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Histiozytosen

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Histopathologie der Haut

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Zusammenfassung

Unter dem Begriff der Histiozytosen werden Erkrankungen zusammenfasst, die sich von Makrophagen oder dendritischen Zellen ableiten. Beide Zelltypen finden ihren Ursprung im Knochenmark. Makrophagen entwickeln sich über Blutmonozyten durch Einwanderung in verschiedene Gewebe zu Makrophagen. Phagozytose verschiedener Materialien und Reifung beeinflussen die Gestalt dieser Zellen, die dadurch morphologisch sehr heterogen werden. Dendritische Zellen stammen ebenso vom Knochenmark ab und finden sich danach in der Haut, in den Lymphknoten und anderen Organen. Langerhans-Zellen sind die dendritischen Zellen der Epidermis. Sie sind der Prototyp einer Antigen-präsentierenden Zelle, die Antigen in der Epidermis aufnimmt, prozessiert und dieses anschließend nach Migration zum Lymphknoten ebendort präsentiert und damit eine Immunantwort initiiert. Langerhans-Zellen (CD1a+, Langerin (CD207)+, S100+) und Makrophagen (CD68+) sind zumeist immunhistochemisch leicht zu unterscheiden. Expressionsprofile können sich jedoch im Rahmen der Zellreifung ändern, und auch Überlappungen kommen vor (Tab. 1). Daraus ergibt sich ein dritter Zelltyp, der im Zusammenhang mit Histiozytosen Erwähnung findet, die sog. indeterminierte Zelle, deren Eigenständigkeit kontrovers diskutiert wird. Wahrscheinlich handelt es sich dabei um Makrophagen oder dendritische Zellen, deren Expressionsprofil von der Norm abweicht (S100+ Makrophagen, CD207- Langerhans-Zellen). Die Einteilung der Histiozytosen kann primär nach Zelltyp, sowie sekundär nach dem klinischen Befund und der Histologie erfolgen (Übersicht „Klassifikation der Histiozytosen“).

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Weiterführende Literatur

Weiterführende Literatur zu Makrophagen- und Langerhans-Zell-Erkrankungen

  • Jaffe R, Chikwava KR (2012) Disorders of histiocytes. In: Hsi ED (Hrsg) Hematopathology, Bd 19. Saunders, Philadelphia, S 3–40

    Google Scholar 

  • Satter EK, High WA (2008) Langerhans cell histiocytosis: a review of the current recommendations of the Histiocyte Society. Pediatr Dermatol 25:291–295

    Article  PubMed  Google Scholar 

  • Swerdlow SH, Campo E, Harris NL et al. (Hrsg) (2008) WHO classification of tumours of haematopoietic and lymphoid tissue. IARC, Lyon

    Google Scholar 

  • Weitzman S, Egeler RM (2005) Histiocytic disorders of children and adults. Cambridge University Press, Cambridge

    Book  Google Scholar 

Weiterführende Literatur zur Langerhans-Zell-Histiozytose (LZH)

  • Allen CE, Li L, Peters TL et al (2010) Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol 184:4557–4567

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Badalian-Very G, Vergilio JA, Degar BA et al (2012) Recent advances in the understanding of Langerhans cell histiocytosis. Br J Haematol 156:163–172

    Article  CAS  PubMed  Google Scholar 

  • Battistella M, Fraitag S, Teillac DH et al (2010) Neonatal and early infantile cutaneous Langerhans cell histiocytosis: comparison of self-regressive and non-self-regressive forms. Arch Dermatol 146:149–156

    Article  PubMed  Google Scholar 

  • Berres ML, Allen CE, Merad M (2013) Pathological consequence of misguided dendritic cell differentiation in histiocytic diseases. Adv Immunol 120:127–161

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hashimoto K, Pritzker MS (1973) Electron microscopic study of reticulohistiocytoma. An unusual case of congenital, self-healing reticulohistiocytosis. Arch Dermatol 107:263–270

