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Clinical Presentation of Mosaic Manifestations of Neurocutaneous Syndrome

Conference Paper · August 2018

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Speaker: Proceeding Book of The 23th Regional Conference of Dermatology Incorporating with the 16th Annual Scientific Meeting of the Indonesian Society

  • f Dermatology and Venereology

August 8th – 11th 2018 Grand City Convex, Surabaya - Indonesia Perhimpunan Dokter Spesialis Kulit dan Kelamin Indonesia (PERDOSKI) Bekerja sama dengan League of Asean Dermatological Societies; Universitas Airlangga ; Rumah Sakit Daerah Dr. Soetomo dan Ikatan Dokter Indonesia

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Speaker: Proceeding Book The 23th Regional Conference of Dermatology Incorporating with the 16th Annual Scientific Meeting of the Indonesian Society

  • f Dermatology and Venereology

Diterbitkan oleh: Perhimpunan Dokter Spesialis Kulit dan Kelamin Indonesia (PERDOSKI) Bekerja sama dengan League of Asean Dermatological Societies; Universitas Airlangga ; Rumah Sakit Daerah Dr. Soetomo dan Ikatan Dokter Indonesia

Reviewer :

  • DR. Dr. M. Yulianto Listiawan, Sp.KK(K), FINSDV, FAADV
  • DR. Dr. Cita Rosita Sigit Prakoeswa, Sp.KK(K), FINSDV, FAADV
  • Prof. DR. Dr. Saut Sahat Pohan,Sp.KK(K), FINSDV, FAADV
  • Prof. DR. Dr. Indropo Agusni, SpKK(K), FINSDV, FAADV
  • Dr. Dian Ardiana, Sp.KK, FINSDV
  • Dr. Agnes Sri Siswati, SpKK(K), FINSDV, FAADV
  • Dr. Danang Triwahyudi, Sp.KK, FINSDV, FAADV
  • Dr. Adri Dwi Prasetyo, Sp.KK

Editor :

  • Dr. Irmadita Citrashanty, Sp.KK
  • Dr. Maylita Sari, Sp.KK
  • Dr. Menul Ayu Umborowati, Sp.KK
  • Dr. Renata Prameswari, Sp.KK

Bambang Agustono Roesmaydita Noernaning Mentari A.md Kep,S.Kep,Ns ISBN : 978 - 602 - 50061 - 5 - 9 Cetakan : Pertama, September 2018 Dicetak : Percetakan Wahana Ilmu

  • Jl. Kali Kepiting No. 97 Surabaya

Isi diluar tanggung jawab percetakan

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018

iii

alaalaik arohatllahi abarokath Sala Seahtera ll rie to o raie an ratite to o liht or i rae o that thi eent an be hel oothl he r eional onerene o Deratolo iantralian Inorporatin ith he th nnal Sientii eetin o the Inoneian Soiet o Deratolo an enereolo hel in ran it

  • ne an eial alt o irlana nierit SrabaaInoneia on t th th

hi biannal onerene i the oiial onre o the eae o S Deratoloial Soietie DS opriin the national eratoloial oietie o Inoneia Sinapore hailan alaia hilippine ietna an aboia hi eent i inorporatin ith th nnal Sientii eetin o Inoneian Soiet o Deratolo an enereolo the biet ientii eratoloit eetin in Inoneia he onerene i epete to ra to attenee ith partiipation ro Inoneian an ontrie o the iaaii reion inlin hina Inia on on an aian hee o the onerene i nhanin iene ae Deratolo ratie in lobaliation ra he onerene ai i to enhane eratoloial pratie bae on bet eiene an brin eratoloial reearhe into linial pratie he onerene alo ai to pate the latet eelopent in eial an rial eratolo nre paper are preente in thi

  • nerene on topi in an ie h a eial ethi an eation alleri an

atoinolo kin ieae tropial inetion kin ieae eal tranitte inetion kin tor oeti eratolo eratorer ener bae eie in eratolo an o on reenter oe ro all oer Inoneia ia the S the an alo ropean ontrie he oittee ol like to thank all preenter an partiipant or the reat ontribtion to thi

  • nerene e alo ol like to all area or enero pport an ollaboration to e thi

eent he oittee alo apoloie i there are an inienie rin the preparation proe an the onerene e hope thi reat ientii eent ill ontribte to the aaneent o eratoloenereolo in the orl

Srabaa l th he oittee o the th eional onerene o Deratolo Inorporatin ith the th nnal Sientii eetin o the Inoneian Soiet o Deratolo an enereolo

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 iv

ORGANIZING AND SCIENTIFIC COMMITTEE 23rd RCD - 16th PIT PERDOSKI 2018

PATRON :

  • Minister of Health Indonesia
  • President of Indonesian Medical Association
  • Rector of Airlangga University

ADVISORY :

  • Mayor of Surabaya
  • President and Excecutive Board of LADS
  • President and Excecutive Board of Indonesian Society Dermatology and Venereology
  • Director of Dr. Soetomo Hospital Surabaya
  • Dean of the Medical Faculty of Airlangga University

BOARD OF COMMITTEE CHAIRPERSON :

  • Dr. Syarief Hidayat, Sp.KK, FINSDV, FAADV

SECRETARY GENERAL :

  • Dr. Srie Prihianti, PhD, Sp.KK, FINSDV, FAADV

ORGANISING COMMITTEE CHAIRPERSON :

  • DR. Dr. M. Yulianto Listiawan, Sp.KK(K), FINSDV, FAADV

VICE-CHAIRPERSON :

  • DR. Dr. Cita Rosita S.Prakoeswa, Sp.KK(K), FINSDV, FAADV

ORGANIZING SECRETARY :

  • Dr. Ni Putu Susari Widianingsih, Sp.KK, FINSDV, FAADV

VICE-SECRETARY :

