Skip to main content
SpringerLink
Log in

Krankheiten mit intraepidermalen Blasen und Pusteln

  • Chapter
Histopathologie der Haut

Zusammenfassung

Der Pemphigus (P.) vulgaris ist die häufigste Erkrankung aus der Pemphisgusgruppe. Der Altersgipfel liegt in der 4. bis 6. Lebensdekade. Es besteht keine Geschlechtsprävalenz, aber eine ethnische mit Häufung bestimmter HLA-Merkmale. Selten wurde die Assoziation mit anderen Autoimmunerkrankungen wie Myasthenia gravis beschrieben. Der P. vulgaris beginnt meist umschrieben mit uncharakteristischen Läsionen an Mundschleimhaut (siehe auch S. 443; Abb. 20.3), Kopfhaut oder Intertrigines, später kommt es zu generalisiertem Befall des Integumentes und sämtlicher hautnaher Schleimhäute unter Einschluss des Laryngopharynx. Klinisch imponieren flächige, schmerzhaft-brennende, nässende Erosionen mit randwärts halskrausenartig zusammengeschobenen Epidermisfetzen, die hämorrhagisch verkrusten oder impetiginisieren. Intakte Epidermis lässt sich durch seitlichen Druck tangential abschieben (Nikolski-Zeichen). Im Verlauf treten auch schlaffe Blasen mit klarem Inhalt ohne weitere Entzündungszeichen auf. Die Abheilung erfolgt in der Regel narbenlos. Die Krankheit verläuft schubweise progredient und endet unbehandelt aufgrund von Flüssigkeits- und Elektrolytverlusten, Sepsis, Bronchopneumonien oder Kachexie häufig letal. Der P. vulgaris spricht gut auf immunsuppressive Therapien an.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  • Amagai M (1999) Autoimmunity against desmosomal cadherins in pemphigus. J Dermatol Science 2o: 92–102

    Article  Google Scholar 

  • Becker BA, Gaspari AA (1993) Pemphigus vulgaris and veg-etans. Dermatol Clin 11: 429–452

    PubMed  CAS  Google Scholar 

  • Hoss DM (1996) Neutrophilic spongiosis in pemphigus. Arch Dermatol 132: 315–318

    Article  PubMed  CAS  Google Scholar 

  • Yesudian PD, Krishnan SG, Jayaraman M, Janaki VR, Yesudian P (1997) Postpemphigus acanthomata: A sign of clinical activity? Int J Dermatol 36: 194–196

    Article  PubMed  CAS  Google Scholar 

  • Beissert S, Metze D, Luger T, Schwarz T (1993) Pemphigus herpetiformis. Hautarzt 44: 594–597

    PubMed  CAS  Google Scholar 

  • Landau M, Brenner S (1997) Histopathologic findings in drug-induced pemphigus. Am J Dermatopathol 19: 411–414

    Article  PubMed  CAS  Google Scholar 

  • Mutasim DF (1993) Drug induced pemphigus. Dermatol Clin 11: 463–471

    Article  PubMed  CAS  Google Scholar 

  • Robinson ND, Hashimoto T, Amagai M, Chan LS (1999) The new pemphigus variants. J Am Acad Dermatol 40: 649–671

    Article  PubMed  CAS  Google Scholar 

  • Ackerman AB (1997). Paraneoplastic pemphigus. In: Ackerman AB, Chongchitnant N, Sanchez J, Guo Y (eds) Histologic diagnosis of inflammatory skin diseases. Williams Wilkins, Baltimore, pp 588–589

    Google Scholar 

  • Anhalt GJ, Kim SC, Stanly JR et al. (199o) Paraneoplastic Pemphigus. An autoimmune mucocutaneus disease associated with neoplasm. N Engl J Med 323: 1729–1735

    Google Scholar 

  • Horn TA, Anhalt GJ (19929 Histologic features of paraneoplastic pemphigus. Arch Dermatol 128: 1091–1095

    Google Scholar 

  • Metze D (1996) Ultrastrukturelle und molekularbiologische Befunde bei akantholytischen Dermatosen. Verh Dtsch Ges Pathol 8o: 220–222

    Google Scholar 

  • Zillikens D, Bröcker EB (1994) Paraneoplastic pemphigus. Induction of autoantibodies against struktural proteins in the skin. Hautarzt 45: 827–823

