Skip to main content
SpringerLink
Log in

Lineare IgA-Dermatose

Linear IgA bullous dermatosis

  • Leitthema
  • Published:
Der Hautarzt Aims and scope Submit manuscript

Zusammenfassung

Die lineare Ig(Immunglobulin)A-Dermatose gehört zu den seltenen blasenbildenden Autoimmundermatosen und kann sich sowohl im Kindesalter als auch im Erwachsenenalter manifestieren. Perlschnurartig/rosettenartig angeordnete pralle Blasen („crown of jewels“) und urtikarielle Plaques, begleitet von starkem Juckreiz, können am ganzen Integument mit Betonung des Kopfes und der anogenitalen Region auftreten. Namensgebend ist die direkte Immunfluoreszenz (DIF), die eine lineare Anordnung von IgA-Autoantikörper entlang der Basalmembran zeigt. Eine histologische und klinische Ähnlichkeit zur Dermatitis herpetiformis Duhring ist gegeben.

Abstract

Linear IgA bullous dermatosis is a rare autoimmune blistering disease that occurs in both children and adults. Strings of pearls, crowns of jewels, rosettes and urticarial plaques can occur on the whole integument with emphasis on the face (particularly perioral area) and genitalia. Pruritus is common and may be severe. The presence of IgA deposits along the basement membrane can usually be identified using direct immunofluorescence (DIF) microscopy. The histological and clinical features of this disorder may mimic those of dermatitis herpetiformis.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4

Literatur

  1. Bowen J (1901) Six cases of bulls dermatosis following vaccination, and resembling dermatitis herpetiformis. J Cutan Dis 19:401–423

    Google Scholar 

  2. Bowen J (1905) Dermatitis herpetiformis in children. J Cutan Dis 23:381–401

    Google Scholar 

  3. Chorzelski TP, Jablonska S (1979) IgA linear dermatosis of childhood (chronic bullous disease of childhood). Br J Dermatol 101(5):535–542

    Article  CAS  Google Scholar 

  4. Wojnarowska F, Marsden RA, Bhogal B, Black MM (1988) Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol 19(5 Pt 1):792–805

    Article  CAS  Google Scholar 

  5. Fortuna G, Marinkovich MP (2012) Linear immunoglobulin A bullous dermatosis. Clin Dermatol 30(1):38–50. https://doi.org/10.1016/j.clindermatol.2011.03.008

    Article  PubMed  Google Scholar 

  6. Collier PM, Wojnarowska F, Welsh K, McGuire W, Black MM (1999) Adult linear IgA disease and chronic bullous disease of childhood: the association with human lymphocyte antigens Cw7, B8, DR3 and tumour necrosis factor influences disease expression. Br J Dermatol 141(5):867–875

    Article  CAS  Google Scholar 

  7. Sitaru C, Goebeler M, Zillikens D (2004) Bullous autoimmune dermatoses (I): Pathogenesis and diagnosis. J Dtsch Dermatol Ges 2(2):123–128 (quiz 139–140)

    Article  Google Scholar 

  8. Lara-Corrales I, Pope E (2010) Autoimmune blistering diseases in children. Semin Cutan Med Surg 29(2):85–91. https://doi.org/10.1016/j.sder.2010.03.005

    Article  CAS  PubMed  Google Scholar 

  9. Taniguchi T, Maejima H, Saito N, Katsuoka K, Haruki S (2009) Case of linear IgA bullous dermatosis-involved ulcerative colitis. Inflamm Bowel Dis 15(9):1284–1285. https://doi.org/10.1002/ibd.20795

    Article  PubMed  Google Scholar 

  10. Jouan N, Plantin P, Berthou C, Gavanou J, Le Roy JP, Schollhammer M et al (1992) Association of IgA linear dermatitis and non-Hodgkin’s malignant lymphoma. Rev Med Interne 13(2):153–155

    Article  CAS  Google Scholar 

  11. Lacour JP, Vitetta A, Ortonne JP (1992) Linear IgA dermatosis and thyroid carcinoma. J Am Acad Dermatol 26(2 Pt 1):257–259