  • Dr. Hanny Nilasari, Sp.KK, FINSDV, FAADV
  • Dr. Ary Widhyasti, M.Kes, Sp.KK, FINSDV

SECRETARIAL AND REGISTRATION COORDINATOR : Dr. Linda Astari, Sp.KK, FINSDV SECRETARIAL :

  • Dr. Astindari, Sp.KK
  • Dr. Dinar Witasari, Sp.KK

Bambang Agustono REGISTRATION :

  • Dr. Septiana Widyantari, Sp.KK
  • Dr. Yuri Widia, Sp.KK
  • Dra. Eka Marta Diana

TREASURER :

  • Dr. Trisniartami Setyaningrum, Sp.KK, FINSDV

VICE-TREASURER :

  • Dr. Inge Ade Kristanti, SpKK, FINSDV, FAADV

VICE –TREASURER II :

  • Dr. Kurniati, Sp.KK

FUND RAISING COORDINATOR :

  • Dr. Andreas Widiansyah,Sp.KK, FINSDV, FAADV

MEMBERS :

  • Dr. Sri Ellyani, Sp.KK, FINSDV, FAADV
  • Dr. Abraham Arimuko, Sp.KK, FINSDV, FAADV
  • Dr. Herliani M Purba, Sp.KK, FINSDV
  • Dr. Jasmin Thalib, Sp.KK
  • Dr. Firdausi Nurharini, Sp.KK

FOOD COORDINATOR :

  • Dr. Trisiswati Indranarum, Sp.KK, FINSDV

MEMBERS :

  • Dr. Noormaini, Sp.KK
  • Dr. Desy Hinda Pramita, Sp.KK
  • Dr. Renata Mayangsari, Sp.KK

SCIENTIFIC COORDINATOR :

  • Dr. Dian Ardiana, Sp.KK, FINSDV

MEMBERS :

  • Prof. Dr. Jusuf Barakbah, SpKK(K), FINSDV, FAADV
  • Prof. DR. Dr. Saut Sahat Pohan,Sp.KK(K), FINSDV, FAADV
  • Prof. DR. Dr. Indropo Agusni, SpKK(K), FINSDV, FAADV
  • Dr. Agnes Sri Siswati, SpKK(K), FINSDV, FAADV
  • Dr. Danang Triwahyudi, Sp.KK, FINSDV, FAADV
  • Dr. Adri Dwi Prasetyo, Sp.KK
  • Dr. Irmadita Citrashanty, Sp.KK
  • Dr. Menul Ayu Umborowati, Sp.KK
  • Dr. Renata Prameswari, Sp.KK
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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018

v

  • dr. Densy Violina
  • dr. Abdul Karim
  • dr. Tjokorde Istri Nindya V
  • dr. Dyatiara Devy R
  • dr. Ade Fernandes
  • dr. Karina Dyahtantri P
  • dr. Marissa Astari R
  • dr. Bernadya Yogatri A
  • dr. Aprilin Krista D
  • dr. Farah Meriana F
  • dr. Silvani Geani
  • dr. Made Putri Hendaria
  • dr. Arisia Fadila
  • dr. Citra Dwi H
  • dr. Brigita Ika R
  • dr. Natalia Tanojo
  • dr. Emma Hidayati S
  • dr. Anindia Indraswari

FREE COMMUNICATION COORDINATOR MEMBERS :

  • Dr. Sawitri, Sp.KK(K), FINSDV, FAADV

:

  • Prof. Dr. Hari Sukanto, SpKK(K), FINSDV, FAADV
  • Dr. Evy Ervianti, Sp.KK(K), FINSDV, FAADV
  • Dr. David Sudarto Oeiria, Sp.KK, FINSDV, FAADV
  • Dr. Novianty Riski Reza, Sp.KK
  • Dr. Medhi Denisa A, Sp.KK
  • Dr. Sylvia Anggraini, Sp.KK

WORKSHOP COORDINATOR MEMBERS :

  • Dr. Maylita Sari, Sp.KK

:

  • Prof. Dr. Sunarko Martodiharjo,Sp.KK(K), FINSDV, FAADV
  • Dr. Fitri Abdullah Jawas, Sp.KK
  • Dr. Diana Tri Ratnasari, Sp.KK
  • Dr. Dhita Karina, Sp.KK
  • Dr. Lunni Gayatri, Sp.KK
  • dr. Densy Violina
  • dr. Abdul Karim
  • dr. Tjokorde Istri Nindya V
  • dr. Dyatiara Devy R
  • dr. Ade Fernandes
  • dr. Karina Dyahtantri P
  • dr. Marissa Astari R
  • dr. Bernadya Yogatri A
  • dr. Aprilin Krista D
  • dr. Farah Meriana F
  • dr. Silvani Geani
  • dr. Made Putri Hendaria
  • dr. Arisia Fadila
  • dr. Citra Dwi H
  • dr. Brigita Ika R
  • dr. Natalia Tanojo
  • dr. Emma Hidayati S
  • dr. Anindia Indraswari

PRE-RCD SYMPOSIUM COORDINATOR MEMBERS :

  • Dr. Hasnikmah Mappamasing, Sp.KK

:

  • Dr. Damayanti, Sp.KK, FINSDV
  • Dr. Marina Rimadhani, Sp.KK
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SLIDE 8

ORGANIZATION and STUDY GROUP COORDINATOR :

  • Dr. Fajar Waskito, M. Kes, Sp.KK, FINSDV, FAADV

MEMBERS :

  • DR. Dr. Afif Nurul Hidayati, Sp.KK, FINSDV
  • Dr. Iskandar Zulkarnain, Sp.KK(K), FINSDV, FAADV
  • Dr. Ary Widhyasti,M.Kes, Sp.KK, FINSDV

EXHIBITION AND EQUIPMENT COORDINATOR :