    Google Scholar 

  • Burge SM (1992) Hailey-Hailey disease: The clinical features, response to treatment and prognosis. Br J Dermatol 126: 275–282

    Article  PubMed  CAS  Google Scholar 

  • Chorzelski T, Kudejko J, Jablonska S (1984) Is papular acantholytic dyskeratosis of the vulva a new entity ? Am J Dermatopathol 6: 557–558

    Article  PubMed  CAS  Google Scholar 

  • Hailey H, Hailey H (1939) Familial benign chronic pemphigus. Arch Dermatol Syphilol 39: 679–685

    Article  Google Scholar 

  • Hamm H, Metze D, Bröcker EB (1994) Hailey-Hailey disease: Eradication by dermabrasion. Arch Dermatol 130: 1143–1149

    Google Scholar 

  • Happle R, Steijlen PM, Kolde G (199o) Naevus corniculatus: A new acantholytic disorder. Br J Dermatol 122: 107–112

    Google Scholar 

  • Hu Z, Bonifas JM, Beech J et al. (2000) Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease. Nat Genet 24: 61–65

    Article  PubMed  CAS  Google Scholar 

  • Langenberg A, Berger TG, Cardelli M, Rodman OG, Estes S, Barron D (1992) Genital benign chronic pemphigus ( Hailey-Hailey disease) presenting as condylomas. J Am Acad Dermatol 26: 951–955

    Google Scholar 

  • Metze D, Hamm H, Schorat A, Luger T (1996) Involvement of the adherens junction - actin filament system in acantholytic dyskeratosis of Hailey-Hailey disease. A histological, ultrastructural, and histochemical study of lesional and non-lesional skin. J Cutan Pathol 23: 211–222

    Google Scholar 

  • Burge-SM, Wikinson JD (1992) Darier-White disease: A review of the clinical features in 163 patients. J Am Acad Dermatol 27: 4o - 5o

    Google Scholar 

  • Derrick EK, Darley CR, Burge S (1995) Comedonal Darier. Br J Dermatol 132: 453–455

    Article  PubMed  CAS  Google Scholar 

  • DiMaio DJM, Cohen PR (1998) Incidental focal acantholytic dyskeratosis. J Am Acad Dermatol 38: 243–237

    Article  PubMed  CAS  Google Scholar 

  • Hoffman MD, Fleming MG, Pearson RW (1995) Acantholytic epidermolysis bullosa. Arch Dermato1131: 586–589

    Google Scholar 

  • Leverkus M, Rose C, Bröcker EB, Hamm H (1999) Intertriginöse akantholytische Dyskeratose. Hautarzt 5o: 733738

    Google Scholar 

  • O’Malley MP, Haake A, Goldsmith L, Berg D (1997) Localized Darier disease. Implications for genetic studies. Arch Dermatol 133: 1134–1138

    Google Scholar 

  • Panja RK (1972) Acrokeratosis verruziformis Hopf–a clinical entitity? Br J Dermatol 96: 643–651

    Article  Google Scholar 

  • Regazzini R, Zambruno G, DeFilippi C, Rosso R, Donadini A (1996) Isolated acral Darier’s disease with hemorrhagic lesions in a kindred. Br J Dermatol 153: 489–490

    Google Scholar 

  • Sakuntabhai A, Ruiz-Perez V, Carter S et al. (1999) Mutations in ATP2A2, encoding a Ca2+ pump, cause Darier disease. Nat Genet 21: 271–277

    Article  PubMed  CAS  Google Scholar 

  • Telfer NR, Burge SM, Ryan TJ (199o) Vesiculo-bullous Darier’s disease. Br J Dermatol 122: 831–834

    Google Scholar 

  • Ackerman AB (1991, 1992). In: Ackerman AB (ed) Clues to diagnosis in dermatopathology. ASCP, Chicago, vol 1, pp 46–48, and vol 2, pp 90–92

    Google Scholar 

  • Grover RW (197o) Transient akantholytic dermatosis. Arch Dermatol loi: 426–434

    Google Scholar 

  • Hashimoto K, Moiin A, Chang MW, Tada J (1996) Sudoriferous acrosyringeal acantholytic disease. A subset of Grover’s disease. J Cutan Pathol 23: 151–164

    Google Scholar 

  • Parsons JM (1996) Transient acantholytic dermatosis (Grover’s disease): A global perspective. J Am Acad Dermatol 35: 653–666

