) in- volved in ARVD9. Desmosomes are important cell-to-cell adhesion junctions predominantly found in epidermis and heart; they are believed to couple cytoskeletal elements to plasma mem- brane in cell-to-cell or cell-to-substrate adhesions. (Heart International 2006; 2: 17-26) KEY WORDS: Arrhythmias, Sudden death, Molecular genetics, Desmosomes INTRODUCTION electrocardiographic depolarization/repolarization changes and arrhythmias of right ventricular origin (5). Ventricu- Arrhythmogenic right ventricular cardiomyopathy/ lar tachycardias are thought to be due to re-entry be- dysplasia (ARVC/D) is a myocardial disease in which tween the abnormal and normal areas of the right ven- myocardium of the right ventricular free wall is partially tricular myocardium. -
De Novo Heterozygous Desmoplakin Mutations Leading to Naxos-Carvajal Disease
Zurich Open Repository and Archive University of Zurich Main Library Strickhofstrasse 39 CH-8057 Zurich www.zora.uzh.ch Year: 2012 De novo heterozygous desmoplakin mutations leading to Naxos-Carvajal disease Keller, Dagmar I ; Stepowski, Dimitri ; Balmer, Christian ; Simon, Françoise ; Guenthard, Joelle ; Bauer, Fabrice ; Itin, Peter ; David, Nadine ; Drouin-Garraud, Valérie ; Fressart, Véronique Abstract: STUDY/PRINCIPLES: Arrythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal-dominantly inherited disease caused by mutations in genes encoding desmosomal proteins and is characterised by fibrofatty replacement occurring predominantly in the right ventricle and canre- sult in sudden cardiac death. Naxos and Carvajal syndrome, autosomal recessive forms of ARVC/D, are characterised by involvement of the right and/or left ventricle in association with palmoplantar kerato- derma and woolly hair. The aim of the present study has been to screen for mutations in the desmosomal protein genes of two unrelated patients with Naxos-Carvajal syndrome. METHODS AND RESULTS: Desmosomal protein genes were screened for mutations by polymerase chain reaction as well as direct sequencing approach. In each patient we identified a single heterozygous de novo mutation in the desmo- plakin gene DSP, p.Leu583Pro and p.Thr564Ile, leading to severe combined cardiac/dermatological and cardiac/dermatological/dental phenotypes. The DSP missense mutations are localised in the N termi- nal domain of desmoplakin. CONCLUSION: The identified variations in DSP involve highly conserved residues. Moreover, the variations are de novo mutations and they are localised in critical protein domains that appear to be mutation hot spots. We assume that these heterozygous variations are causal for the mixed Naxos-Carvajal syndrome phenotype in the screened patients. -
Impact of UVA on Pruritus During UVA/B-Phototherapy of Inflammatory Skin Diseases : a Randomized Double-Blind Study
Zurich Open Repository and Archive University of Zurich Main Library Strickhofstrasse 39 CH-8057 Zurich www.zora.uzh.ch Year: 2017 Impact of UVA on pruritus during UVA/B-phototherapy of inflammatory skin diseases : a randomized double-blind study Maul, Julia-Tatjana ; Kretschmer, Lorenz ; Anzengruber, Florian ; Pink, Andrew ; Murer, Carla ; French, Lars E ; Hofbauer, Günther F L ; Navarini, Alexander A Abstract: BACKGROUND Narrowband (TL-01) UVB phototherapy (UVB nb) is effective in treating inflammatory skin disease. The addition of UVA is traditionally advocated to reduce pruritus, butlacks evidence for this recommendation. OBJECTIVES The aim of this study was to assess the effect of UVB nb and UVA phototherapy in combination compared against UVB nb monotherapy on pruritus, disease activity, and quality of