  • Dr. Bagus Haryo Kusumo, Sp.KK

MEMBERS :

  • Dr. Lukman Ariwibowo, Sp.KK
  • Dr. Riski Haris , Sp.KK

CEREMONIAL, CULTURAL NIGHT & TOUR PROGRAMS COORDINATOR :

  • Dr. Evy Ervianti, Sp.KK(K), FINSDV, FAADV

MEMBERS :

  • Dr. Abraham Arimuko, Sp.KK, FINSDV, FAADV
  • Dr. Linda Astari, Sp.KK, FINSDV

CEREMONIAL COORDINATOR MEMBER :

  • Dr. Maria Ulfa, Sp.KK

:

  • Dr. Asri Bindusari, Sp.KK

CULTURAL NIGHT COORDINATOR MEMBER :

  • Dr. Rahmadewi, Sp.KK, FINSDV

:

  • Dr. Dwi Murtiastutik, Sp.KK, FINSDV, FAADV

TOUR COORDINATOR MEMBER :

  • Dr. Myrna Safrida, Sp.KK

:

  • Dr. Raphael Gratiano, Sp.KK, FINSDV

SOCIAL EVENT COORDINATOR MEMBERS :

  • Dr. Ita Puspita Dewi, Sp.KK, FINSDV

:

  • Dr. Ika Soelistina, Sp.KK
  • Dr. Hasrulliana Ningsih W, Sp.KK
  • Dr. Pramita Ariyanti, Sp.KK

TRANSPORTATION & ACOMODATION COORDINATOR : Dr. Diah Mira Indramaya, Sp.KK, FINSDV MEMBERS :

  • Dr. Rully Setia AD, Sp.KK, FINSDV
  • Dr. Agustina Tri P, Sp.KK

SECURITY AND MEDICAL SERVICE COORDINATOR : Dr. Eko Riyanto, Sp.KK MEMBER :

  • Dr. Prasti Adhi, Sp.KK

PUBLICATION, MEDIA & PROMOTION and DOCUMENTATION COORDINATOR :

  • Dr. Nurfithria Ika Putri, Sp.KK

MEMBERS :

  • Dr. Kinanti Prabawaningrum, Sp.KK
  • Dr. Diah Nugrahaeni, Sp.KK

23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 vi

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 vii Table of Content ................................................................................................ ………………………………………. ii

  • iii

iv Preface ……………………………………………………………………………………………………………….. Organizing and Scientific Committee 23rd RCD-PIT PERDOSKI 2018 …………………………………………… GLOBAL AND BASIC KNOWLEDGE 1. Global Health Security The Dermatovenereologist Role In Shaping The Better World Iwan Trihapsoro ………………………………………………………………………………………………… 1 2. General Considerations of Medical Ethics Agus Purwadianto………………………………………………........................................................................... 2 3. Evidence ased edicine in ealth are eform and ost ontrol ra Implication for Dermatology and Venerology Cita Rosita Sigit Prakoeswa …………………………………………………………………………………….. 3 4. Continuing Dermatologic Education for Dermatologists Henry Foong Bon Bee …………………………………………………………………………………………... 8 5. New Understanding of Skin Biology Endi Novianto ……………………………………………………………………................................................ 9 6. Structure & Function of he Skin ranslation of Skin iology Into herapies & Improved atients are Retno Widowati Soebaryo……………………………………………………………………………………….. 12 7. The ole of Vitamin D in Skin Diseases Endi Novianto ………………………………………………………………………………………………….... 15 ALLERGY AND IMMUNOLOGY 8. Basic Understanding of Innate Immunity and Adaptive Immune System Retno Widowati Subaryo……………………………………………………………………………………….... 19 9. Immune System Alterations to Treat Inflammatory Skin Diseases Oki Suwarsa…………………………………………………………………………………………………….... 21 10. T-Cell and B-Cell Modulation in Dermatologic herap Hardyanto Soebono ……………………………………………………………………………………………... 25 11. Chronic ruritus: ossible auses and echanism Septiana Widyantari ……………………………………………………………………………………….......... 28 12. Moisturizer Selection in hronic ruritus Siti Aisyah Boediarja.............................................................................................................................................. 33 13. The ole of oisturizer ombination of Ceramide, Menthol, and Polidocanol ream in ild topic Dermatitis Cita Rosita Sigit Prakoeswa……………………………………………................................................................ 37 14. Management of Severe topic Dermatitis in Adult Kiran Godse …………………………………………………………………………………………………….. 40 15. Different echanism of Immune esponse in educing llergic eaction owards Lactobacillus Plantarum IS – 10506 Supplementation in Mice odel and in uman Subjects Inggrid S. Surono ……………………………………………………………………………………………….. 44 16. Irritant Contact Dermatitis Due to Occupational Agent Rohna Rdizwan …………………………………………………………………………………………………. 48 17. Moisturizer ole in ccupational ontact Dermatitis Fajar Waskito …………………………………………………………………………………………………… 51 18. Pemphigus and Pemphigoid: Evidence Based Updates Daisuke Tsuruta …………………………………………………………………………………………………. 54 19. Treatment lans for Pemphigus and Pemphigoid Based on Current Evidences I.G.A.A. Elis Indira ……………………………………………………………………………………………… 55