    Article  PubMed  CAS  Google Scholar 

  • Ackerman AB (1991). In: Ackerman AB (ed) Clues to diagnosis in dermatopathology. ASCP, Chicago, vol 1, pp 7577

    Google Scholar 

  • Cerroni L, Zenahlik P, Kerl H (1995) Specific cutaneous infiltrates of B-cell chronic lymphocytic leukemia arising at the site of herpes zoster and herpes simplex scars. Cancer 76: 26–31

    Article  PubMed  CAS  Google Scholar 

  • McCrary ML, Severson J, Tyring SK (1999) Varicella zoster virus. J Am Acad Dermatol 41: 1–14

    Article  PubMed  CAS  Google Scholar 

  • Metze D (1997) Explaining clinical and histopathological findings by electron microscopy–Herpesvirus infection. Dermatopathology: Practical Conceptual 3: 12–13

    Google Scholar 

  • Munoz E, Valks R, Fernandez-Herrera J, Fraga J (1997) Herpetic syringitis associated with eccrine squamous syringometaplasia in HIV-positive patient. J Cutan Pathol 24: 425–428

    Article  PubMed  CAS  Google Scholar 

  • Pereira FA (1996) Herpes simplex: evolving concepts. J Am Acad Dermatol 35: 503–520

    Article  PubMed  CAS  Google Scholar 

  • Reimann S, Kütting B, Metze D (2000) Spezifische Hautin-filtrate eines extrakutanen B-Zell-Lymphoms in einem 3 abheilenden Herpes simplex. Hautarzt 51: 256–259

    PubMed  CAS  Google Scholar 

  • Resnik K, DiLeonardo M (2000) Herpes incognito. Am J ermatopathol 22: 144–150

    Article  CAS  Google Scholar 

  • Carneiro SJC, Dickson JE, Knox JM (1972) Familial dyskeratotic comedones. Arch Derm 105: 249–251

    Article  PubMed  CAS  Google Scholar 

  • Ständer S, Bütten A, Metze D (2001) Familiäre dyskeratotische Komedonen–eine seltene Entität. Hautarzt 52: 533–536

    Article  PubMed  Google Scholar 

  • Vakilzadeh F, Kolde G (1985) Relapsing linear acantholytic dermatosis. Br J Dermatol 112: 349–355

    Article  PubMed  CAS  Google Scholar 

  • Duschet P, Happle R, Schwarz T, Gschnait F (1995) Relapsing linear acantholytic dermatosis. Br J Dermatol 33: 920–922

    CAS  Google Scholar 

  • Bardach H, Gebhart W, Luger T (1982) Genodermatose bei einem Brüderpaar: M. Dowling-Degos, Grover, Darier, Hailey-Hailey oder M. Galli-Galli. Hautarzt 33: 378383

    Google Scholar 

  • Schiller M, Kütting B, Metze D (1999) Umschriebene retikuläre Hyperpigmentierungen - ein weites klinisches Spektrum. Hautarzt 5o: 58o - 585

    Google Scholar 

  • Darmstadt GL, Lane AT (1994) Impetigo: an overview. Pediatr Dermatol n: 293–303

    Google Scholar 

  • Melish ME (1982) Staphylococcal, streptococci and the skin. Review of impetigo and the stapyholococcal scalded skin syndrome. Semin Dermatol 1: lot-109

    Google Scholar 

  • Noble WC (1998) Skin bacteriology and the role of staphylococcus aureus in infection. Br J Dermatol 139: 9–12

    Article  PubMed  Google Scholar 

  • Amon RB, Dimond RL (1975) Toxic epidermal necrolysis. Rapid differentiation between staphylococcal and drug induced disease. Arch Dermatol un: 1433–1437

    Google Scholar 

  • Cribier B, Piemont Y, Grosshans E (1994) Staphylococcal scalded skin syndrome in adults. J Am Acad Dermatol 30: 319–324

    Article  PubMed  CAS  Google Scholar 

  • Elias PM, Fritsch P, Epstein EH (1977) Staphylococcal scalded skin syndrome. Clinical features, pathogenesis, and recent microbiological and biochemical development. Arch Dermatol 113: 207–219

    Google Scholar 

  • Hurwitz RM, Ackerman AB (1985) Cutaneous pathology of toxic shock syndrome. Am J Dermatopathol 7: 563–578

