DOCSLIB.ORG

Lichen Myxedematosus: a Rare Group of Cutaneous Mucinosis*

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Lichen Myxedematosus: a Rare Group of Cutaneous Mucinosis* 462462Particular characteristicsSoares de Sá BC, of atopicMoredo eczema LF, Gomes in tropical EE, de environments. Araújo ESS, DupratTheREVIEW Tropical JP Environment... 462 s Lichen myxedematosus: a rare group of cutaneous mucinosis* Ramiro Eugenio Cárdenas-Gonzalez1, Maira Elizabeth Herz Ruelas1, Jorge Ocampo Candiani1 DOI: http://dx.doi.org/10.1590/abd1806-4841.20198478 Abstract: Cutaneous mucinoses are a heterogeneous group of dermatoses in which excess deposition of mucin in the dermis gives the skin a waxy appearance, with papules and plaques that can vary from self-healing mucinosis to even disrupting the normal shape of a patient’s face, conferring a leonine facies, or be part of life threatening diseases like scleromyxedema. This review will describe the most recent classification on lichen myxedematosus in the generalized (scleromyxedema) and the localized forms, as well as the different organ systems involved in scleromyxedema, diagnostic workup, current management, and prognosis. Keywords: Glycosaminoglycans; Mucinoses; Mucins; Scleromyxedema INTRODUCTION Cutaneous mucinoses are a heterogeneous group of skin di- in 1953, Montgomery and Underwood distinguished scleromyxe- sorders characterized by an abnormal dermal deposition of mucin. dema from scleroderma and generalized myxedema, describing Mucin is composed of glycosaminoglycans, hyaluronic acid, and four different clinical patterns, a generalized lichenoid eruption, a dermatan sulfate. It is a component of the extracellular matrix and discrete papular form, a localized or generalized lichenoid plaque, is produced by fibroblasts. Mucin can absorb 1,000 times its own and an urticarial plaque type.4 weight in water, and its main function is to maintain the salt and water balance within the dermis.1 Definition The cause of its abnormal cutaneous deposition remains LM represents a group of rare cutaneous diseases charac- unclear, although there are diverse proposed physiopathogenic me- terized by papules, nodules, and plaques of mucin deposition that chanisms, including paraproteinemia and serum factors that could give the skin a waxy appearance, with a chronic course and a poor promote mucin production within the skin. therapeutic response.5 This article describes lichen myxedematosus (LM), a cuta- neous mucinosis first reported as a systemic disorder associated Pathogenesis with paraproteinemia and extracutaneous involvement. A recent The pathogenesis of scleromyxedema is unknown. The classification by Rongioletti et al. includes a generalized papular and most accepted hypothesis is that circulating cytokines such as IL- sclerodermoid presentation (scleromyxedema), as well as localized, 1, TNF-alpha, and TGF-beta, which stimulate glycosaminoglycan atypical, and intermediate forms with or without systemic invol- synthesis and proliferation of fibroblasts, could play a role.6 In vi- vement.2 tro serum of patients with scleromyxedema can stimulate synthesis History of DNA by fibroblasts. Although monoclonal gammopathy occurs, Scleromyxedema was a term first introduced by Gottron in more often IgG is observed in patients with disseminated disease, 1954,3 giving credit to his former teacher Arndt for the original des- and levels of the paraproteinemia do not correlate with disease se- cription of a chronic dermatosis in a female patient. Nevertheless, verity. Moreover, tissue mucin deposition in autopsies does not cor- in 1908 Dubreuilh had previously described an identical dermatosis relate with the clinical findings.7 under the name of fibromes miliaries folliculaires: sclerodermie. Later Received 28 May 2018. Accepted 14 January 2019. * Study conducted at the Dermatology Service, Hospital Universitario, Facultad de Medicina, Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México. Financial support: None. Conflict of Interest: None. 1 Dermatology Service, Hospital Universitario, Facultad de Medicina, Universidad Autónoma de Nuevo León, Monterrey, Nuevo León, México. MAILING ADDRESS: Ramiro Eugenio Cárdenas-Gonzalez E-mail: [email protected] ©2019 by Anais Brasileiros de Dermatologia An Bras Dermatol. 