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018

viii 20. Side ffect of iological gent for soriasis reatment Wong Su-Ni…………………………………………………………………………............................................. 59 21. Cytokine Targeted Therapies in Psoriasis Sudigdo Adi …………………………………………………………………………………………………….. 60 22. Psoriasis : Insight into reatment Decisions Tjut Nurul Alam Jacoeb ………………………………………………………………………………………… 61 23. Diagnosis and Treatment of The Scalp and Nail Psoriasis Benny E Wiryadi……………………………………………………………………………………………….. 62 24. Immunopathogenesis Role of Cytokines in Proriasis Winston T. Warouw …………………………………………….......................................................................... 63 25. The ole of iologic gent of TNF ntagonist and IL12/23 Inhibitors in Psoriasis reatment Irma D. Roesyanto…………………………………………………….................................................................. 67 26. Use of loporine to reat soriasis Made Wardhana ………………………………………………………………………………………………… 71 27. Genetics and he otential for redictive ests in dverse Drug eactions Endang Sutedja ………………………………………………............................................................................... 72 28. SJS/TEN: What’s New Chung Wen Hung ……………………………………………………………………………………………….. 76 29. Cuntaneous dverse Drug eactions : Drug eaction with osinophilia and Systemic Symptoms (DSS) Syndrome : npredictable and ife-hreatening ultisystem Involvement Robert A. Schwartz ……………………………………………………………………………………………… 77 DERMATOMYCOLOGY 30. Epidemiology of Superficial Dermatomycosis in Indonesia Made Swastika ………………………………………………………………………………………………….. . 78 31. Recent Diagnostic ools in ungal Skin Infection Kusmarinah Bramono ………………………………………………………………………………………… … 79 32. Who Says inea apitis is Difficult? Rataporn Ungpakorn ………………………………………………………………….......................................... 83 33. Prognostic ure ate of Malasseziafolicullitis in Indonesia Pieter Levinus Suling……………………………………………………………………………………………. 84 34. Dystrophic nguium ften isdiagnose as Onychomycosis: ow to reat hem : Update Sunarso Suyoso ………………………………………………………………………………………………….. 87 35. Tinea Genitalis Stephan Lautenschlager …………………………………………………………................................................. 91 36. Primary Candida nychomycosis-does It xist? Steven K.W Chow……………………………………………………………………………………………….. 92 37. Impact of Malassezia Species on Psoriasis, Eczema, Acne, Seborrheic Dermatitis, Pruritus And Therapeutic Approaches Sandra Widaty ………………………………………………………………………………………………….... 93 38. Evidence Based Treatment lan of Onychomycosis Evy Ervianti ……………………………………………………………………………………………………... 97 LEPROSY 39. Historical pproah to odern eprosy radication olicy in Japan-a naental uman ight

  • f atients and amilies was iolated by he overnment

Shinzo Izumi ……………………………………………………………………………………………………. 104 40. Preventing erve unction Impairment in Leprosy William Faber ………………………………………………………………………………………………….... 106 41. Post xposure hemoprophylaxiz in eprosy Wim van Brakel ………………………………………………………………..................................................... 108

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 ix 42. The fficacy of opical uman mniotic embrane Stem ell – onditioned edium n hronic lantar lcer of eprosy Cita Rosita Sigit Prakoeswa …………………………………………………………………………………….. 111 43. Characterization Of Stromal Vascular Fraction From Treated Leprosy Patients: A Preliminary Report Sondang P. Sirait ……………………………………………………………………………………………….. 119 44. Lucio Phenomene and Mycobacterium lepromatosis in Indonesia Indropo Agusni …...…………………………………………………………………………………………….. 123 OTHER SKIN INFECTION 45. Bacterial Skin Infection : Guideline for he ontrol and revention of MRSA in Dermatology Kuntaman…………………………………………………………………........................................................... 124 46. Streptococcus Infection: Resistent and Treatment Guideline Moerbono Moechtar…………………………………………………………………........................................... 126 47. Streptococcal Infection : Immunopathogenic Factor and Therapy Tantari S.H.W. ……………………………………………………………………………................................... 130 48. Indonesian Guidelines for Cutaneous Tuberculosis Ennesta Asri …………………………………………………………………………………………………….. 133 49. The Singapore Experience in Managing Non Tuberculous Mycobacteria Skin Infection Ang Chia Chun …………………………………………………………………………………………………. 137 50. Atypical Mycobacterial Infections: Difficult Diagnosis and Therapy William Faber …………………………………………………………………………………………………... 138 51. Frambusia (Yaws) Menul Ayu Umborowati ………………………………………………………………………………………... 140 52. Cutaneous and Mucocutaneous Leishmaniasis Bagus Haryo Kusumaputra ……………………………………………………………………………………... 144 53. Cutaneous Anthrax Marlous Grijsen ………………………………………………………………………………………………… 148 54. Biological spect on utaneous Disorder elated Human Herpes Viruses (HHV) Prasetyadi Mawardi ……………………………………………………............................................................... 149 55. Novel Anti Herpes Viruses Drug: Efficacy and effectiveness

  • A. A. Gede Putra Wiraguna…………………………………………………………........................................... 150

56. Human Herpes Virus Vaccine for Future Qaira Anum ……………………………………………………………………………………………………... 153 57. Diagnostic tools for HHV and Varicella Zoster Virus Hans Lumintang ………………………………………………………………………………………………… 158 58. Post Herpetic Neuralgia (PHN) : How do e anage hat?

  • Dr. Nurwestu Rusetiyanti, SpKK, M.Kes………………………………………………………………….......... 159

59. The Varicella - Zoster Virus Vaccines : Benefits and Risks Afif Nurul Hidayati ……………………………………………………………................................................... 162 60. Scabies : Basic Knowledge Anis Irawan Anwar……………………………………………………………………………………………… 166 61. Management of Scabies and Its elation to nti-Scabies Drug esistance Agnes Sri Siswati …………………………………………………………........................................................... 168 SEXUAL TRANSMITTED DISEASES 62. Global rends and linical resentations of Sexually ransmitted Infection Stephan Lautenschlager ……………………………………………………………………………………….... 172 63. Syphilis and HIV Satiti Retno Pudjiatii…………………………………………………………………………………………….. 173

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018

x 64. Rapid Test: Diagnosis & Treatment Dwi Murtiastutik …………………………………………………………............................................................ 177 65. Bacterial resistence in Gonorrhoea infection Jusuf Barakbah, …………………………………………………………………………………………………. 181 66. Skin and mucosal disorder related to HIV infection Jusuf Barakbah ………………………………………………………………………………………………….. 186 67. HPV testing & contributing of HPV vaccine for HPV infection Wresti Indriatmi ………………………………………………………………………………………………… 190 68. Management of HPV Infection and Immunomodulator roles Nurdjannah Jane …………………………………………………………............................................................ 193 69. Genital Herpes in pregnancy Hanny Nilasari ………………………………………………………………………………………………….. 196 70. Open Clinical trial of Herpes Simplex virus infection, Varicella soster virus infection and Condyloma accuminata. Hans Lumintang ………………………………………………………………………………………………… 198 71. Molluscum Contagiosum In Immunocompromised Patient And Its Management Izazi Hari Purwoko …………………………………………………………………............................................ 199 PEDIATRIC DERMATOLOGY 72. Clinical Persentation of Mosaic Manifestations of Neurocutaneous Syndrome Retno Danarti …………………………………………………………………………………………………… 203 73. The Relation etween utrition and ediatrics Skin ondition Sawitri …………………………………………………………………………………………………………... 207 74. Skin Care of The Newborn : Do’s and Dont’s Mirawati Setyorini ……………………………………………………………………………………………… 210 75. Recent Issues in Epidermolysis Bullosa in hildren: Wound Care pproach Inne Arline Diana ……………………………………………………….............................................................. 214 76. Staphylococcal Scalded Skin Syndrome: How to Differentiate with ther Similar Dermatosis Iskandar Zulkarnain …………………………………………………………………………………………...... 218 GERIATRIC DERMATOLOGY 77. Basic nderstanding of Skin ondition in Elderly Shannaz Nadia Yusharyahya …………………………………………………………………………………… 222 78. Update in Skin Immunosenescence in Geriatric opulation Lili Legiawati …………………………………………………………................................................................ 225 79. Management of erotic Skin in Elderly Lili Legiawati …………………………………………………………………………………………………… 229 80. Difficult Case: Management of Elderly Chronic Pruritus Yulia Farida Yahya……………………………………………………………………….................................... 232 81. Treatment of on-elanoma Skin Cancer in Geriatric Patients Tran Hau Kang ………………………………………………………………….................................................. 235 COSMETIC DERMATOLOGY AND LASER 82. Clinical Science of Medium Depth Peels Trisniartami Setyaningrum ……………………………………………………………....................................... 237 83. Combination eeling for Rejuvenation and esthetic Nelva Karmila Yusuf …………………………………………………………………………………………… 241 84. Efficacy And Possible Complication of Medium Depth Peeling in Asian Skin Reti Hindritiani………………………………………………………………………………………………….. 244 85. Acne Management Guidelines in Indonesia Sjarif M. Wasitaatmadja………………………………………………………………………………………… 247

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 xi 86. Glycemic Index and Acne Puguh Riyanto ………………………………………………………................................................................... 248 87. Treatment of Post Acne Lesion, yperpigmentation and Atrophic Scar Abraham Arimuko ………………………………………………………............................................................ 249 88. State of The Art Laser Scar Therapy

  • M. Yulianto Listiawan ……………………………………………...................................................................... 250

89. Update in Diagnosis of Melasma Irma Bernadette S. Sitohang …………………………………….......................................................................... 252 90. Topical Tranexamic Acid Versus Hydroquinone as Whitening Agent Anis Irawan Anwar……………………………………………………………………………………………… 254 91. Oral Glutathione As A Skin Whitening Agent: A Randomized, Double-Blind, Placebo-Controlled, Multicenter Clinical Trial Irma Bernadette S. Sitohang …………………………………….......................................................................... 257 92. Laser in elasma: Yes or No Ni Putu Susari …………………………………………………………………………………………………... 259 93. Profile of Vitiligo Patients Treated at The Outpatient Clinic of The Department

  • f Dermatovenereology Cipto Mangunkusumo Hospital, Jakarta 2015 – 2017

Lis Surachmiati Suseno ………………………………………………………………………………………… 262 94. The Role of Stem Cell in Vitiligo Dian Ardiana ………………………………………………………………………............................................. 265 95. Phototherapy for Vitiligo Ni Putu Ary Widyasti Bandem ……………………………………………......................................................... 269 96. Diagnostic hallenge in Hair loss Sinta Murlistyarini …………………………………………………………………………………………....… 271 97. Hair Care for Hairloss Patient Rahmadewi ……………………………………………………………………………....................................... 274 98. Minimally Invasive ombined reatment for he ging ace David Sudarto Oeiria ………………………………………………………….................................................... 278 99. Non Invasive Body Contouring: Heat vs. Cold. vs. Injections Aryani Soedharmono ………………………………………………………………………………………...…. 279

  • 100. Male Rejuvenation Procedures (Non-Invasive Procedures)

Ni Putu Susari W …………………………………………………….….............................................................. 281

  • 101. Choosing Best Combination Procedures for he Male Patients

Edwin Juanda …………………………………………………………………………………………………… 284

  • 102. Latest News and Research on Botox

Sri Ellyani ………………………………………………………………............................................................. 286

  • 103. Topical Herbal Treatment for Rejuvenation

Maylita Sari………………………………………………………………............................................................ 287

  • 104. Combination of CO2 Laser Fractional and Growth Factor on Acne Scar Treatment
  • M. Yulianto Listiawan ………………………………………………................................................................... 292

TUMOR AND SKIN SURGERY

  • 105. Role of dermoscopy : Beyond Early Skin ancer Detection

Larisa Paramitha ………………………………………………………………………………………………... 297

  • 106. MOHS icrographic Surgery for Skin umor

Adhimukti T. Sampurna ………………………………………………………………………............................ 300

  • 107. Kaposi's Sarcoma 2018 : A Continuing Conundrum

Robert A. Schwart ……………………………………………………………………………………………… 303

  • 108. Asian Blepharoplasty

Gunawan Budisantoso ………………………………………………………………………………………….. 304

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  • 109. Liposuction in Small rea

Susanti Budiamal, ……………………………………………………………...................................................... 307

  • 110. Update on athophysiology on ound and Scar anagement

David S. Perdanakusuma………………………………………………………………………………………... 309

  • 111. Facial Flap Surgery

Budi Harjandi W. ……………………………………………………………………………………………….. 313

  • 112. Patophysiology of Wound Healing Process
  • M. Yulianto Listiawan ……………………………………………….................................................................. 316

DERMATOPATHOLOGY

  • 113. Role of Immunopathology in Dermatopathology

Risye Arisanty ………………………………………………………………………………………………….. 318

  • 114. Recent Development on Cutaneous Blistering Disease: The Bridge Between Dermatopathology

and Molecular Biology Noppadon Noppakun …………………………………………………………………………………………… 319

  • 115. Dermatopathologic Diagnosis of soriasis and ther ritropapuloskuamous Diseases

Willy Sandhika………………………………………………………………………………………………….. 320

  • 116. Neoplasm in Dermatopathology Perspective: How To Evaluate and Distinguish Cutaneous

Tumors Including Melanocytic, Squamous and Lymphoid from Mimics Selviyanti Padma ……………………………………………………………………………………………….. 324

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 203

Clinical Presentation of Mosaic Manifestations of Neurocutaneous Syndrome

Retno Danarti Department of Dermatology and Venereology Faculty of Medicine, Public Health, and Nursing Universitas Gadjah Mada/ Dr. Sardjito Hospital Gedung Radiopoetro lantai 3, Jalan Farmako Sekip, Yogyakarta 55281 Phone +62818269272, Fax: +62274560700; Email: danarti@ugm.ac.id Abstract Background: The skin and the central nervous system are derived from a common embryonic origin during fetal

  • development. Several neurocutaneous syndrome may manifest in mosaic patterns with cutaneous involvement. Hence, the

skin can be a window into the central nervous system. Dermatologists are in a privileged position when studying human mosaicism, and can help early recognition in the diagnosis of neurologic disease. This article focuses on several important neurocutaneous syndromes with clinical mosaic manifestation. Aim: To study the the clinical variability of mosaic manifestations in patients diagnosed with neurocutaneous syndrome in Sardjito hospital Yogyakarta Indonesia between 2007-2017. Result: In period of 2007-2017 we found 68 cases of neurocutaneous syndrome with mosaic skin manifestation. The patterns of cutaneous mosaicism in neurocutaneous disorders encountered in our dermatology polyclinic were lines of Blaschko (in incontinentia pigmenti, Schimmelpenning syndrome including phacomatosis pigmentokeratotica, pigmentary mosaic of the Ito type); checkerboard pattern (Sturge-Weber syndrome); patchy pattern without midline separation (giant congenital melanocytic nevus/ neurocutaneous melanosis); lateralization pattern (Congenital hemidysplasia ichthyosiform nevus and limb defect (CHILD) syndrome); as well as type 1 neurofibromatosis 1 (NF1) and tuberous sclerosis; and type 2 segmental manifestation of NF1. Conclusion: Dermatologists should be familiar with mosaic manifestations in neurocutaneous syndrome to ensure early recognition which can help to make proper diagnosis, formulating treatment plans, anticipating potential complications, making appropriate referrals, and offering genetic counseling to families. Keywords: Cutaneous mosaicism, Neurocutaneous syndrome, Type 1 segmental mosaic, Type 2 segmental mosaic. Introduction The skin and the central nervous system are derived from a common embryonic origin during fetal development. Several neurocutaneous syndrome may manifest in mosaic patterns with cutaneous involvement. Hence, the skin can be a window into the central nervous system. Dermatologists are in a privileged position when studying human mosaicism, and can help early recognition in the diagnosis of neurologic disease. The aim of this study is to highlight the clinical variability of mosaic manifestations in patients diagnosed with neurocutaneous syndrome in Sardjito hospital Yogyakarta Indonesia from 2007-2017. This article focuses on several important neurocutaneous syndromes with clinical mosaic manifestation. Reviews A mosaic is an organism composed of two or more genetically different cell lines originating from one homogeneous

  • zygote. Mosaicism results when a postzygotic mutation occurs in gonadal (germline mosaicism) or other cells (somatic

mosaicism), leading to an organism with two or more genetically different cells populations originated from a genetically homogeneous zygote.[1] Cutaneous mosaicisms are the clinical expressions of these disorders. Six archetypical patterns of cutaneous mosaicism can so far be distinguished: (1) lines of Blaschko, (2) checkerboard pattern, (3) phylloid pattern, (4) patchy pattern without midline separation, (5) lateralization pattern, and (6) sash-like pattern.[2,3] Neurocutaneous syndrome is a phenotypically and genetically diverse group of multisystem disorders which the characteristic skin lesions are seen in association with abnormalities of the central nervous system. The constellation of features in some neurocutaneous syndromes reflects the fact that affected tissues shares an embryonic origin in the neural crest, which gives rise to both the skin and nervous system.[4] In the period of Januari 2007-December 2017, we found 68 cases of neurocutaneous syndrome presented with cutaneous mosaic manifestation, with the age between 1 day - 49 years old, 38 females and 30 males. Table 1 and Figure 1 shows the patterns of cutaneous mosaicism and diagnoses.

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 204 Table 1. Patterns of cutaneous mosaicism Patterns of cutaneous mosaicism Diseases Number Gender Lines of Blaschko Incontinentia pigmenti 6 6 Females Schimmelpenning syndrome 4 2 Females 2 Males Phacomatosis pigmentokeratotica 2 2 Females Pigmentary mosaic of the Ito type 13 7 Females 6 Males Checkerboard pattern Sturge-Weber syndrome 7 5 Females 2 Males Patchy pattern without midline separation Giant congenital melanocytic nevus/ neurocutaneous melanosis 5 3 Females 2 Males Lateralization pattern CHILD syndrome 1 1 Female Moreover we found 6 patients with type 1 segmental neurofibromatosis type 1 and 12 patients with type 1 mosaic of tuberous sclerosis complex, as well as type 2 segmental manifestation of neurofibromatosis 1 (NF1) in the form of plexiform NF. Figure 1. From left: Lines of Blaschko, checkerboard pattern, patchy pattern without midline separation, lateralization pattern

  • 1. Lines of Blaschko

The lines of Blaschko reflect epigenetic mosaicism.[2,3] In this form the linear pattern can be inherited from one generation to the next one. A classical example is the Lyon effect of X inactivation giving rise to linear skin lesions in incontinentia pigmenti. The clinical diagnoses we made for our neurocutaneous syndrome with Blaschko line were incontinentia pigmenti, Schimmelpenning syndrome, phacomatosis pigmentokeratotica, and pigmentary mosaic of the Ito type. Incontinentia Pigmenti Incontinentia pigmenti (IP, Bloch-Sulzberger syndrome, MIM 30830) is a rare X-linked dominant multisystem disease affecting CNS, eyes, dentition, nails, and hair, of which several skin manifestations are the first occuring sign. It is hemizygous lethal in most males, with the incidence of approximately 1:40000.[5] The disorder is characterized by linear skin lesions following the lines of Blaschko, that develop in four stages. However, it cannot always be clearly separated from each other and sometimes overlap. During the first months of life, an episode of inflammation and blistering of the skin occurs, being arranged along Blaschko’s lines. Later, these lesions are replaced by hyperkeratotic verrucous areas, whereas in elder females a linear or patchy hyperpigmentation is noted.[5,6] IP is caused by mutations in the IKBKG gene, which was previously known as the NEMO gene. The IKBKG gene localizes to Xq28, where a deletion in the IKBKG exon 4 to exon 10 causes the majority of cases of IP. Schimmelpenning Syndrome Schimmelpenning syndrome (nevus sebaceous syndrome, MIM 163200) includes sebaceous nevus which follows Blaschko’s lines and skeletal abnormalities, neurologic defects, and ocular anomalies.[7] This syndrome may be associated with papular nevus spilus in the form of phacomatosis pigmentokeratotica that represents a pseudodidymosis. The disease is caused by a postzygotic HRAS mutation in a multipotent progenitor cell.[8]

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 205 Phacomatosis Pigmentokeratotica Phacomatosis pigmentokeratotica was recognized by Happle et al. as a variant of Schimmelpenning syndrome. It is characterized by the coexistence of nevus sebaceus following the lines of Blaschko and papular nevus spilus with associated neurologic abnormalities including mental deficiency, seizures, hemiparesis, hyperhidrosis, cutaneous dysesthesia, muscular weakness, and sensory or motor neuropathy.[7] Linear Hypomelanosis in Narrow Bands (Pigmentary Mosaic of the Ito Type) These disorders are characterized by depigmented skin lesions following Blaschko’s lines. Linear hypomelanosis is an umbrella term that includes a considerable amount of different forms of cellular mosaicism that may or may not be discernible at the cytogenetic level.[2,9] Linear hypomelanosis is sometimes associated with neurologic or other extracutaneous defects, but again it should be borne in mind that such cases do not represent one distinct phenotype but a group of numerous different mosaic states.[2]

  • 2. Checkerboard pattern

The distinctive pattern is alternating squares of aberrant tissues arranged in flag-like or block-like patterns, as seen in Sturge-Weber syndrome. Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by combination of a facial port-wine birthmarks with capillary/venous malformations in the eye and leptomeninges. Historically, a facial capillary malformation was considered to have a significant chance of being part of SWS if it affects the region innervated by the first branch of the trigeminal nerve. However, the vascular malformations in SWS seems to follow the distribution of the embryonic facial vasculature rather than the trigeminal nerve branches.[10] The disease is caused by a somatic mosaic mutation in the GNAQ gene located on chromosome 9q21, which plays an important role in vascular development.[11] The recent discovery of the GNAQ gene in 2013 clarified that Sturge-Weber syndrome represents the severe end of a clinical spectrum that also includes more benign isolated port-wine birthmarks (previously known as port-wine stains) in the mildest form.[11]

  • 3. Patchy pattern without midline separation

The characteristic pattern is large patches not respecting the dorsal and ventral midline. One classical example is giant congenital melanocytic nevi (GCMN). Approximately 6–11% of patients with GCMN and numerous satellite nevi involving the head, posterior neck, or paravertebral area are at the greatest risk of developing neurocutaneous melanosis (NCM).[12] In 2013, Kinsler et al.[13] found that most cases of multiple congenital nevi and neuromelanosis are caused by heterozygosity for a postzygotic NRAS codon 61 mutation originating from one single progenitor cell.