    Article  CAS  PubMed  Google Scholar 

  • Haupt R, Minkov M, Astigarraga I et al (2013) Langerhans cell histiocytosis (LCH): guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 60:175–184

    Article  PubMed  PubMed Central  Google Scholar 

  • Kapur P, Erickson C, Rakheja D et al (2007) Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas Children’s Medical Center. J Am Acad Dermatol 56:290–294

    Article  PubMed  Google Scholar 

  • Lichtenstein L, Histiocytosis X (1953) Integration of eosinophilic granuloma, Letterer-Siwe disease and Hand-Schüller-Christian disease as related manifestations of a single nosologic entity. Arch Pathol 56:84–102

    CAS  Google Scholar 

  • Lieberman PH, Jones CR, Steinman RM et al (1996) Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol 20:519–552

    Article  CAS  PubMed  Google Scholar 

  • Pileri SA, Grogan TM, Harris NL et al (2002) Tumours of histiocytes and accessory dendritic cells: an immunohistochemical approach to classification from the International Lymphoma Study Group based on 61 cases. Histopathology 41:1–29

    Article  CAS  PubMed  Google Scholar 

  • Sagransky MJ, Deng AC, Magro CM (2013) Primary cutaneous Langerhans cell sarcoma: a report of four cases and review of the literature. Am J Dermatopathol 35:196–204

    Article  PubMed  Google Scholar 

Weiterführende Literatur zur Xanthogranulom-Familie

  • Battaglini J, Olsen TG (1984) Disseminated xanthosiderohistiocytosis, a variant of xanthoma disseminatum, in a patient with a plasma cell dyscrasia. J Am Acad Dermatol 11:750–755

    Article  CAS  PubMed  Google Scholar 

  • Breier F, Zelger B, Reiter H et al (2002) Papular xanthoma: a clinicopathological study of 10 cases. J Cutan Pathol 29:200–206

    Article  PubMed  Google Scholar 

  • Burgdorf WH, Zelger B (2004) JXG, NF1, and J alphabet soup or a clinical issue? Pediatr Dermatol 21:174–176

    Article  PubMed  Google Scholar 

  • Burgdorf WH, Kusch SL, Nix TE Jr et al (1981) Progressive nodular histiocytoma. Arch Dermatol 117:644–649

    Article  CAS  PubMed  Google Scholar 

  • Caputo R, Grimalt R, Gelmetti C et al (1993) Unusual aspects of juvenile xanthogranuloma. J Am Acad Dermatol 29:868–870

    Article  CAS  PubMed  Google Scholar 

  • Caputo R, Veraldi S, Grimalt R et al (1995) The various clinical patterns of xanthoma disseminatum. Considerations on seven cases and review of the literature. Dermatology 190:19–24

    Article  CAS  PubMed  Google Scholar 

  • Chang MW, Frieden IJ, Good W (1996) The risk of intraocular juvenile xanthogranuloma: survey of current practices and assessment of risk. J Am Acad Dermatol 34:445–449

    Article  CAS  PubMed  Google Scholar 

  • Dehner LP (2003) Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 27:579–593

    Article  PubMed  Google Scholar 

  • Fett N, Liu RH (2011) Multicentric reticulohistiocytosis with dermatomyositis-like features: a more common disease presentation than previously thought. Dermatology 222:102–108

    Article  PubMed  Google Scholar 

  • Gianotti F, Caputo R, Ermacora E et al (1986) Benign cephalic histiocytosis. Arch Dermatol 122:1038–1043

    Article  CAS  PubMed  Google Scholar 

  • Goltz RW, Laymon CW (1954) Multicentric reticulohistiocytosis of the skin and synovia. Arch Dermatol 69:717–730

    CAS  Google Scholar 

  • Hilker O, Kovneristy A, Varga R et al (2013) Progressive nodular histiocytosis. J Dtsch Dermatol Ges 11:301–307

    PubMed  Google Scholar 

  • Janssen D, Harms D (2005) Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the Kiel pediatric tumor registry. Am J Surg Pathol 29:21–28