2019;94(4):462-9. Lichen myxedematosus: a rare group of cutaneous mucinosis 463 Diagnostic criteria and classification Scleromyxedema The latest classification for LM by Rongioletti et al. distin- Scleromyxedema is a rare skin disorder clinically characte- guishes between three different subgroups of LM, each with diffe- rized by a disseminated eruption of 2-3 mm waxy, firm, dome-sha- rent diagnostic criteria: generalized LM (scleromyxedema), a locali- ped or flat-topped papules and nodules that may coalesce to form zed form, and an atypical variant which shares characteristics of the plaques involving the head, neck, trunk, and extremities. The scalp first two, but fulfilling diagnostic criteria for neither of them.5 and mucosa are generally not affected.9 Papules are commonly ar- Diagnostic criteria for generalized LM or scleromyxedema ranged in a linear array (Figure 2) and the surrounding skin has a are as follows: (a) microscopic triad of mucin deposition, fibroblast sclerodermoid appearance. There is no significant predominance by proliferation, and fibrosis; (b) monoclonal gammopathy, predomi- gender, and it is most common in adults between the fifth and sixth nantly IgG λ and less frequently IgG k; (c) absence of thyroid disease. decades of life.10 For the localized LM variant, five subtypes can be distin- Mucin deposition within the dermis is responsible for the guished: (a) discrete papular LM; (b) acral persistent papular mu- cutaneous findings. A sclerodermoid eruption with multiple papu- cinosis; (c) self-healing papular mucinosis; (d) papular mucinosis les, edema, and erythema – as well as papular induration over the of infancy; and (e) nodular LM. All of them must have the clinical ears and glabella – confer a leonine facies (Figure 3).11,12 Other skin and histopathological characteristics of LM but without paraprotei- findings include the doughnut sign, which is a central depression nemia, systemic involvement, or thyroid disease (Figure 1). surrounded by an elevated rim on extensor proximal phalangeal Atypical subtypes do not fulfill the mentioned criteria and joints. Deep furrowing can also be evident on the back. Skin thicke- share characteristics of both scleromyxedema and localized LM; the ning in chronic scleromyxedema can result in diminished articular latter is also known as papular mucinosis.5 movement and difficulty when opening the mouth. Pruritus and Recently, Nofal et al. proposed a grading system for LM ba- dysesthesia are also common complaints.10,11 sed on the extension of cutaneous involvement and the presence of By definition, a monoclonal gammopathy, more commonly systemic manifestations: G1 (mild), limited pure cutaneous involve- IgG λ and systemic involvement such as neurological, rheumatoid, ment; G2 (moderate), extensive pure cutaneous involvement; or G3 cardiac, pulmonary, gastrointestinal, hematologic, and ocular mani- (severe), limited or extensive cutaneous involvement plus systemic festations must be present in scleromyxedema. manifestations.8 Extracutaneous involvement has been reported in 70% to 77% of patients. Generalized Discrete Papular (scleromyxedema) Neurological involvement in scleromyxedema Paraproteinemia with or without hyperviscosity might ex- Acral Persistent Papular Mucinosis plain neurologic manifestations such as encephalopathy, seizures, cerebrovascular events, transient focal neurologic disturbances, and Lichen peripheral neuropathy observed in scleromyxedema.13,14 Mucin depo- Myxedematosus Self-healing Papular Mucinosis sition in the brain has not been demonstrated to be responsible for these. Peripheral neuropathy in scleromyxedema remains unclear, Papular Mucinosis of and carpal tunnel syndrome has been explained as a deposition of Infancy mucin within the wrist or secondary to direct median nerve toxicity.3 Localized (papular) Other neurological conditions related to scleromyxedema Nodular are considered iatrogenic, secondary to treatments with cytotoxic agents.15,16 Scleromyxedema without monoclonal gammopathy Localized form with monoclonal gammopathy/systemic symptoms Atypical/intermediate Localized form with combined features Not specified FIGURE 1: Classification of lichen myxedematosus by Rongioletti et al. FIGURE 2: Papules arranged in a linear array An Bras Dermatol. 