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Angiokeratome

Zuletzt geändert von Thomas Brinkmeier am 2025/12/01 20:26

Angiokeratome

Pa: dilatierte Kapillaren im Corium, bedeckt von einer hyperkeratotischen Epidermis

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Etlg: - solitäres Angiokeratom

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Vork: meist Jugendliche

KL: schwärzlicher, flacher, münzgroßer, hyperkeratotischer Knoten

Lok: Prädilektionsstelle: untere Extremität

DD: malignes Melanom

Lit:  

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- Angiokeratoma circumscriptum

Engl: Angiokeratoma circumscriptum naeviforme

KL: verruköse, blaue Plaques

Lok: Prädilektionsstelle: Extremitäten

Lit: - Our Dermatol Online. 2016;7(4):489-491.  

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- Dermatol Online J. 2020 Nov 15;26(11):13030/qt8v6170qz

- Angiokeratoma Mibelli

Vork: Jugendliche mit Akrozyanose

KL: winzige blauschwarze keratotische Papeln an Fingern und Zehen

DD: Verrucae vulgares

- Angiokeratoma Fordyce

KL: kleine, violette Papeln am Skrotum

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So: in seltenen Einzelfällen assoziiert mit erhöhtem Druck in den skrotalen Venen aufgrund von Hernien, Nebenhoden- oder Harnwegstumoren oder Varikozelen

Lit: Indian J Dermatol Venereol Leprol. 2024 Jun 19:1-3. http://doi.org/10.25259/IJDVL_257_2024

Lit: - JAAD Case Rep. 2020 Dec 14;8:50-52. http://doi.org/10.1016/j.jdcr.2020.12.007

- Dermatol Pract Concept. 2021 Oct 1;11(4):e2021121. http://doi.org/10.5826/dpc.1104a121

- Angiokeratoma corporis diffusum Fabry

Syn: - Morbus Fabry

- Thesaurismosis hereditaria lipoidica

Histr: Erstbeschreibung durch Anderson und Fabry im Jahre 1898

Def: genetisch bedingte lysosomale Speicherkrankheit/Sphingolipidose als Multisystemerkrankung (Haut, Herz, Niere, Auge, ZNS) mit sehr variabler klinischer Expression

So: Fälle mit rein kutaner Symptomatik sind selten  

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Gen: X-chromosomal-rezessiv (Xq21)

Ät: alpha-Galaktosidase-Mangel mit pathologischer Ablagerung des Phosphatids Ceramidtrihexosid (oder Trihexosylceramid) v. a. in Endothelien

DD: Beta-Mannosidose

Lit: J Dermatol 2004; 31: 931-5

Aus: Fälle mit normaler Enzymaktivität sind beschrieben.

Lit: J Dtsch Dermatol Ges. 2018 Jun;16(6):769-771. http://doi.org/10.1111/ddg.13530_g

Pg: ischämischer Defekt der abhängigen Gewebe

Man: meist erst nach dem 10. Lj.

Vork: - meist hemizygote Männer

- Inzidenz von 1/40.000

- Weibliche (heterozygote) Pat. (Carrier) zeigen einen Manifestationsindex von nur ca. 20% bei insgesamt milderer Symptomatik.

DD: - Purpura pigmentosa progressiva

- Angioma serpiginosum

KL: - Hautveränderungen

Bef: - multiple kleine Angiokeratome

Bed: Leitsymptom

Lok: Prädilektionsstellen sind die Periumbilikal- und Inguinalregion

- Hypotrichose, Hypotrichie

- Lymphödem

- Hypohidrose / Hypohidrosis bis Anhidrose

Kopl: Temperaturerhöhung bei körperlicher Belastung

Hi: Angiokeratome

Bef: - Hyperkeratose und Akanthose mit lang ausgezogenen Reteleisten

- Kapillarektasien im Stratum papillare

- stark PAS-positive Gefäßwände

- Lipidnachweis in den Gefäßwänden

Meth: Sudanschwarz- oder Sudanrot-Färbung

EM: Endothelzellen der Gefäße in nichtbefallener Haut: Nachweis von intrazytoplasmatischen, osmophilen, lamellenartigen, elektronendichten Einschlusskörperchen

