Fig. 1. Lentigo Simplex. Elongated Rete Ridges, Hyperpigmentation of The
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Paraneoplastic Syndrome Presenting As Giant Porokeratosis in a Patient with Nasopharyngeal Cancer
Paraneoplastic Syndrome Presenting As Giant Porokeratosis in A Patient with Nasopharyngeal Cancer Fitri Azizah, Sonia Hanifati, Sri Adi Sularsito, Lili Legiawati, Shannaz Nadia Yusharyahya, Rahadi Rihatmadja Department of Dermatology and Venereology, Faculty of Medicine Universitas Indonesia / Dr. Cipto Mangunkusumo National General Hospital Keywords: porokeratosis, giant porokeratosis, paraneoplastic syndrome, nasopharyngeal Abstract: Giant porokeratosis is a rare condition in which the hyperkeratotic plaques of porokeratosis reach up to 20 cm in diameter. Porokeratosis is characterized clinically by hyperkeratotic papules or plaques with a thread-like elevated border. Although rare, porokeratosis has been reported in conjunction with malignancies suggesting a paraneoplastic nature. Associated malignancies reported were hematopoietic, hepatocellular, and cholangiocarcinoma. We report a case of giant porokeratosis in a patient with nasopharyngeal cancer responding to removal of the primary cancer by chemoradiotherapy. 1 INTRODUCTION regress completely after the treatment of malignancy, suggestive of paraneoplastic syndrome. Porokeratosis is a chronic progressive disorder of keratinization, characterized by hyperkeratotic papules or plaques surrounded by a thread-like 2 CASE elevated border corresponds to a typical histologic hallmark, the cornoid lamella . O regan, 2012) There Mr. SS, 68-year-old, was referred for evaluation of are at least six clinical variants of porokeratosis pruritic, slightly erythematous plaques with raised, recognized with known genetic disorder.1 Some hyperpigmented border of one and a half year clinical variant of porokeratosis has been reported in duration on the extensor surface of both legs. The the setting of immunosuppressive conditions, organ lesions shown minimal response to potent topical transplantation, use of systemic corticosteroids, and corticosteroids and phototherapy given during the infections, suggesting that impaired immunity may last 8 months in another hospital. -
Features of Reactive White Lesions of the Oral Mucosa
Head and Neck Pathology (2019) 13:16–24 https://doi.org/10.1007/s12105-018-0986-3 SPECIAL ISSUE: COLORS AND TEXTURES, A REVIEW OF ORAL MUCOSAL ENTITIES Frictional Keratosis, Contact Keratosis and Smokeless Tobacco Keratosis: Features of Reactive White Lesions of the Oral Mucosa Susan Müller1 Received: 21 September 2018 / Accepted: 2 November 2018 / Published online: 22 January 2019 © Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract White lesions of the oral cavity are quite common and can have a variety of etiologies, both benign and malignant. Although the vast majority of publications focus on leukoplakia and other potentially malignant lesions, most oral lesions that appear white are benign. This review will focus exclusively on reactive white oral lesions. Included in the discussion are frictional keratoses, irritant contact stomatitis, and smokeless tobacco keratoses. Leukoedema and hereditary genodermatoses that may enter in the clinical differential diagnoses of frictional keratoses including white sponge nevus and hereditary benign intraepithelial dyskeratosis will be reviewed. Many products can result in contact stomatitis. Dentrifice-related stomatitis, contact reactions to amalgam and cinnamon can cause keratotic lesions. Each of these lesions have microscopic findings that can assist in patient management. Keywords Leukoplakia · Frictional keratosis · Smokeless tobacco keratosis · Stomatitis · Leukoedema · Cinnamon Introduction white lesions including infective and non-infective causes will be discussed -
Review an Overview of the Pale and Clear Cells of the Nipple Epidermis
Histol Histopathol (2009) 24: 367-376 Histology and http://www.hh.um.es Histopathology Cellular and Molecular Biology Review An overview of the pale and clear cells of the nipple epidermis M.F. Garijo, D. Val and J.F. Val-Bernal Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Summary. The stratified squamous epithelium of the exterior. In the non-lactating breast these duct openings nipple-areola complex may contain pale or clear cells are usually filled with plugs of keratin. The nipple and including: Paget’s disease cells (PDCs), Toker cells areola are covered by keratinizing stratified squamous (TCs), and so-called clear cells (CCs). Paget’s disease is epithelium similar to that seen in the epidermis an uncommon presentation of breast carcinoma. PDCs elsewhere in the body. The collecting ducts show a are large, atypical, have abundant, pale-staining double epithelial and myoepithelial lining. The cytoplasm that may contain mucin secretion vacuoles epithelium is columnar and the myoepithelial cells lie and bulky heterochromatic nuclei. They are commonly between the epithelial layer and the basal lamina. A concentrated along the basal layer and stain for EMA, cross section of the major ducts shows an irregular, CAM5.2, cytokeratin 7, and HER2/neu oncoprotein. TCs pleated or serrated outline and an investment with are bland cells with roundish and scant chromatin nuclei. muscular tissue. The areola dermis contains numerous They are found incidentally and are reactive for EMA, sebaceous glands. Some of them open directly onto the CAM5.2, and cytokeratin 7, but show negativity for surface, whereas others drain into a collecting duct or HER2/neu oncoprotein. -
An Update on the Biology and Management of Dyskeratosis Congenita and Related Telomere Biology Disorders
Expert Review of Hematology ISSN: 1747-4086 (Print) 1747-4094 (Online) Journal homepage: https://www.tandfonline.com/loi/ierr20 An update on the biology and management of dyskeratosis congenita and related telomere biology disorders Marena R. Niewisch & Sharon A. Savage To cite this article: Marena R. Niewisch & Sharon A. Savage (2019) An update on the biology and management of dyskeratosis congenita and related telomere biology disorders, Expert Review of Hematology, 12:12, 1037-1052, DOI: 10.1080/17474086.2019.1662720 To link to this article: https://doi.org/10.1080/17474086.2019.1662720 Accepted author version posted online: 03 Sep 2019. Published online: 10 Sep 2019. Submit your article to this journal Article views: 146 View related articles View Crossmark data Citing articles: 1 View citing articles Full Terms & Conditions of access and use can be found at https://www.tandfonline.com/action/journalInformation?journalCode=ierr20 EXPERT REVIEW OF HEMATOLOGY 2019, VOL. 12, NO. 12, 1037–1052 https://doi.org/10.1080/17474086.2019.1662720 REVIEW An update on the biology and management of dyskeratosis congenita and related telomere biology disorders Marena R. Niewisch and Sharon A. Savage Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA ABSTRACT ARTICLE HISTORY Introduction: Telomere biology disorders (TBDs) encompass a group of illnesses caused by germline Received 14 June 2019 mutations in genes regulating telomere maintenance, resulting in very short telomeres. Possible TBD Accepted 29 August 2019 manifestations range from complex multisystem disorders with onset in childhood such as dyskeratosis KEYWORDS congenita (DC), Hoyeraal-Hreidarsson syndrome, Revesz syndrome and Coats plus to adults presenting Telomere; dyskeratosis with one or two DC-related features. -
Inflammatory Skin Disease Every Pathologist Should Know
Inflammatory skin disease every pathologist should know Steven D. Billings Cleveland Clinic [email protected] General Concepts • Pattern recognition – Epidermal predominant vs. dermal predominant • Epidermal changes trump dermal changes – Distribution of the inflammatory infiltrate • Superficial vs. superficial and deep • Location: perivascular, interstitial, nodular – Nature of inflammatory infiltrate • Mononuclear (lymphocytes and histiocytes) • Mixed (mononuclear and granulocytes) • Granulocytic • Correlation with clinical presentation • Never diagnose “chronic nonspecific dermatitis” Principle Patterns: Epidermal Changes Predominant • Spongiotic pattern • Psoriasiform pattern – Spongiotic and psoriasiform often co-exist • Interface pattern – Basal vacuolization • Perivascular infiltrate or • Lichenoid infiltrate Principle Patterns: Dermal Changes Predominant • Superficial perivascular • Superficial and deep perivascular • Interstitial pattern – Palisading granulomatous – Nodular and diffuse • Sclerosing pattern • Panniculitis • Bullous disease • Miscellaneous Spongiotic Dermatitis • Three phases – Acute – Subacute – Chronic • Different but overlapping histologic features Spongiotic Dermatitis • Acute spongiotic dermatitis – Normal “basket-weave” stratum corneum – Pale keratinocytes – Spongiosis – Spongiotic vesicles (variable) – Papillary dermal edema – Variable superficial perivascular infiltrate of lymphocytes often with some eosinophils – Rarely biopsied in acute phase Spongiotic Dermatitis • Subacute spongiotic dermatitis – Parakeratosis -
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Index A Becker nevus and Becker nevus syndrome , Acantholysis, transient superfi cial , 158–159 14, 15, 86, 88–90 Acanthosis nigricans , 19, 20, 141–142, 145, 146 BFH. See Basaloid follicular hamartoma (BFH) Acne nevus of Munro , 91 Binary genodermatoses , 23, 115–118 Acne vulgaris , 26, 200–201, 203 aplasia cutis congenita Acrokeratoelastoidosis , 146 and nevus psiloliparus , 116, 117 AHO. See Albright’s hereditary osteodystrophy (AHO) and nevus sebaceous , 116 Aicardi syndrome , 188–189 nevus sebaceus and melorheostosis , 116 AKT1 mutation , 79, 83, 137 phacomatosis pigmentokeratotica , 115–116 Albinism-deafness syndrome, X-linked , 189–191 phacomatosis pigmentovascularis , 116–118 Albinism, X-linked oculocutaneous , 54 Birt-Hogg-Dubé syndrome. See Hornstein-Knickenberg Albright’s hereditary osteodystrophy (AHO) , 19, 20, syndrome 151–154 Blaschkitis , 49, 195, 205, 208 Allelic didymosis , 23–24, 109–111 Blaschko, Alfred , 9, 45, 47–51 capillary didymosis , 109–111 Blaschko dermatitis , 195 cutis tricolor , 110–111 Blaschko’s lines , 45–59, 63–64, 74 Darier disease , 111, 112 analogous patterns in other organs , 53–58 epidermolytic ichthyosis of Brocq , 111 in animals , 51, 54–56 Amelogenesis imperfecta , 53 atypical lines , 63–64 Anemic halo. See Rhodoid nevus brindle trait , 51, 54, 55 Angiokeratoma circumscriptum , 96 in broad bands , 46, 52–53 Angioma serpiginosum , 96–97 in chimeric mice , 51, 54 Angora hair nevus , 15, 86, 88–89 dwarf zebu, brindle trait , 54, 55 Apert syndrome , 91 embryonic cells , 49, 50 Archetypical patterns , 45–59 in epidermal nevi , 50, 52 Arteriovenous fi stulas , 20, 138, 156 on head and neck , 47, 50 Art nouveau , 57 intraoral lesions , 52 Atopic dermatitis , 26, 194, 195, 197–199 murine brain , 54, 56 ATP7A gene , 188 in narrow bands , 52 ATP2C1 mutation. -
Incidental Focal Acantholytic Dyskeratosis in the Setting of Rosacea
Letter to the Editor http://dx.doi.org/10.5021/ad.2013.25.4.518 Incidental Focal Acantholytic Dyskeratosis in the Setting of Rosacea Sang-Yeon Park, Hae Jin Lee, Jae Yong Shin, Sung Ku Ahn Department of Dermatology, Yonsei University Wonju College of Medicine, Wonju, Korea Dear Editor: direct immunofluorescence (Fig. 2). In the serial sections, Focal acantholytic dyskeratosis (FAD) was first described we observe the same findings. Differential diagnosis for by Ackerman1 in 1972 as a distinct histopathological pat- the possibility of polymorphous light eruption, systemic tern associated with various cutaneous conditions, and lupus erythematosus, contact dermatitis, and dermatitis with classic histopathological findings including supra- artefacta should be considered. But given these clinical basal clefting, hyperkeratosis and parakeratosis, and the and histopathological features, a diagnosis of rosacea with presence of acantholytic and dyskeratotic cells at the FAD was reached. The patient was then admitted to epidermis. While FAD can be observed in many various hospital for treatment with doxycycline 100 mg and cutaneous lesions including benign and/or malignant antihistamines. After one week, the lesions had remarka- epithelial lesions, fibrohistiocytic lesions, inflammatory bly improved. The patient was then discharged, and lesions, melanocytic and/or follicular lesions2-4. These continued on the same therapeutic regimen for an histopathological findings may also extend into the additional month, bythe time all lesions were nearly surrounding tissues, which often appear to be clinically resolved. normal. A 42-year-old woman was presented to our To date, the etiology of FAD has been attributed to department with multiple erythematous pruritic papules numerous sources including hormones, viral infection, and tiny vesicles on her face. -
ECZEMA) Shankar Pratap K