    Article  PubMed  Google Scholar 

  • Jih DM, Salcedo SL, Jaworsky C (2002) Benign cephalic histiocytosis: a case report and review. J Am Acad Dermatol 47:908–913

    Article  PubMed  Google Scholar 

  • Miettinen M, Fetsch JF (2006) Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases. Am J Surg Pathol 30:521–528

    Article  PubMed  Google Scholar 

  • Mowbray M, Schofield OM (2007) Juvenile xanthogranuloma en plaque. Pediatr Dermatol 24:670–671

    Article  PubMed  Google Scholar 

  • Ngendahayo P, de Saint AN (2012) Mitotically active xanthogranuloma: a case report with review of the literature. Am J Dermatopathol 34:e27–e30

    Article  PubMed  Google Scholar 

  • Sanchez Yus E, Requena L, Villegas C et al (1995) Subcutaneous juvenile xanthogranuloma. J Cutan Pathol 22:460–465

    Article  CAS  PubMed  Google Scholar 

  • Seward JL, Malone JC, Callen JP (2004) Generalized eruptive histiocytosis. J Am Acad Dermatol 50:116–120

    Article  PubMed  Google Scholar 

  • Shapiro PE, Silvers DN, Treiber RK et al (1991) Juvenile xanthogranulomas with inconspicuous or absent foam cells and giant cells. J Am Acad Dermatol 24:1005–1009

    Article  CAS  PubMed  Google Scholar 

  • Skinner M, Briant M, Morgan MB (2011) Erdheim-Chester disease: a histiocytic disorder more than skin deep. Am J Dermatopathol 33:e24–e26

    Article  PubMed  Google Scholar 

  • Tajirian AL, Malik MK, Robinson-Bostom L et al (2006) Multicentric reticulohistiocytosis. Clin Dermatol 24:486–492

    Article  PubMed  Google Scholar 

  • Torrelo A, Juarez A, Hernandez A et al (2009) Multiple lichenoid juvenile xanthogranuloma. Pediatr Dermatol 26:238–240

    Article  PubMed  Google Scholar 

  • Tran DT, Wolgamot GM, Olerud J et al (2008) An ‘eruptive’ variant of juvenile xanthogranuloma associated with Langerhans cell histiocytosis. J Cutan Pathol 35(Suppl 1):50–54

    Article  PubMed  Google Scholar 

  • Winkelmann RK, Muller SA (1963) Generalized eruptive histiocytoma. Arch Dermatol 88:586–596

    Article  CAS  PubMed  Google Scholar 

  • Zelger B, Burgdorf W (2013) In: Plewig G, Landthaler M, Burgdorf W, Hertl M, Ruzicka T (Hrsg) Braun-Falco's Dermatologie, Venerologie und Allergologie. Springer, Heidelberg, S 1818

    Google Scholar 

  • Zelger BW, Cerio R (2001) Xanthogranuloma is the archetype of non-Langerhans cell histiocytoses. Br J Dermatol 145:369–371

    Article  CAS  PubMed  Google Scholar 

  • Zelger B, Cerio R, Orchard G et al (1992) Histologic and immunohistochemical study comparing xanthoma disseminatum and histiocytosis X. Arch Dermatol 128:1207–1212

    Article  CAS  PubMed  Google Scholar 

  • Zelger B, Cerio R, Soyer HP et al (1994) Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. Am J Dermatopathol 16:577–584

    Article  CAS  PubMed  Google Scholar 

  • Zelger BG, Zelger B, Steiner H et al (1995a) Solitary giant xanthogranuloma and benign cephalic histiocytosis–variants of juvenile xanthogranuloma. Br J Dermatol 133:598–604

    Article  CAS  PubMed  Google Scholar 

  • Zelger BW, Staudacher C, Orchard G et al (1995b) Solitary and generalized variants of spindle cell xanthogranuloma (progressive nodular histiocytosis). Histopathology 27:11–19