    Article  PubMed  CAS  Google Scholar 

  • Chimenti S, Ackerman AB (1981) Is subcorneal pustular dermatosis of Sneddon and Wilkinson an entity sui generis? Am J Dermatopathol 3: 363–376

    Article  PubMed  CAS  Google Scholar 

  • Hashimoto T, Inamoto N, Nakamura K, Nishikawa T (1987) Intercellular IgA dermatosis with clinical features of sub-corneal pustular dermatosis. Arch Dermatol 123: 10621065

    Google Scholar 

  • Sanchez NP, Perry HO, Muller SA, Winkelmann RK (1983) Subcorneal pustular dermatosis and pustular psoriasis. A clinicopathologic correlation. Arch Dermatol 119: 715–721

    Google Scholar 

  • Sneddon IB, Wilkinson DS (1956) Subcorneal pustular dermatosis. Br J Dermatol 68: 385–394

    Article  PubMed  CAS  Google Scholar 

  • Wallach D, Janssen F, Vignon-Pennamen MD, LemarchandVenencie F, Cottenot F (1987) Atypical neutrophilic dermatosis with subcorneal IgA deposits. Arch Dermatol 123: 790–795

    Article  PubMed  CAS  Google Scholar 

  • Freeman Rg, Spiller R, Knox JM (1960) Histopathology of ery- thema toxicum neonatorum. Arch Dermatol 82: 586–589

    Article  Google Scholar 

  • Ramamurthy RS, Reveri M, Esterly NB, Fretzin DF, Pildes RS (1976) Transient neonatal pustular melanosis. J Pediatr 88: 831–835

    Article  PubMed  CAS  Google Scholar 

  • Vignon-Pennamen MD, Wallach D (1986) Infantile acropustulosis. A clinicopathologic study of six cases. Arch Dermatol 122: 1155–1160

    Google Scholar 

  • Weedon D (1997) Vesiculobullous pattern. In: Weedon D (ed) Skin pathology. Churchill Livingstone, Edinburgh, pp 109–145

    Google Scholar 

  • Moreau A, Dompmartin A, Castel B, Remond B, Leroy D (1995) Drug-induced acute generalized exanthematous pustulosis with positive patch tests. Int J Dermatol 34: 263–266

    Article  PubMed  CAS  Google Scholar 

  • Roujeau JC, Bioulac-Sage P, Bourseau C et al. (1991) Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol 127: 1333–1338

    Article  PubMed  CAS  Google Scholar 

  • Spencer JM, Silvers DN, Grossman ME (1994) Pustular eruption after drug exposure: Is it pustular psoriasis or a pustular drug eruption? Br J Dermatol 130: 514–519

    Article  PubMed  CAS  Google Scholar 

  • Tan RSH (1974) Acute generalized pustular bakterid. Br J Dermatol 91: 209–215

    Article  PubMed  CAS  Google Scholar 

  • Pye RJ, Peachey RDG, Burton JL (1979) Erosive pustular dermatosis of the scalp. Br J Dermatol 100: 559–566

    Article  PubMed  CAS  Google Scholar 

  • Marzano AV, Capsoni F, Berti E, Gasparini G, Bottelli S, Caputo R (1996) Amicrobial pustular dermatosis of cutaneous folds associated with autoimmune disorders: A new entity? Dermatology 193: 88–93

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. D. Metze
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Universitätsklinik für Dermatologie und Venerologie, Karl-Franzens-Universität Graz, Auenbruggerplatz 8, A-8036, Graz, Österreich

    Helmut Kerl  & Lorenzo Cerroni  & 

  2. Universitäts-Hautklinik, Eberhard-Karls-Universität Tübingen, Liebermeisterstraße 25, D-72076, Tübingen, Deutschland

    Claus Garbe

  3. Klinik für Dermatologie und Venerologie, Universität zu Lübeck, Ratzeburger Allee 160, D-23562, Lübeck, Deutschland

    Helmut H. Wolff

Rights and permissions

Reprints and permissions

Copyright information

© 2003 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Metze, D. (2003). Krankheiten mit intraepidermalen Blasen und Pusteln. In: Kerl, H., Garbe, C., Cerroni, L., Wolff, H.H. (eds) Histopathologie der Haut. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-07784-9_13

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-07784-9_13

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-07785-6

  • Online ISBN: 978-3-662-07784-9

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation