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Lymphomatoide Papulose (LYP)

Zuletzt geändert von Thomas Brinkmeier am 2025/12/14 23:05

Lymphomatoide Papulose (LYP)

Engl: lymphomatoid papulosis

Def: chronische, periodisch auftretende, meist benigne, multifokale (polyklonale), lymphoproliferative Erkrankung der Haut im Sinne eines nodulären T-Zell-Pseudolymphoms bzw. CD30-positiven, niedrig malignen kutanen T-Zell-Lymphoms

Histr: Erstbeschreibung durch Dupont im Jahre 1965

Man: - meist Erwachsenenalter

- selten im Kindesalter

Lit: Br J Dermatol. 2014 Apr 19. http://doi.org/10.1111/bjd.13061 (Frankreich)

PT: CS (25 Pat.)

Vork: Männer überwiegen Frauen

KL: 3-20 mm große, z. T. ulzerierte Knoten der Haut (meist nicht mehr als 20 Knoten)

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Lok: Rumpf ist Prädilektionsstelle

So: orale, genitale, akrale Sonderform

Pa: Aspekte von Benignität und Malignität (vergleichbar mit der großfleckigen Parapsoriasis)

DD: - Pityriasis lichenoides

- kleinzelliges pleomorphes CTCL (kutanes T-Zell-Lymphom)

- Skabies

- papulöses Syphilid (L II)

Di: Biopsie: Histologie + Immunphänotypbestimmung + T-Zell-Rezeptor-Genrearrangement

- Histologie

Etlg: Verschiedene Subtypen (A-C dominieren)

- Typ A: disseminierte, teils aggregierte, teils multinukleäre oder Reed-Sternberg-ähnliche CD30-positive atypische Lymphozyten plus ein gemischtzelliges entzündliches Begleitinfiltrat  

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Lit:  

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- Typ B: epidermotropes Infiltrat kleiner atypischer Lymphozyten mit zerebriformer Kernpolymorphie (ähnlich der Mycosis fungoides)

Vork: selten (nur ca. 10% d. F.)

- Typ C: große Aggregate von CD30-positiven Zellen mit einem spärlichen gemischtzelligen Begleitinfiltrat (ähnlich dem anaplastishen großzelligen T-Zell-Lymphom)

- Typ D: Epidermotropismus von CD8-positiven und CD30-positiven T-Zellen (ähnlich dem kutanen aggressiven CD8-positiven zytotoxischen T-Zell-Lymphom)

Lit:  

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- Typ E: angioinvasiver und angiodestruktiver Typ mit Infiltraten von CD30-positiven Lymphozyten, die häufig CD8 koexprimieren (in seltenen Fällen auch CD56)

Lit: -  

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- JAAD Case Rep. 2020 Aug 20;6(11):1170-1172

- Typ mit chromosomalem 6p25-Rearrangement

- Immunphänotypbestimmung/Immunhistochemie:

Bef: - CD4+

- CD30+

Vork: auf einem variablen Prozentsatz der Zellen

Aus: histologischer Typ B (CD3+, CD4+, CD30-)

- CD134+

Vork: 1/3 d. F.

Aus: nicht bei benignen lymphozytären Infiltraten oder Mycosis fungoides

Lit: Br J Dermatol 2003; 145: 885-91

- Gal-3+ (Galectin-3)

DD: niedrig bei transformierter Mycosis fungoides

Lit: Dermatology. 2015;231(2):164-70 (Hildesheim)

- T-Zell-Rezeptor-Genrearrangement:

Erg: klonale T-Zell-Proliferation (in jedem Knoten ein anderer Klon)

Vork: ca. 60-70% d. F.

Allg: Die rearrangierte Gensequenz, die (später) in T-Zell-Rezeptor-spezifische mRNA transkribiert wird, ist ein molekularer Fingerabdruck sowohl für den individuellen Lymphozyten als auch für das aus ihm entstehende Lymphom.

So: akrale lymphomatoide Papulose

Lit: J Cutan Pathol. 2020 Dec 2. http://doi.org/10.1111/cup.13929

Verl: chronisch mit Schüben und tendentieller Spontanremission

Prog: sehr gut

Kopl: Entwicklung eines anderen Lymphomtyps

Etlg: - Mycosis fungoides

- großzelliges CD30-positives T-Zell-Lymphom

- Morbus Hodgkin

Vork: 4-20% d. F.

CV: lebenslanges Follow-up ist erforderlich

Th: - abwartende Haltung ohne spezifische Therapie

- Exzision

Ind: solitäre oder lokalisierte Läsionen

- Glukokortikoide

Appl: topisch, ggf. intraläsional

Altn: - HN2

Syn: Stickstofflost, Mechlorethamin

Engl: nitrogen mustard

Lit: J Dtsch Dermatol Ges. 2024 Apr 3. http://doi.org/10.1111/ddg.15371

- Imiquimod 5% Creme

Lit: J Am Acad Dermatol. 2006 Mar;54(3):546-7

- MTX

Appl: - oral

Bed: GS zur Rezidivprophylaxe

Ind: Erwachsene mit persistierenden Formen

Dos: 5-20 mg/Woche

- lokal

Lit: JAAD Case Rep. 2021 Dec 14;20:31-33. http://doi.org/10.1016/j.jdcr.2021.11.020

- PUVA

Co: - IFN-alpha

- Retinoide

Altn: - UVA1

Lit: J Am Acad Dermatol 2005; 52: 530-2

- 311 nm Excimer-Laser

Lit: - Photodermatol Photoimmunol Photomed. 2006 Jun;22(3):168-71

Lit: CR (Erstbeschreibung)

- JAAD Case Rep. 2020 Apr 18;6(6):495-497

- photodynamische Therapie (PDT)

Lit: Pediatr Dermatol. 2020 Jul 3. http://doi.org/10.1111/pde.14244

- Antibiotika

Stoff: Tetrazykline, Erythromycin

Appl: oral

Ind: Kinder

- Dapson

EbM: CR

- Bexaroten

Def: RXR-selektives Retinoid

Lit: Dermatology 2003; 206: 142-7, J Dermatol. 2017 Oct 3. http://doi.org/10.1111/1346-8138.14095

Appl: oral oder topisch

- Anti-CD30-Ak

Stoff: Brentuximab Vedotin

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- Mycophenolat mofetil (MMF)

Lit: J Cutan Med Surg. 2013 Oct 1;17(5):332-4

PT: CR

  
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