    Article  CAS  PubMed  Google Scholar 

  • Zelger BW, Sidoroff A, Orchard G et al (1996) Non-Langerhans cell histiocytoses. A new unifying concept. Am J Dermatopathol 18:490–504

    Article  CAS  PubMed  Google Scholar 

  • Zelger BG, Orchard G, Rudolph P et al (1998) Scalloped cell xanthogranuloma. Histopathology 32:368–374

    Article  CAS  PubMed  Google Scholar 

Weiterführende Literatur zu Xanthomen

  • Altmann J, Winkelmann RK (1962) Diffuse normolipemic plane xanthoma. Arch Dermatol 85:633–640

    Article  Google Scholar 

  • Blankenship DW, Zech L, Mirzabeigi M et al (2013) Verruciform xanthoma of the upper-extremity in the absence of chronic skin disease or syndrome: a case report and review of the literature. J Cutan Pathol 40:745–752

    Article  PubMed  Google Scholar 

  • Braun-Falco O, Eckert F (1991) Macroscopic and microscopic structure of xanthomatous eruptions. Curr Probl Dermatol 20:54–62

    Article  CAS  PubMed  Google Scholar 

  • Cruz PD Jr, East C, Bergstresser PR (1988) Dermal, subcutaneous, and tendon xanthomas: diagnostic markers for specific lipoprotein disorders. J Am Acad Dermatol 19:95–111

    Article  PubMed  Google Scholar 

  • Cumberland L, Dana A, Resh B et al (2010) Verruciform xanthoma in the setting of cutaneous trauma and chronic inflammation: report of a patient and a brief review of the literature. J Cutan Pathol 37:895–900

    Article  PubMed  Google Scholar 

  • Ferrara G, Palombi N, Lipizzi A et al (2007) Nonnecrobiotic necrobiotic xanthogranuloma. Am J Dermatopathol 29:306–308

    Article  PubMed  Google Scholar 

  • Finan MC, Winkelmann RK (1987) Histopathology of necrobiotic xanthogranuloma with paraproteinemia. J Cutan Pathol 14:92–99

    Article  CAS  PubMed  Google Scholar 

  • Kossard S, Winkelmann RK (1980) Necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol 3:257–270

    Article  CAS  PubMed  Google Scholar 

  • Marcoval J, Moreno A, Bordas X et al (1998) Diffuse plane xanthoma: clinicopathologic study of 8 cases. J Am Acad Dermatol 39:439–442

    Article  CAS  PubMed  Google Scholar 

  • Mohsin SK, Lee MW, Amin MB et al (1998) Cutaneous verruciform xanthoma: a report of five cases investigating the etiology and nature of xanthomatous cells. Am J Surg Pathol 22:479–487

    Article  CAS  PubMed  Google Scholar 

  • Shafer WG (1971) Verruciform xanthoma. Oral Surg Oral Med Oral Pathol 31:784–789

    Article  CAS  PubMed  Google Scholar 

  • Wood AJ, Wagner MV, Abbott JJ et al (2009) Necrobiotic xanthogranuloma: a review of 17 cases with emphasis on clinical and pathologic correlation. Arch Dermatol 145:279–284

    Article  PubMed  Google Scholar 

  • Zelger B, Eisendle K, Mensing C et al (2007) Detection of spirochetal micro-organisms by focus-floating microscopy in necrobiotic xanthogranuloma. J Am Acad Dermatol 57:1026–1030

    Article  PubMed  Google Scholar 

Weiterführende Literatur zu den Sonderformen

  • Destombes P (1965) Adenites avec surcharge lipidique, de l'enfant ou de l'adulte jeune, observees aux Antilles et au Mali. (Quatre observations) [Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali. (4 cases)]. Bull Soc Pathol Exot Filiales 58:1169–1175

    CAS  PubMed  Google Scholar 

  • Foucar E, Rosai J, Dorfman R (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7:19–73