    Article  CAS  Google Scholar 

  12. Schmidt E, Zillikens D (2011) The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 108(23):399–405. https://doi.org/10.3238/arztebl.2011.0399 (i–iii)

    Article  PubMed  PubMed Central  Google Scholar 

  13. Mobacken H, Kastrup W, Ljunghall K, Lofberg H, Nilsson LA, Svensson A et al (1983) Linear IgA dermatosis: a study of ten adult patients. Acta Derm Venereol 63(2):123–128

    CAS  PubMed  Google Scholar 

  14. Smith SB, Harrist TJ, Murphy GF, Halperin AJ, Newell JB, Fallon JT et al (1984) Linear IgA bullous dermatosis v dermatitis herpetiformis. Quantitative measurements of dermoepidermal alterations. Arch Dermatol 120(3):324–328

    Article  CAS  Google Scholar 

  15. Guide SV, Marinkovich MP (2001) Linear IgA bullous dermatosis. Clin Dermatol 19(6):719–727

    Article  CAS  Google Scholar 

  16. Van L, Browning JC, Krishnan RS, Kenner-Bell BM, Hsu S (2018) Dermatitis herpetiformis: Potential for confusion with linear IgA bullous dermatosis on direct immunofluorescence. Dermatol Online J 14(1):21

    Google Scholar 

  17. Wojnarowska F, Collier PM, Allen J, Millard PR (1995) The localization of the target antigens and antibodies in linear IgA disease is heterogeneous, and dependent on the methods used. Br J Dermatol 132(5):750–757

    Article  CAS  Google Scholar 

  18. Cozzani E, Kanitakis J, Nicolas JF, Schmitt D, Thivolet J (1994) Comparative study of indirect immunofluorescence and immunoblotting for the diagnosis of autoimmune pemphigus. Arch Dermatol Res 286(6):295–299

    Article  CAS  Google Scholar 

  19. Hashimoto T, Ishiko A, Shimizu H, Tanaka T, Dodd HJ, Bhogal BS et al (1996) A case of linear IgA bullous dermatosis with IgA anti-type VII collagen autoantibodies. Br J Dermatol 134(2):336–339

    Article  CAS  Google Scholar 

  20. Ghohestani RF, Nicolas JF, Kanitakis J, Claudy A (1997) Linear IgA bullous dermatosis with IgA antibodies exclusively directed against the 180- or 230-kDa epidermal antigens. J Invest Dermatol 108(6):854–858

    Article  CAS  Google Scholar 

  21. Bhogal B, Wojnarowska F, Marsden RA, Das A, Black MM, McKee PH (1987) Linear IgA bullous dermatosis of adults and children: an immunoelectron microscopic study. Br J Dermatol 117(3):289–296

    Article  CAS  Google Scholar 

  22. Horiguchi Y, Ikoma A, Sakai R, Masatsugu A, Ohta M, Hashimoto T (2008) Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan. J Dermatol 35(11):737–743. https://doi.org/10.1111/j.1346-8138.2008.00561.x

    Article  CAS  PubMed  Google Scholar 

  23. Wojnarowska F, Kirtschig G, Khumalo N (2001) Treatment of subepidermal immunobullous diseases. Clin Dermatol 19(6):768–777

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg, Standort Marburg, Baldingerstr. 1, 35043, Marburg, Deutschland

    H. A. Juratli

  2. Klinik für Dermatologie, Venerologie und Dermatoonkologie, Fakultät für Medizin, Semmelweis Universität, Budapest, Ungarn

    M. Sárdy

Authors
  1. H. A. Juratli
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Sárdy
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to H. A. Juratli.

Ethics declarations

Interessenkonflikt

H.A. Juratli und M. Sárdy geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren. Alle Patienten, die über Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts zu identifizieren sind, haben hierzu ihre schriftliche Einwilligung gegeben.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Juratli, H.A., Sárdy, M. Lineare IgA-Dermatose. Hautarzt 70, 254–259 (2019). https://doi.org/10.1007/s00105-019-4377-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00105-019-4377-9

Schlüsselwörter

Keywords

Search

Navigation