Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3

Marcel F. Jonkman, Jacqueline Schuur, Freark Dijk, Klaas Heeres, Marcelus C J M De Jong, Jan B. Van Der Meet, Kim B. Yancey, Hendri H. Pas

Research output: Contribution to journalArticle

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Abstract

Background: The inflammatory variant of epidermolysis bullosa acquisita (EBA) may clinically closely resemble bullous or cicatricial pemphigoid. Patients with inflammatory EBA have IgG autoantibodies against type VII collagen. Patients with anti-epiligrin cicatricial pemphigoid have IgG autoantibodies against laminin 5. Observation: We describe a patient with inflammatory EBA exhibiting nonscarring oral and vaginal involvement. Indirect immunofluorescence using skin substrate lacking an epidermal basement membrane molecule, direct immunoelectron microscopy, immunoblot, and immunoprecipitation studies revealed the simultaneous presence of circulating IgG autoantibodies against type VII collagen and laminin α3. A final diagnosis of EBA was based on the sublamina densa level of blister formation. Conclusion: This case illustrates the clinical and immunological overlap between EBA and anti-epiligrin cicatricial pemphigoid, a unique finding that may have developed as a consequence of epitope spreading.

Original languageEnglish (US)
Pages (from-to)227-231
Number of pages5
JournalArchives of Dermatology
Volume136
Issue number2
StatePublished - Feb 2000

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Epidermolysis Bullosa Acquisita
Collagen Type VII
Laminin
Autoantibodies
Benign Mucous Membrane Pemphigoid
Immunoglobulin G
Bullous Pemphigoid
Immunoelectron Microscopy
Blister
Indirect Fluorescent Antibody Technique
Immunoprecipitation
Basement Membrane
Epitopes
Observation
Skin
kalinin

ASJC Scopus subject areas

  • Dermatology

Cite this

Jonkman, M. F., Schuur, J., Dijk, F., Heeres, K., De Jong, M. C. J. M., Van Der Meet, J. B., ... Pas, H. H. (2000). Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3. Archives of Dermatology, 136(2), 227-231.

Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3. / Jonkman, Marcel F.; Schuur, Jacqueline; Dijk, Freark; Heeres, Klaas; De Jong, Marcelus C J M; Van Der Meet, Jan B.; Yancey, Kim B.; Pas, Hendri H.

In: Archives of Dermatology, Vol. 136, No. 2, 02.2000, p. 227-231.

Research output: Contribution to journalArticle

Jonkman, MF, Schuur, J, Dijk, F, Heeres, K, De Jong, MCJM, Van Der Meet, JB, Yancey, KB & Pas, HH 2000, 'Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3', Archives of Dermatology, vol. 136, no. 2, pp. 227-231.
Jonkman MF, Schuur J, Dijk F, Heeres K, De Jong MCJM, Van Der Meet JB et al. Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3. Archives of Dermatology. 2000 Feb;136(2):227-231.
Jonkman, Marcel F. ; Schuur, Jacqueline ; Dijk, Freark ; Heeres, Klaas ; De Jong, Marcelus C J M ; Van Der Meet, Jan B. ; Yancey, Kim B. ; Pas, Hendri H. / Inflammatory variant of epidermolysis bullosa acquisita with IgG autoantibodies against type VII collagen and laminin α3. In: Archives of Dermatology. 2000 ; Vol. 136, No. 2. pp. 227-231.
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