2019;94(4):462-9. 464 Cardenas-González RE, Ruelas MEH, Candiani JO Dermato-neuro syndrome, presenting with fever, seizures, thyroid studies to rule out myxedema of thyroid disease are requi- and coma, along with a flu-like prodrome, is a rare complication red for adequate diagnosis. Additional studies to evaluate systemic of scleromyxedema. The pathophysiology is poorly understood, disease such as an echocardiogram or hepatic ultrasound may be but monoclonal gamma paraproteinemia and blood hyperviscosity necessary. have been postulated as causes for this rare complication.17,18 Treatment is empirical, although IVIG has been used suc- Localized forms of LM cessfully in combination with plasmapheresis. Other treatments Discrete papular LM described in the literature include thalidomide, corticosteroids, and Discrete papular lichen myxedematosus (DPLM) is a rare melphalan.15,19 entity more commonly found in male patients and is strongly as- sociated with HIV. An overstimulation of fibroblasts is present, lea- Cardiac involvement in scleromyxedema ding to dermal mucinosis.24 Hepatitis C infections have also been Cardiac
Load more

Recommended publications
  • Program Md Dermatology
    PROGRAM MD DERMATOLOGY (Revised with effect from 2015-2016 onwards) MD Dermatology 1 Contents Goal ....................................................................................................................................... 3 Objectives to be achieved by an individual at the end of 3 years of training ........................ 3 Programme outcomes ............................................................................................................ 4 Programme specific outcomes ............................................................................................... 5 Tentative schedule for three years of md training ................................................................. 5 Topics related to allied basic sciences ................................................................................... 7 Assessments/ examinations ................................................................................................. 12 Final examination ................................................................................................................ 12 Theory syllabus .................................................................................................................... 13 Fundamentals of cutaneous diagnosis- ................................................................................ 13 Tumours ............................................................................................................................... 14 Disorders of pigmentation ..................................................................................................
    [Show full text]
  • Cutaneous Mucinoses: an Overview Aefan Ul Bari
    Cutaneous mucinoses: an overview Aefan ul Bari Review Article Cutaneous mucinoses: an overview Arfan ul Bari PAF Hospital Sargodha Abstract The cutaneous mucinoses are a group of connective tissue disorders characterized by the deposition of mucin, either focally or diffusely, in the interstices of the dermis. The diseases may occur as a primary (metabolic) event or as a secondary (catabolic) process in certain dermatoses such as lupus erythematosus and dermatomyositis. Systemic abnormalities are seen with most of these disorders. A brief review and classification of the disorders included in this group is given here. Key words Cutaneous mucinoses, dermal mucinoses, mucin, connective tissue disorders. The cutaneous mucinoses are a which may be either dermal or follicular, heterogeneous group of diseases so named and into neoplastic-hamartomatous because of the variable amount of mucin mucinoses. Histopathologic diagnosis is accumulation in the skin or within the hair particularly difficult for dermal mucinoses follicle. Mucin is jelly-like acid and requires clinicopathologic correlation. mucopolysacharide (glycosaminoglycan) of Three histologic clues, namely the pattern of the ground substances and probably plays a mucin distribution (diffuse or focal), the role in extravascular exchange of level of mucin deposit in the dermis and metabolites. It is produced by fibroblasts some additional findings may help and is composed of hyaluronic acid bound to diagnosis. Follicular mucinoses have the heparin and chondroitin sulphate.1,2 We easiest