life. METHODS In this double-blind randomised clinical trial 53 patients suffering from inflammatory skin diseases with pronounced itching (Visual Analogue Scale (VAS) for pruritus5) were randomised in to two treatment groups. One group received UVB nb (311nm) phototherapy alone and another group received a combination of UVB nb and UVA (320-400nm) phototherapy. UV therapy was performed three times per week over 16 weeks. Pruritus (VAS and 5-D itch score), disease activity and quality of life (Dermatology Life Quality Index, DLQI) were assessed at baseline and weeks 4, 8, 12, and 16. RESULTS In both treatment groups there was a reduction in pruritus scores, disease activity and DLQI. No difference in pruritus score, disease activity, and quality of life could be detected between thegroup receiving UVB nb alone and those receiving UVB nb combined with UVA. -
A Study of Cutaneous Manifestations Associated with Diabetes Mellitus
International Journal of Advances in Medicine Kadam MN et al. Int J Adv Med. 2016 May;3(2):296-303 http://www.ijmedicine.com pISSN 2349-3925 | eISSN 2349-3933 DOI: http://dx.doi.org/10.18203/2349-3933.ijam20161079 Research Article A study of cutaneous manifestations associated with diabetes mellitus Manish N. Kadam1*, Pravin N. Soni2, Sangita Phatale3, B. Siddramappa4 1Department of Skin & VD, Indian Institute of Medical Science & Research Medical College, Badnapur, Jalna, Maharashtra, India 2 Department of General Medicine, Indian Institute of Medical Science & Research Medical College, Badnapur, Jalna, Maharashtra, India 3Department of Physiology, MGM Medical College, Aurangabad, Maharashtra, India 4Department of Skin & VD, KLE’s Medical College, Belgaum, Karnataka, India Received: 21 March 2016 Accepted: 19 April 2016 *Correspondence: Dr. Manish N. Kadam, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Background: Diabetes mellitus is a fairly common medical disorder that involves almost every specialty in its rd spectrum of clinical manifestation, and up to 1/3 of patients with diabetes mellitus are estimated to have cutaneous changes. In other words using broadest criteria all subjects with diabetes will diabetes will develop cutaneous manifestations of this disease. Methods: A total of 100 diabetic patients with cutaneous manifestations, who attended skin OPD at Civil Hospital, Belgaum, Karnataka, India and K.L.E’S Hospital and Medical Research Centre, were randomly selected. -
Zbornik Sestanka: Alergijske Bolezni Kože
} ZDRUŽENJE SLOVENSKIH DERMATOLOGOV } ALERGOLOŠKA IN IMUNOLOŠKA SEKCIJA SZD } UNIVERZITETNA KLINIKA ZA PLJUČNE BOLEZNI IN ALERGIJO GOLNIK } DERMATOVENEROLOŠKA KLINIKA, UKC LJUBLJANA Zbornik sestanka: Alergijske bolezni kože Ptuj, Hotel Mitra 12-13. februar 2010 Izdajatelj Univerzitetna klinika za pljučne bolezni in alergijo Golnik Uredniki zbornika Tanja Planinšek Ručigaj, Tomaž Lunder, Mitja Košnik Recenzenta Nada Kecelj Leskovec, Mitja Košnik Tehnični urednik Robert Marčun Organizacija srečanja Sandi Luft, Robert Marčun Ptuj, Hotel Mitra 12-13. februar 2010 CIP - Kataložni zapis o publikaciji Narodna in univerzitetna knjižnica, Ljubljana 616.5(082) ALERGIJSKE bolezni kože : zbornik sestanka, Ptuj, 12.-13. februar 2010 / [uredniki zbornika Tanja Planinšek Ručigaj, Tomaž Lunder, Mitja Košnik]. - Golnik : Bolnišnica, Klinika za pljučne bolezni in alergijo, 2010 ISBN 978-961-6633-27-7 1. Planinšek Ručigaj, Tanja 249783808 2 Strokovno srečanje Združenja slovenskih dermatologov in Alergološke in imunološke sekcije z naslovom Alergijske bolezni kože so omogočili: Beiersdorf Lex Schering Plough AstraZeneca Bayer Dr.Gorkič Glaxo SmithKline Ewopharma IRIS Janssen-Cilag Krka LKB L'OREAL Slovenija MSD Nycomed Oktal Pharma Pharmagan PROVENS 3 Alergijske bolezni kože Strokovni odbor: predsednik : Tomaž Lunder, Mitja Košnik, člani : Vlasta Dragoš, Helena Rupnik, Maja Kalač Pandurovič, Marko Vok, Maja Zierkelbach, Nada Kecelj Leskovec, Mihaela Zidarn Organizacijski odbor: predsednik : Sandi Luft in Robert Marčun; člani : Tanja Kmecl, Aleksandra Dugonik, -
