Anti-laminin 5 mucous membrane pemphigoid

Sandrine Benoit; Enno Schmidt; Cassian Sitaru; Christian Rose; Matthias Goebeler; Eva B. Bröcker; Detlef Zillikens

doi:10.1111/j.1610-0387.2005.05826.x

Anti-laminin 5 mucous membrane pemphigoid

Sandrine Benoit^*, Enno Schmidt, Cassian Sitaru, Christian Rose, Matthias Goebeler, Eva B. Bröcker, Detlef Zillikens

^*Corresponding author for this work

5 Citations (Scopus)

Abstract

A 70-year-old patient presented erosions on the oral mucosa and skin, predominantly in perianal and inguinal areas. Histopathologically, subepidermal blistering was observed. By direct immunofluorescence microscopy, linear deposits of IgG and C₃ were seen at the dermal-epidermal junction. By indirect immunofluorescence microscopy, binding of IgG autoantibodies to the dermal side of NaCI-split skin was detected. These autoantibodies were shown to target both the non-processed and processed α₃- as well as the β₃-subunit of laminin 5 by immunoblot analysis using extracellular matrix of cultured human keratinocytes. Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement.

Translated title of the contribution	Schleimhautpemphigoid mit autoantikörpern gegen laminin 5
Original language	English
Journal	JDDG - Journal of the German Society of Dermatology
Volume	4
Issue number	1
Pages (from-to)	41-44
Number of pages	4
ISSN	1610-0379
DOIs	https://doi.org/10.1111/j.1610-0387.2005.05826.x
Publication status	Published - 01.2006

Research Areas and Centers

Academic Focus: Center for Infection and Inflammation Research (ZIEL)

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10.1111/j.1610-0387.2005.05826.x

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title = "Anti-laminin 5 mucous membrane pemphigoid",

abstract = "A 70-year-old patient presented erosions on the oral mucosa and skin, predominantly in perianal and inguinal areas. Histopathologically, subepidermal blistering was observed. By direct immunofluorescence microscopy, linear deposits of IgG and C3 were seen at the dermal-epidermal junction. By indirect immunofluorescence microscopy, binding of IgG autoantibodies to the dermal side of NaCI-split skin was detected. These autoantibodies were shown to target both the non-processed and processed α3- as well as the β3-subunit of laminin 5 by immunoblot analysis using extracellular matrix of cultured human keratinocytes. Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement.",

author = "Sandrine Benoit and Enno Schmidt and Cassian Sitaru and Christian Rose and Matthias Goebeler and Br{\"o}cker, {Eva B.} and Detlef Zillikens",

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language = "English",

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T1 - Anti-laminin 5 mucous membrane pemphigoid

AU - Benoit, Sandrine

AU - Schmidt, Enno

AU - Sitaru, Cassian

AU - Rose, Christian

AU - Goebeler, Matthias

AU - Bröcker, Eva B.

AU - Zillikens, Detlef

PY - 2006/1

Y1 - 2006/1

N2 - A 70-year-old patient presented erosions on the oral mucosa and skin, predominantly in perianal and inguinal areas. Histopathologically, subepidermal blistering was observed. By direct immunofluorescence microscopy, linear deposits of IgG and C3 were seen at the dermal-epidermal junction. By indirect immunofluorescence microscopy, binding of IgG autoantibodies to the dermal side of NaCI-split skin was detected. These autoantibodies were shown to target both the non-processed and processed α3- as well as the β3-subunit of laminin 5 by immunoblot analysis using extracellular matrix of cultured human keratinocytes. Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement.

AB - A 70-year-old patient presented erosions on the oral mucosa and skin, predominantly in perianal and inguinal areas. Histopathologically, subepidermal blistering was observed. By direct immunofluorescence microscopy, linear deposits of IgG and C3 were seen at the dermal-epidermal junction. By indirect immunofluorescence microscopy, binding of IgG autoantibodies to the dermal side of NaCI-split skin was detected. These autoantibodies were shown to target both the non-processed and processed α3- as well as the β3-subunit of laminin 5 by immunoblot analysis using extracellular matrix of cultured human keratinocytes. Based on these results, the diagnosis of an anti-laminin 5 mucous membrane pemphigoid was established. Oral treatment with dapsone, combined with intravenous dexamethasone pulse therapy, led to rapid improvement.

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Anti-laminin 5 mucous membrane pemphigoid

Abstract

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