    [Show full text]
  • Discrete Papular Dermal Mucinosis with Hashimoto Thyroiditis: a Case Report
    Discrete Papular Dermal Mucinosis With Hashimoto Thyroiditis: A Case Report Ilgen Ertam, MD; Nezih Karaca, MD; Can Ceylan, MD; Alican Kazandi, MD; Sibel Alper, MD The cutaneous focal mucinoses are a group of neck. During this time, the patient was being followed connective tissue disorders characterized by by the rheumatology department for fibromyalgia and deposition of mucin found either focally or dif- connective tissue disorder. Because of the cutaneous fusely in the dermis. A 47-year-old woman pre- lesions, she was sent for dermatologic evaluation. sented with asymptomatic flesh-colored papules There was no history of preceding trauma or insect on the neck, inguinal area, intergluteal area, bites. The patient had undergone a subtotal thyroid- vulvar area, and extremities of 5 months’ dura- ectomy 21 years prior but had not used any thyroid tion. There was no history of preceding trauma or medication before she was referred to our clinic. insect bites. The patient had undergone a subto- During dermatologic examination, we found flesh- tal thyroidectomy 21 years prior but had not used colored, well-defined, smooth papules that measured any thyroid medication before she was referred to approximately 1.531 cm in size on the genital region, our clinic. Thyroid ultrasonographyCUTIS was consistent fingers, face, and scalp. The lesions extended from with Hashimoto thyroiditis. During dermatologic the labium majus and labium minus to the interglu- examination, flesh-colored, well-defined, smooth teal and coccygeal region (Figures 1–3). They also papules that measured approximately 1.531 cm appeared symmetrically at the level of the anterior in size on the genital region, fingers, face, and femoral region and on the dorsal and palmar areas of scalp were seen.
    [Show full text]
  • Self-Assessment Answers Cases 1 - 15
    Self-Assessment Answers Cases 1 - 15 Chairs: Richard Carr (UK) Rossitza Lazova (USA) Self Assessment Preçis XXXVIII Symposium of the International Society of Dermatopathology, Glasgow, 28-30 September 2017 Self-Assessment Answers Part 1 – Friday 29th September 2017 No Time First Name Surname Country Selected Cases Page 1 16:20 Mona Abdel-Halim Egypt Fluoroscopy: radiodermatitis 3 2 16:26 Asha Kubba India Papulonecrotic tuberculid 5 3 16:32 Doina Ivan USA Crystaloglobulinemia 6 4 16:38 Francisco Bravo Peru Gnathosmiasis 7 5 16:44 Jose Cardoso Portugal Acral persistent papular mucinosis 9 6 16:50 Agnes Carlotti France Anaplastic Kaposi's Sarcoma 11 7 16:56 Benedicte Cavalier-Balloy France Acantholytic extrammary genital Pagets disease 12 8 17:02 Heung Chong UK Plaque like myofibroblastic tumour of infancy 14 9 17:08 David Cassarino USA Mycoplasma induced rash & mucositis syndrome 15 (EM-like) 10 17:14 Reem El Bahtimi Dubai Cutaneous lymphadenoma 16 11 17:20 Lydia Essary USA Primary cutaneous coccidiodomycosis 18 12 17:26 Maxwell Fung USA Sclerodermoid GVHD w cutaneous amyloid 19 13 17:32 Anjela Galan USA Granuloma-annulare-like mets. Breast 20 14 17:38 Joerg Schaller Germany Randalls disease (non-amyloid light chains) 22 15 17:44 Katrin Kerl Switzerland Lavamisol induced occlusive vasculitis 24 2 Self Assessment Preçis XXXVIII Symposium of the International Society of Dermatopathology, Glasgow, 28-30 September 2017 Case 1 Mona R.E. Abdel-Halim, MD, Dip Dermpath (ICDP-UEMS) DIAGNOSIS FLOUROSCOPY INDUCED CHRONIC RADIODERMATITIS Clinical Summary: A 66-year-old male patient presented with a well defined square shaped indurated/sclerotic focally ulcerated plaque with atrophic areas on the right sub-scapular area of 2 years duration.