Research Article International Ayurvedi c Medical Journal ISSN:2320 5091 A HISTOPATHOLOGICAL STUDY ON LEECH APPLICATION IN THE MANAGEMENT OF VICARCIKA (ECZEMA) Shankar Pratap K. M.1 Rao Dattatreya 2 Sai Prasad 3 1Dept. of Shalya Tantra , Santhigiri Ayurvedic College , Palakkad, Kerala, India 2Dept. of Shalya Tantra, S.V. Ayurvedic College, Tirupati , Andhra Pradesh , India 3Dept. of Pathology, S. V. Medical College, Tirupati, Andhra Pradesh, India ABSTRACT To assess the efficacy of Leech application in the management of Vicarcik a (Eczema) with Histopathological study, the present study with 10 patients having the classical symptoms of Vicarcika, were randomly selected as per the inclusion and exclusion criteria from O.P.D. & I.P.D. sections of Shalya department, S.V. Ayurvedic Hospital, Tirupati. Minimum 4 sittings of Leech application was carried out with seven days interval. Total duration of treatment was 6 weeks. Biopsy samples were collected from the lesion site before and after treatment. Histopathological examination was done by the pathologist. In eczema (dermatitis) the leech application therapy gives excellent response by reducing the inflammatory component, hyperkeratosis, spongiosis, irregular acanthosis and by evoking a granulation tissue response in the dermis and in most of the cases with complete recovery from the lesion. Most of the cases in the study were chronic dermatitis and sebhoric keratosis, almost all local/focal pigmented lesion is totally relieved by leech therapy especially in cases of sebhoric keratos is. In the present study it was found that, leech application evokes significant changes at histological level specifically in reduction of inflammatory component, hyperkeratosis, spongiosis and irregular acanthosis. -
Diffuse Drug-Induced Dermatitis Following Sclerotherapy for Telangiectasias Libby MW, Caitlin WH, Deniz OA and Heller JA*
Case Report iMedPub Journals Journal of Vascular and Endovascular Surgery 2016 http://www.imedpub.com/ Vol.1 No.3:17 DOI: 10.21767/2573-4482.100017 Diffuse Drug-Induced Dermatitis following Sclerotherapy for Telangiectasias Libby MW, Caitlin WH, Deniz OA and Heller JA* Department of Surgery, Johns Hopkins Vein Center, Johns Hopkins Medical Centers, Baltimore, USA *Corresponding author: Jennifer A Heller, 10755 Falls Road, Pavilion 1 Suite 360, Lutherville, MD 21903, Tel: 4105500415; Email: [email protected] Received date: June 14, 2016; Accepted date: July 26, 2016; Published date: August 02, 2016 Copyright: © 2016 Libby MW, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Abstract Sclerotherapy is an effective treatment modality for the management of lower extremity telangiectasias. Although localized dermatologic reactions are known complications of this procedure, systemic reactions are rare. Here, we present a case of a diffuse drug eruption following sclerotherapy for the treatment of bilateral lower extremity telangiectasias. Salient clinical and physical exam findings are described. Management strategies for the treatment of drug eruptions are outlined. This is the first case report that we know of describing a diffuse drug eruption associated with sclerotherapy. It is important to recognize the possibility of diffuse drug eruptions secondary to sclerotherapy treatment so that expectant management may be initiated expeditiously in affected patients. Figure 1: Drug eruption following sclerotherapy. The patient was referred for evaluation by a dermatologist, Case Report who initially diagnosed diffuse dermatitis based on clinical A 64-year-old woman with hypertension and bilateral lower exam. -
JMSCR Vol||07||Issue||03||Page 281-286||March 2019
JMSCR Vol||07||Issue||03||Page 281-286||March 2019 www.jmscr.igmpublication.org Index Copernicus Value: 79.54 ISSN (e)-2347-176x ISSN (p) 2455-0450 DOI: https://dx.doi.org/10.18535/jmscr/v7i3.51 Histopathological Evaluation of Collagen Profiles in Spongiotic Dermatitis Authors Anjali Patankar, Ramya Gandhi*, Erli Amel Ivan Department of Pathology, Sri Manakula Vinayagar Medical College and Hospital, Kalitheerthalkuppam, Puducherry, 605 107 India *Corresponding Author Dr Ramya Gandhi Associate Professor, Department of Pathology, Sri Manakula Vinayagar Medical College and Hospital, Kalitheerthalkuppam, Puducherry, 605 107, India Email: [email protected], Mobile no- 9787740891 Abstract Context: Spongiotic Dermatitis is a common clinical condition also known as eczema, characterized by rashes and itching further may progress to scarring. The terms eczema and dermatitis are often used interchangeably to denote a polymorphic inflammatory reaction pattern involving the epidermis and dermis. Spongiosis refers to intraepidermal edema. Aim: To assess the orientation of collagen in eczematous spongiotic dermatitis. Material and Methods: The present study was done in the Department of Pathology, Sri Manakula Vinayagar Medical College, Pondicherry. Sixty diagnosed cases of spongiotic dermatitis diagnosed over a period of five years were taken in the study. Orientation of collagen was evaluated using Masson’s trichrome stain. Results: Spongiotic dermatitis occurred in all age groups but commonly seen in middle age to elderly. Male to female ratio was 1:1.2 The most common symptom was itching (85%) followed by scaling (63.3%). Most common site was upper extremities (80%). subacute cases were predominantly seen (38.3%) followed by chronic cases (33.3%). -
Boards Fodder Disorders of Dyschromia (Hypo- and Hyperpigmentation) by Parin Pearl Rimtepathip, MD, and Janna Mieko Vassantachart, MD
boards fodder Disorders of dyschromia (hypo- and hyperpigmentation) by Parin Pearl Rimtepathip, MD, and Janna Mieko Vassantachart, MD Genetic conditions Gene Disorder Pathophysiology Clinical Features (Unique Features) Mutation Dyskeratosis XLR (MC): Reduced telomerase activ- Male > Female. Bone marrow failure up to Congenita DKC 1 ity and abnormally short- 90% (increase risk of hematopoietic malig- (Zinsser-Engman- ened telomeres chro- nancies) + triad of abnormal skin pigmenta- Cole syndrome) AD: TERT, mosomal instability/cellu- tion (poikilodermatous patches of face/neck/ TERC lar replication dysfunction upper torso), onychodystrophy, premalignant oral leukoplakia (vs benign oral leukoplakia in Pachyonychia Congenita type I) Dyschromatosis AD: ADAR Heterozygous mutations in Presents by 6-years-old with hyper/hypopig- Symmetrica (SDAR the gene encodes an RNA mented macules restricted to sun-exposed Hereditaria gene) specific adenosine deami- skin on the dorsal aspects of bilateral (Reticulate nase extremities and face Acropigmentation of Dohi) Naegeli- AD: Location of expression of Allelic to DPR. Brown gray reticulated hyper- Franceschetti- Keratin keratin 14 - Basal kerati- pigmentation typically localized to abdomen, Jadassohn 14 nocytes develops around age 2 and improves after Syndrome (NFJS) puberty. Other findings: PPK + adermato- glyphia (no finger prints) + dental anomalies including early loss of teeth (not seen in DPR) + hypohidrosis + onychodystrophy Dermatopathia AD: Location of expression of Allelic to NFJS. Unique features: diffuse non- Pigmentosa Keratin keratin 14 - Basal kerati- scarring alopecia (not seen in NFJS) + ony- Reticularis (DPR) 14 nocytes chodystrophy + adermatoglyphia + persistent reticulated hyperpigmentation of torso and proximal UE + No dental anomalies Dyschromatosis AD/AR: Mutation in ATP bind- Japanese. Torso predominant with mottled Universalis ABCB6 ing cassette subfamily B, appearance, nail dystrophy, and pterygium. -
Review of the Literature
Review Clinical and Histopathological Features and Potential Pathological Mechanisms of Skin Lesions in COVID-19: Review of the Literature Gürkan Kaya 1,*, Aysin Kaya 2 and Jean-Hilaire Saurat 2 1 Departments of Dermatology and Clinical Pathology, University Hospital of Geneva, 1205 Geneva, Switzerland 2 Department of Clinical Pharmacology and Toxicology, University of Geneva, 1205 Geneva, Switzerland; [email protected] (A.K.); [email protected] (J.-H.S.) * Correspondence: [email protected] Received: 24 June 2020; Accepted: 29 June 2020; Published: 30 June 2020 Abstract: In recent weeks, several reports have emerged of skin lesions with different clinical presentations in COVID-19 cases. All dermatologists should be aware of these cutaneous lesions, which may be early clinical symptoms of infection. We reviewed the literature on cutaneous manifestations in the PubMed database from December 2019 and June 2020. From the cases described as case reports or series in 57 recent articles, it appears that skin lesions (i) are highly varied, (ii) may not be related to the severity of the condition and (iii) resolve spontaneously in a few days. The frequency of these lesions in COVID-19 patients varies between 1.8% and 20.4%. The major clinical forms described were maculopapular eruptions, acral areas of erythema with vesicles or pustules (pseudochilblain), urticarial lesions, other vesicular eruptions and livedo or necrosis. The lesions were mainly localized in the trunk and extremities. The majority of patients were male, aged between 4.5 and 89 years. A minority of the patients were children presenting with acral, chilblain-like lesions, papulo-vesicular eruptions or Kawasaki disease-like pediatric inflammatory multisystem syndrome.