Carvajal Syndrome: a Rare Variant of Naxos Disease
2020 Extended Abstract Journal of Clinical Cardiology and Research Vol.3 No.1 World Cardiology Summit 2020: Carvajal Syndrome: A Rare Variant of Naxos Disease Madhu KJ Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, India Introduction: tachycardia with HR 140/min, RR 40/min, BP 80/60 mmhg, Carvajal syndrome additionally recognized as ‘Striate SPO2 72%, raised JVP, with B/L basal crepitation’s, S3 gallop palmoplantar keratoderma with woolly hair and present. Echocardiographic examination published a dilatation cardiomyopathy is a cutaneous situation inherited in an of the proper and left ventricles with a left ventricular ejection autosomal recessive sample due to a defect in desmoplakin fraction of 35% and biventricular trabecular configuration, gene. The pores and skin sickness affords as a striate predominantly of the left ventricle and additionally grade I palmoplantar keratoderma mainly at web sites of pressure. The tricuspid and Mitral valve insufficiency. Patient used to be dealt affected person is at danger of surprising cardiac loss of life due with with diuretics, beta-blockers’ and ACE inhibitors. to dilated cardiomyopathy related with this entity. A variant of Carvajal syndrome additionally recognized as ‘Striate Naxos disease, said as Carvajal syndrome, has been described palmoplantar keratoderma with woolly hair and in households from India and Ecuador. Clinically, it gives with cardiomyopathy is a cutaneous circumstance inherited in an the equal cutaneous phenotype and predominantly left autosomal recessive sample due to a defect in desmoplakin ventricular involvement. gene. The pores and skin disorder gives as a striate palmoplantar keratoderma specially at web sites of pressure. -
Views in Allergy and Immunology
CLINICAL REVIEWS IN ALLERGY AND IMMUNOLOGY Dermatology for the Allergist Dennis Kim, MD, and Richard Lockey, MD specific laboratory tests and pathognomonic skin findings do Abstract: Allergists/immunologists see patients with a variety of not exist (Table 1). skin disorders. Some, such as atopic and allergic contact dermatitis, There are 3 forms of AD: acute, subacute, and chronic. are caused by abnormal immunologic reactions, whereas others, Acute AD is characterized by intensely pruritic, erythematous such as seborrheic dermatitis or rosacea, lack an immunologic basis. papules associated with excoriations, vesiculations, and se- This review summarizes a select group of dermatologic problems rous exudates. Subacute AD is associated with erythematous, commonly encountered by an allergist/immunologist. excoriated, scaling papules. Chronic AD is associated with Key Words: dermatology, dermatitis, allergy, allergic, allergist, thickened lichenified skin and fibrotic papules. There is skin, disease considerable overlap of these 3 forms, especially with chronic (WAO Journal 2010; 3:202–215) AD, which can manifest in all 3 ways in the same patient. The relationship between AD and causative allergens is difficult to establish. However, clinical studies suggest that extrinsic factors can impact the course of disease. Therefore, in some cases, it is helpful to perform skin testing on foods INTRODUCTION that are commonly associated with food allergy (wheat, milk, llergists/immunologists see patients with a variety of skin soy, egg, peanut, tree nuts, molluscan, and crustaceous shell- Adisorders. Some, such as atopic and allergic contact fish) and aeroallergens to rule out allergic triggers that can dermatitis, are caused by abnormal immunologic reactions, sometimes exacerbate this disease. -
Table I. Genodermatoses with Known Gene Defects 92 Pulkkinen
92 Pulkkinen, Ringpfeil, and Uitto JAM ACAD DERMATOL JULY 2002 Table I. Genodermatoses with known gene defects Reference Disease Mutated gene* Affected protein/function No.† Epidermal fragility disorders DEB COL7A1 Type VII collagen 6 Junctional EB LAMA3, LAMB3, ␣3, 3, and ␥2 chains of laminin 5, 6 LAMC2, COL17A1 type XVII collagen EB with pyloric atresia ITGA6, ITGB4 ␣64 Integrin 6 EB with muscular dystrophy PLEC1 Plectin 6 EB simplex KRT5, KRT14 Keratins 5 and 14 46 Ectodermal dysplasia with skin fragility PKP1 Plakophilin 1 47 Hailey-Hailey disease ATP2C1 ATP-dependent calcium transporter 13 Keratinization disorders Epidermolytic hyperkeratosis KRT1, KRT10 Keratins 1 and 10 46 Ichthyosis hystrix KRT1 Keratin 1 48 Epidermolytic PPK KRT9 Keratin 9 46 Nonepidermolytic PPK KRT1, KRT16 Keratins 1 and 16 46 Ichthyosis bullosa of Siemens KRT2e Keratin 2e 46 Pachyonychia congenita, types 1 and 2 KRT6a, KRT6b, KRT16, Keratins 6a, 6b, 16, and 17 46 KRT17 White sponge naevus KRT4, KRT13 Keratins 4 and 13 46 X-linked recessive ichthyosis STS Steroid sulfatase 49 Lamellar ichthyosis TGM1 Transglutaminase 1 50 Mutilating keratoderma with ichthyosis LOR Loricrin 10 Vohwinkel’s syndrome GJB2 Connexin 26 12 PPK with deafness GJB2 Connexin 26 12 Erythrokeratodermia variabilis GJB3, GJB4 Connexins 31 and 30.3 12 Darier disease ATP2A2 ATP-dependent calcium 14 transporter Striate PPK DSP, DSG1 Desmoplakin, desmoglein 1 51, 52 Conradi-Hu¨nermann-Happle syndrome EBP Delta 8-delta 7 sterol isomerase 53 (emopamil binding protein) Mal de Meleda ARS SLURP-1 -
495 L. Rudnicka Et Al. (Eds.), Atlas of Trichoscopy, DOI 10.1007/978-1
Index A with patchy, partial regrowth , 207 Acne keloidalis nuchae , 332 pigtail regrowing hairs in , 218 Acquired hair shaft dystrophy Pohl-Pinkus constriction , 212 cicatricial alopecia , 41 regularly coiled hairs , 206 lichen planopilaris , 288 short damaged hair , 211 Acquired trichorrhexis nodosa , 163, 164 tapered hairs , 212, 215 Actinic keratosis (AK) trichorrhexis nodosa , 211, 216 nonpigmented , 422 trichoscopic features , 205, 206, 219 pigmented , 423 tulip hairs in , 216 Addison’s disease , 484, 492 upright regrowing hairs in , 217 AGA . See Androgenetic alopecia (AGA) vellus hairs in , 218 Air bubbles in vivo re fl ectance confocal microscopy (RCM) , 219 heavy vs. light pressure on dermoscope , 130 yellow dots , 57–60, 208, 209 in immersion fl uid , 129 zigzag hairs in , 216, 217 AK . See Actinic keratosis (AK) Alopecia areata incognita , 231, 246, 251 Algorithms dark lines in , 254 androgenetic alopecia , 453, 454 regrowing hairs , 252, 253 3-A system , 453–455 S-shaped hairs , 252, 253 cicatricial alopecia , 455 tadpole-like hairs , 253 clinical trials , 453 yellow dots , 62, 252, 254 differential diagnosis of , 453–455 Alopecia groenlandica . See Traction alopecia erythema , 456 Alopecia mucinosa monitoring , 454 general medicine , 488 noncicatricial alopecia systemic lymphoproliferative diseases , 476 diffuse , 454 Alopecia totalis , 207 focal , 455 Amicrobial pustulosis scaling , 456 in discoid lupus erythematosus , 92, 314 scalp psoriasis , 454, 456 general medicine , 485, 486 two-step method , 453 Amorphous hair residues,