    [Show full text]
  • Reticular Erythematous Mucinosis: a Rare Cutaneous Mucinosis Amer Ali Almohssen1, Ragha Vasantha Suresh2,Robert A
    Acta Dermatovenerol Croat 2019;27(1):16-21 REVIEW Reticular Erythematous Mucinosis: A Rare Cutaneous Mucinosis Amer Ali Almohssen1, Ragha Vasantha Suresh2,Robert A. Schwartz2 1Dermatopathology, State University of New York Downstate Medical School & The Ackerman Academy of Dermatopathology New York City, NY, USA; 2Dermatology and Pathology, Rutgers New Jersey Medical School, Newark, New Jersey, USA Corresponding Author ABSTracT Reticular erythematous mucinosis (REM) is a rare form of pri- Amer Ali Almohssen, MD mary cutaneous mucinosis, most often involving the midline of the upper chest or back in middle-aged women. REM bears clinical and histopatho- Dermatopathology Fellow, State University logic resemblance to lupus erythematosus tumidus (LET), dermatomyosi- of New York tis, scleredema, and lichen myxedematosus. Early recognition and diagno- Downstate Medical School & The Ackerman sis of REM is particularly relevant to exclude the abovementioned diseases, as REM is more benign and has fewer systemic consequences. Academy of Dermatopathology New York City KEY WORDS: reticular erythematous mucinosis, lupus erythematous tu- New York midus, scleredema, dermatomyositis, lichen myxedematosus, papular mu- USA cinosis [email protected] Received: December 27, 2017 Accepted: February 9, 2019 INTRODUCTION The cutaneous mucinoses are a diverse group of pathologic resemblance to lupus erythematosus tu- disorders in which elevated levels of mucin are found midus (LET), dermatomyositis, scleredema, and pap- in the skin, predominantly in the dermis (1). The pri- ular mucinosis. However, recent evidence supports mary cutaneous mucinoses are characterized by mu- the recognition of REM as a distinct entity, based on cin deposition as the major histological feature, and its distinctive clinical and histologic features of this include scleromyxedema, scleredema, and lichen disorder.
    [Show full text]
  • Discrete Papular Mucinosis: a Rare Subtype of Lichen Myxedematosus Wongsiya Viarasilpa MD, Wareeporn Disphanurat MD
    198 Case report Thai J Dermatol, October-December, 2019 Discrete papular mucinosis: A rare subtype of lichen myxedematosus Wongsiya Viarasilpa MD, Wareeporn Disphanurat MD. ABSTRACT: VIARASILPA W, DISPHANURAT W. DISCRETE PAPULAR MUCINOSIS: A RARE SUBTYPE OF LICHEN MYXEDEMATOSUS. THAI J DERMATOL 2019; 35: 198-205. DIVISION OF DERMATOLOGY, DEPARTMENT OF MEDICINE, FACULTY OF MEDICINE, THAMMASAT UNIVERSITY, PATHUMTHANI, THAILAND. Lichen myxedematosus is a chronic, progressive idiopathic cutaneous mucinosis characterized by localized or generalized papular eruption of unknown etiology in which mucin deposition in the dermis is the distinctive histologic feature. The classification system was revised into three clinicopathological subsets, localized lichen myxedematosus, scleromyxedema and atypical forms of lichen myxedematosus. We report a rare subtype of lichen myxedematosus, discrete papular subtype, presented with papular eruption on the back, chest, face and neck. Histopathology showed focal mucin accumulation in upper and mid reticular dermis with scattered stellate fibroblasts among mucinous material, confirmed by Alcian blue staining. Her serum protein electrophoresis showed polyclonal immunoglobulin, serology for hepatitis C and HIV were negative and her thyroid function test was normal. She was diagnosed with localized forms of lichen myxedematosus, discrete papular subtype and was treated with an excellent response to topical corticosteroids and oral hydroxychloroquine combination therapy. Key words: lichen myxedematosus, papular mucinosis, skin-colored papules From: Division of dermatology, Department of Medicine, Faculty of Medicine, Thammasat University, Pathumthani, Thailand Corresponding author: Wareeporn Disphanurat MD, email: [email protected] Received: 2 April 2019 Revised: 23 September 2019 Accepted: 6 November 2019 Vol.35 No.4 Viarasilpa W and Disphanurat W 199 myxedematosus is a rare entity and has less prevalence than scleromyxedema4, 6-12.