  • 4. Lateralisation pattern

It is characterized by unilateral diffuse involvement with a sharp midline demarcation, particularly strict on the ventral aspect of the trunk, which can be seen in CHILD syndrome. CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects; MIM 308050) is an X-linked dominant male-lethal trait caused by mutations in the NSDHL gene [NAD(P)H steroid dehydrogenase-like protein].[14,15] The disorder is characterized by an inflammatory nevus associated with ipsilateral limb anomalies in the form of hypoplasia or aplasia of bones; in addition, ipsilateral visceral or neurological defects may be associated.[7] The nevus either exclusively or preponderantly involves one side of the body and shows two different patterns of distribution. In more severe cases, there is a striking lateralization diffusely affecting one side of the body with a strict midline demarcation. On the other hand, the lesions may follow Blaschko’s lines. Both patterns are often present and intermingled.[2] Segmental Mosaicism in Neurocutaneous Syndrome Segmental mosaicism occurring in autosomal dominant skin disorders can be categorized into two types. Type 1 segmental mosaicism reflects heterozygosity originating from a postzygotic new mutation occurring in an otherwise healthy embryo. Type 2 segmental mosaicism results from postzygotic loss of heterozygosity occurring in a heterozygous embryo.[16] Mosaic NF 1 is an example.[2] Segmental (mosaik) NF 1 reflects postzygotic mosaicism of the NF1 mutation[17] and the typical clinical features are limited to distinct body segments.[18] In the involved area, either neurofibromas alone, pigmentary changes alone or both lesions may be present, with pigmentary changes that typically develop in childhood and neurofibromas developing in the adolescence or adulthood. Type 2 segmental mosaicism appears much earlier than the nonsegmental phenotype showing pronounced clinical features being superimposed on the ordinary, nonsegmental involvement of the disease, meaning that the remaining skin is affected to a degree as noted in the ordinary, nonmosaic phenotype.[2,16] In NF1, type 2 segmental mosaicism appears to occur more frequently than the type 1 segmental manifestation. All sizable plexiform neurofibromas, are an important diagnostic criterion of NF1 and represent a type 2 segmental manifestation of NF1.[2,19] Conclusion The neurocutaneous disorders represent a broad spectrum of genetically and clinically heterogeneous syndromes that are frequently encountered by dermatologist. Dermatologists should be familiar with mosaic manifestations in neurocutaneous syndrome to ensure early recognition which contributes to make proper diagnosis, formulating treatment plans, making appropriate referrals, and offering genetic counseling to families. The important point is to recognize that, the skin is a diagnostic window to the CNS, and thus its common features should not be overlooked.

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23th RCD - 16th PIT PERDOSKI 2018, Surabaya - Indonesia, August 8-11th 2018 206 References 1. Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol. 1993; 129(11): 1460-1470. 2. Happle R. Mosaicism in Human Skin. 2014. Heidelberg, Germany: Springer. 3. Happle R. The categories of cutaneous mosaicism: A proposed classification. Am J Med Genet A. 2016; 170A(2): 452-459. 4. Rosser T. Neurocutaneous disorders. Continuum (Minneap Minn). 2018; 24(1, Child Neurology): 96-129. 5. Swinney CC, Han DP, Karth PA. Incontinentia pigmenti: A comprehensive review and update. Ophthalmic Surg Lasers Imaging Retina. 2015; 46(6): 650-657. 6. Minic S, Trpinac D, Obradovic M. Incontinentia pigmenti diagnostic criteria update. Clin Genet. 2014; 85(6): 536-542. 7. Happle R. The group of epidermal nevus syndromes Part I. Well defined phenotypes. J Am Acad Dermatol. 2010; 63(1): 1-22. 8. Groesser L, Herschberger E, Sagrera A, et al. Phacomatosis pigmentokeratotica is caused by a postzygotic HRAS mutation in a multipotent progenitor cell. J Invest Dermatol. 2013; 133(8): 1998-2003. 9. Ruggieri M, Pratico AD. Mosaic neurocutaneous disorders and their causes. Semin Pediatr Neurol. 2015; 22(4): 207- 233.

  • 10. Waelchli R, Aylett SE, Robinson K, et al. New vascular classification of port-wine stains: Improving prediction of

Sturge-Weber risk. Br J Dermatol. 2014; 171(4): 861-867.

  • 11. Shirley MD,Tang H, Gallione CJ, et al. Sturge-Weber syndrome and port-wine stains caused by somatic mutation in
  • GNAQ. N Engl J Med. 2013; 368(21): 1971-1979.
  • 12. Lovett A, Maari C, Decarie JC, et al. Large congenital melanocytic nevi and neurocutaneous melanocytosis: One

pediatric center's experience. J Am Acad Dermatol. 2009; 61(5): 766-774.

  • 13. Kinsler VA, Thomas AC, Ishida M, et al. Multiple congenital melanocytic nevi and neurocutaneous melanosis are

caused by postzygotic mutations in codon 61 of NRAS. J Invest Dermatol. 2013; 133(9): 2229-2236.

  • 14. König A, Happle R, Bornholdt D, et al. Mutations in the NSDHL gene, encoding a 3beta-hydroxysteroid

dehydrogenase, cause CHILD syndrome. Am J Med Genet. 2000; 90(4): 339-346.

  • 15. Danarti R, Grzeschik KH, Radiono S, et al. Left-sided CHILD syndrome caused by a nonsense mutation in exon 7 of

the NSDHL gene. Eur J Dermatol. 2010; 20(5): 634-635.

  • 16. Happle R. A rule concerning the segmental manifestation of autosomal dominant skin disorders. Review of clinical

examples providing evidence for dichotomous types of severity. Arch Dermatol. 1997; 133(12): 1505-1509.

  • 17. Tinschert S, Naumann I, Stegmann E, et al. Segmental neurofibromatosis is caused by somatic mutation of the

neurofibromatosis type 1 (NF1) gene. Eur J Hum Genet. 2000; 8(6): 455-459.

  • 18. Tanito K, Ota A, Kamide R, et al. Clinical features of 58 Japanese patients with mosaic neurofibromatosis 1. J
  • Dermatol. 2014; 41(8): 724-728.
  • 19. Happle R. Large plexiform neurofibromas may be explained as a type 2 segmental manifestation of neurofibromatosis
  • 1. Am J Med Genet. 2001; 98(4): 363-364.
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