    CAS  PubMed  Google Scholar 

  • Frater JL, Maddox JS, Obadiah JM et al (2006) Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case. J Cutan Med Surg 10:281–290

    Article  PubMed  Google Scholar 

  • Kuo TT, Chen TC, Lee LY et al (2009) IgG4-positive plasma cells in cutaneous Rosai-Dorfman disease: an additional immunohistochemical feature and possible relationship to IgG4-related sclerosing disease. J Cutan Pathol 36:1069–1073

    Article  PubMed  Google Scholar 

  • Ratzinger G, Burgdorf WH, Metze D et al (2005) Indeterminate cell histiocytosis: fact or fiction? J Cutan Pathol 32:552–560

    Article  PubMed  Google Scholar 

  • Rezk SA, Spagnolo DV, Brynes RK et al (2008) Indeterminate cell tumor: a rare dendritic neoplasm. Am J Surg Pathol 32:1868–1876

    Article  PubMed  Google Scholar 

  • Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 87:63–70

    CAS  PubMed  Google Scholar 

  • Shamburek RD, Brewer HB Jr, Gochuico BR (2001) Erdheim-Chester disease: a rare multisystem histiocytic disorder associated with interstitial lung disease. Am J Med Sci 321:66–75

    Article  CAS  PubMed  Google Scholar 

  • Sidoroff A, Zelger B, Steiner H et al (1996) Indeterminate cell histiocytosis–a clinicopathological entity with features of both X- and non-X histiocytosis. Br J Dermatol 134:525–532

    Article  CAS  PubMed  Google Scholar 

  • Tsai JW, Tsou JH, Hung LY et al (2010) Combined Erdheim-Chester disease and Langerhans cell histiocytosis of skin are both monoclonal: a rare case with human androgen-receptor gene analysis. J Am Acad Dermatol 63:284–291

    Article  PubMed  Google Scholar 

  • Volpicelli ER, Doyle L, Annes JP et al (2011) Erdheim-Chester disease presenting with cutaneous involvement: a case report and literature review. J Cutan Pathol 38:280–285

    Article  PubMed  PubMed Central  Google Scholar 

  • Wang KH, Chen WY, Liu HN et al (2006) Cutaneous Rosai-Dorfman disease: clinicopathological profiles, spectrum and evolution of 21 lesions in six patients. Br J Dermatol 154:277–286

    Article  PubMed  Google Scholar 

  • Wood GS, Hu CH, Beckstead JH et al (1985) The indeterminate cell proliferative disorder: report of a case manifesting as an unusual cutaneous histiocytosis. J Dermatol Surg Oncol 11:1111–1119

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Universitätsklinik für Dematologie und Venerologie, Medizinische Universität, Anichstr. 35, 6020, Innsbruck, Ӧsterreich

    G. Ratzinger & B. Zelger

  2. Universitätsklinik für Dematologie und Allergologie, Ludwig Maximilian Universität, München, Deutschland

    W. Burgdorf

Authors
  1. G. Ratzinger
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  2. W. Burgdorf
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  3. B. Zelger
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Correspondence to G. Ratzinger .

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Editors and Affiliations

  1. Universitätsklinik für Dermatologie, Medizinische Universität Graz, Graz, Austria

    Lorenzo Cerroni

  2. Universitäts-Hautklinik, Eberhard-Karls-Universität, Tübingen, Germany

    Claus Garbe

  3. Universitäts-Hautklinik Münster, Münster, Germany

    Dieter Metze

  4. Dermatopathologie Friedrichshafen, Friedrichshafen, Germany

    Heinz Kutzner

  5. Universitätsklinik für Dermatologie und, Medizinische Universität Graz, Graz, Steiermark, Austria

    Helmut Kerl

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Ratzinger, G., Burgdorf, W., Zelger, B. (2016). Histiozytosen. In: Cerroni, L., Garbe, C., Metze, D., Kutzner, H., Kerl, H. (eds) Histopathologie der Haut. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45133-5_26

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