    [Show full text]
  • Flesh-Colored Papular Eruption
    DERMATOPATHOLOGY DIAGNOSIS CLOSE ENCOUNTERS WITH THE ENVIRONMENT Flesh-Colored Papular Eruption Vanessa B. Voss, MD; Claudia I. Vidal, MD, PhD Eligible for 1 MOC SA Credit From the ABD This Dermatopathology Diagnosis article in our print edition is eligible for 1 self-assessment credit for Maintenance of Certification from the American Board of Dermatology (ABD). After completing this activity, diplomates can visit the ABD website (http://www.abderm.org) to self-report the credits under the activity title “Cutis Dermatopathology Diagnosis.” You may report the credit after each activity is completed or after accumu- lating multiple credits. A 48-year-old black man presented with a rash of 7 months’ duration that started on the face and spread to the body. He had extreme pruritus, increased stiffness in the hands and joints,copy and paresthesia. Physical examination revealed an eruption of 2- to 4-mm, flesh-colored papules with follicu- lar accentuation on the face, neck, bilateral uppernot extremities, back, and thighs. Do H&E, original magnification ×100. The best diagnosis is: a. infundibulofolliculitis b. interstitial granulomaCUTIS annulare c. papular mucinosis/scleromyxedema d. reticular erythematous mucinosis e. scleredema PLEASE TURN TO PAGE 362 FOR DERMATOPATHOLOGY DIAGNOSIS DISCUSSION From Saint Louis University, Missouri. Dr. Voss is from the School of Medicine. Dr. Vidal is from the Department of Dermatology. The authors report no conflict of interest. Correspondence: Claudia I. Vidal, MD, PhD, Department of Dermatology, Anheuser-Busch Institute, 4th Floor, Room 402, 1755 S Grand Blvd, St Louis, MO 63104 ([email protected]). WWW.CUTIS.COM VOLUME 98, DECEMBER 2016 361 Copyright Cutis 2016.
    [Show full text]
  • Acral Persistent Papular Mucinosis
    Journal of Pakistan Association of Dermatologists 2004; 14: 253-256. Case Report Acral persistent papular mucinosis: A rare variant of cutaneous mucinosis Arfan ul Bari, Muhammad Rashid*, Simeen ber Rahman** Dermatology Department, PAF Hospital Sargodha *ENT Department, PAF Hospital Sargodha ** Dermatology Department, Military Hospital, Rawalpindi, Pakistan Abstract Acral persistent papular mucinosis (APPM) is a distinctive form of dermal mucinosis not associated with systemic diseases. We report such a case in a sixty years old male who presented with few small papular lesions over both of his ears. These were managed successfully with excision and cryosurgery. Key words Papular mucinosis, acral persistent papular mucinosis, scleromyxedema, lichen myxedematosus Introduction rare, affects adults of both sexes equally and appears between ages 30 and 80. It is Papular mucinosis is one of the cutaneous chronic and may be progressive. The deposit diseases that presents as flesh- primary lesions are waxy, 2-to-4-mm, colored dermal papules mostly on the acral dome-shaped or flat-topped papules. parts of the body. There is confusion with Frequently, they may coalesce into regard to the terminology of this entity in plaques or appear in a linear array. Less the literature. Localized form of the frequently, urticarial, nodular, or disease has been called papular mucinosis sometimes annular lesions may be or lichen myxedematosus and generalized, appreciated. The dorsal aspect of the confluent papular forms with sclerosis are hands, face, elbows, and extensor portions known as scleromyxedema.1,2 Although of the extremities are most frequently papular mucinosis is frequently used as a affected. Mucosal lesions are absent.