- Auge

Bef: - Cornea verticulata

Bef: verwirbelte subepitheliale Einlagerungen in die Kornea

Bed: typisches ophthalmologisches Zeichen

DD: NW von Chloroquin, Chlorpromazin, Indometacin, Amiodaron, Penicillamin

- vermehrte Schlängelung bzw. Aneurysmen der Konjunktival- und Retinagefäße

- subepithelial abgelagerte Glykosphingolipide

Meth: Spaltlampe

- neurologische Symptome (fakultativ):

Bef: - temperaturabhängige Akroparästhesien (palmoplantar) bis hin zu quälenden Extremitätenschmerzen

Bed: typischer anamnestischer Befund

- geistige Retardierung

- Epilepsie

- zerebrale Insulte

- Paralysen

- Polyneuropathie

- progredientes Nierenversagen

- Neigung zu arteriellen Thrombosen

Kopl: Herzinfarkt

Di: - pränatal möglich!

Meth: Amniozentese

- interdisziplinäre Diagnostik und Therapie: Dermatologie, Innere Medizin, Nephrologie, Kardiologie, Gastroenterologie, Neurologie, Pädiatrie, Ophthalmologie, HNO, Humangenetik

Lab: - alpha-Galaktosidase im Plasma erniedrigt, Ceramidtrihexosid im Plasma erhöht

- Bestimmung des alpha-Galaktosidase-Gehalts in Leukozyten

- Lipoidzylinder und lipidspeichernde Makrophagen ("Maulbeerzellen") im Urin

Prog: - wird durch renale und kardiale Beteiligung bestimmt

- ohne Enzymsubstitution für männliche Pat. meist Tod im 3.-4. Lebensjahrzehnt

Lit:  

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Th: - kausal

Przp: Enzymsubstitution durch alpha-Galaktosidase

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Wirk: Effekte hinsichtlich v.a. kardiologischer und nephrologischer Symptome und Komplikationen

Note: keine Wirkung auf die Angiokeratome

- symptomatisch

Ind: - Angiokeratome (s. unten)

- Parästhesien

Stoff: Phenytoin oder Carbamazepin

- Niereninsuffizienz

Meth: Dialyse oder Transplantation

- akrale pseudolymphomatöse Angiokeratome im Kindesalter

Engl: acral pseudolymphomatous angiokeratoma of children (APACHE)

Histr: Erstbeschreibung durch Crow im Jahre 1980

Vork: sehr selten

Lok: meist an Händen oder Füßen

Lit: McFaddin, C., Greene, J., & Parekh, P. (2015). Linear acral pseudolymphomatous angiokeratoma of children with associated nail dystrophy. Dermatology Online Journal, 21(7). https://escholarship.org/uc/item/9kt5z5gw

Th: - CO2-Laserung

Lit: Dermatol Ther. 2018 Sep 7. http://doi.org/10.1111/dth.12682

- Rapamycin Salbe

Lit: Pediatr Dermatol. 2020 Jun 26. http://doi.org/10.1111/pde.14240

- Fukosidase

Ät: autosomal-rezessiver L-Fukosidase-Defekt

Vork: frühe Kindheit

KL: - multiple Angiokeratome

- meist schwere geistige Retardierung

- Minderwuchs

- spastische Lähmungen

So: Halo-Angiokeratom

Vork: selten

Lit:  

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Th: Entfernung der Angiokeratome

Meth: - Exzision

Altn: - Kryotherapie

- Elektrokaustik

- Kürettage

- Laser

Mat: - Argon-Laser

Bed: historisch

- Blitzlicht-gepumpter gepulster Farbstofflaser

- KTP 532 nm-Laser

Lit: Br J Dermatol 2004; 150: 620-2

PT: CR

Meth: 20 J/qcm, 0,7-1,5 Hz, 5 mm Fleckgröße, 50 ms Pulszeit, mehrere Sitzungen

Pos: - weniger Erythembildung als bei anderen Lasersystemen,

- Arbeitsfähigkeit i. d. R. binnen 24 h nach Laserung wieder gegeben

Co: Er:YAG-Laser

Lit: J Dermatol 2004; 31: 943-5

PT: CR

Ind: Angiokeratoma Fordyce der Glans penis

- Sklerotherapie

Lit: Dermatol Surg. 2019 Feb 13. http://doi.org/10.1097/DSS.0000000000001837

Ind: Angiokeratom der Vulva

  
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