    [Show full text]
  • (2) October 2011\Protocol Book\Program-Speaker Page-Oc
    Chicago Dermatological Society October 2011 Monthly Educational Conference Program Information Continuing Medical Education Certification and Case Presentations Wednesday, October 12, 2011 David Fretzin Lecture Conference Host: Department of Dermatology University of Illinois at Chicago Chicago, Illinois University of Illinois at Chicago C UIC Student Center West (SCW) - 828 S. Wolcott Ave., 2nd Floor Registration, lectures, slide viewing, lunch and committee meetings C UIC Outpatient Care Center, Dermatology Clinic - 1801 W. Taylor, Suite 3E Patient viewing only (no registration at this location! Protocol books will be available.) UIC parking – Use the Wood Street Parking Structure, 1100 S. Wood at the intersection of (Wood & Grenshaw, just south of the UIC Outpatient Care Center) See reverse side for detailed campus map From the Eisenhower Expressway, exit at Ashland/Paulina. Proceed south on Ashland to Taylor. Turn west on Taylor approximately two blocks to Wood St. Turn south on Wood for the entrance to the parking lot. Program Conference Locations Student Center West (SCW) – 828 S. Wolcott, 2nd Floor Dermatology Clinic, 1801 W. Taylor St., Suite 3E 8:00 a.m. Registration Opens Student Center West, 2nd floor Foyer 9:00 a.m. - 10:00 a.m. Resident Lecture – SCW Chicago Room A-C "Spectrum of CD30+ Lymphoproliferative Diseases" Samuel Hwang, MD, PhD 9:30 a.m. - 10:45 a.m. Clinical Rounds Patient & Poster Viewing Dermatology Clinic, Suite 3E Slide Viewing Student Center West, Room 213 A/B 11:00 a.m. - 12:15 p.m. General Session - SCW Chicago Room A-C FRETZIN LECTURE: "An Update on Th17 Cells in Psoriasis" Samuel Hwang, MD, PhD 12:15 p.m.
    [Show full text]
  • 2014 Slide Library Case Summary Questions & Answers With
    2014 Slide Library Case Summary Questions & Answers with Discussions 51st Annual Meeting November 6-9, 2014 Chicago Hilton & Towers Chicago, Illinois The American Society of Dermatopathology ARTHUR K. BALIN, MD, PhD, FASDP FCAP, FASCP, FACP, FAAD, FACMMSCO, FASDS, FAACS, FASLMS, FRSM, AGSF, FGSA, FACN, FAAA, FNACB, FFRBM, FMMS, FPCP ASDP REFERENCE SLIDE LIBRARY November 2014 Dear Fellows of the American Society of Dermatopathology, The American Society of Dermatopathology would like to invite you to submit slides to the Reference Slide Library. At this time there are over 9300 slides in the library. The collection grew 2% over the past year. This collection continues to grow from member’s generous contributions over the years. The slides are appreciated and are here for you to view at the Sally Balin Medical Center. Below are the directions for submission. Submission requirements for the American Society of Dermatopathology Reference Slide Library: 1. One H & E slide for each case (if available) 2. Site of biopsy 3. Pathologic diagnosis Not required, but additional information to include: 1. Microscopic description of the slide illustrating the salient diagnostic points 2. Clinical history and pertinent laboratory data, if known 3. Specific stain, if helpful 4. Clinical photograph 5. Additional note, reference or comment of teaching value Teaching sets or collections of conditions are especially useful. In addition, infrequently seen conditions are continually desired. Even a single case is helpful. Usually, the written submission requirement can be fulfilled by enclosing a copy of the pathology report prepared for diagnosis of the submitted case. As a guideline, please contribute conditions seen with a frequency of less than 1 in 100 specimens.
    [Show full text]
  • UC Davis Dermatology Online Journal
    UC Davis Dermatology Online Journal Title Acral Persistent Papular Mucinosis: is it an under-diagnosed disease? Permalink https://escholarship.org/uc/item/0n04458v Journal Dermatology Online Journal, 20(3) Authors Alvarez-Garrido, Helena Najera, Laura Garrido-Rios, Anastasia Alejandra et al. Publication Date 2014 DOI 10.5070/D3203021757 License https://creativecommons.org/licenses/by-nc-nd/4.0/ 4.0 Peer reviewed eScholarship.org Powered by the California Digital Library University of California Volume 20 Number 3 March 2014 Photo Vignette Acral Persistent Papular Mucinosis: is it an under-diagnosed disease? Helena Alvarez-Garrido, Laura Najera, Anastasia Alejandra Garrido-Ríos, Susana Córdoba-Guijarro, María Huerta-Brogeras, Marta Aguado-Lobo, Jesus Borbujo Dermatology Online Journal 20 (3): 10 Hospital Universitario de Fuenlabrada, Department of Dermatology, Spain Correspondence: Helena Alvarez-Garrido Hospital Universitario de Fuenlabrada [email protected] Abstract Acral persistent papular mucinosis is a subtype of localized lichen myxedematosus. It presents as acrally located papules with a benign, but persistent course. It is a scarcely reported disease. We present a female with both the clinical and histopathological described criteria Introduction Acral persistent papular mucinosis (APPM) is a type of papular mucinosis. Also known as lichen myxedematosus or scleromyxedema, this is a group of diseases is characterized by mucin deposits in skin. We can classify these diseases into clinicopathological types. The first type is a generalized/sclerodermoid form, also called scleromyxedema, with systemic manifestations. The second is a localized form without systemic involvement. There are five subtypes: discrete papular, nodular, self-healing papular mucinosis, papular mucinosis of infancy, and acral persistent papular mucinosis.
    [Show full text]
  • Cutaneous Mucinosis Case with Characteristic Lion Face Appearance Selami Aykut Temiz1 , Recep Dursun1 , Arzu Ataseven1 , İlkay Özer1 , Sıddıka Fındık2
    DOI: 10.5152/cjms.2018.527 Case Report Cutaneous Mucinosis Case with Characteristic Lion Face Appearance Selami Aykut Temiz1 , Recep Dursun1 , Arzu Ataseven1 , İlkay Özer1 , Sıddıka Fındık2 1Department of Dermatology, Necmettin Erbakan University Meram School of Medicine, Konya, Turkey 2Department of Pathology, Necmettin Erbakan University Meram School of Medicine, Konya, Turkey ORCID IDs of the Author: S.A.T: 0000- 0003-4878-0045; R.D.: 0000-0002-1279-574X; A.A.: 0000-0001-5372-0712; İ.Ö.: 0000-0001-6170- 0930; S.F.: 0000-0002-3364-7498 Cite this article as: Temiz SA, Dursun R, Ataseven A, Özer İ, Fındık S. Cutaneous Mucinosis Case with Characteristic Lion Face Appearance. Cyprus J Med Sci 2018; 3: 117-9. ABSTRACT Cutaneous mucinosis includes a heterogeneous group of skin diseases characterized by the deposition of mucin in the interval of the dermis. Mucin is a protein ordinarily found as part of the dermal connective tissues. Mucin is a mucopolysaccharide produced by mast cells and fibroblasts and includes hyaluronic acid and sulfated glycosaminoglycans. Because hyaluronic acid holds water, in disease states where mucin production is increased, the dermal connective tissue swells, which is defined as myxedematous. Systemic mucin deposition may include systemic involvement; monoclonal gammopathy or paraproteinemia have been detected in the large majority (83.2%) of scleromyxedema cases. A 50-year-old female presented with pruritic, flesh-colored papules spread throughout the body. She also exhibited a characteristic lion face appearance. These lesions had first appeared 18 months before in the neck. The protein electrophoresis and bone marrow biopsy of the patient were normal.
    [Show full text]