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Observation
March 20, 2019

Overlap of Bullous, Anti–Laminin-332, and Anti-p200 Pemphigoid With Concomitant Anti–Contactin-1–Positive Inflammatory Polyneuropathy Treated With Intravenous Immunoglobulins as a Manifestation of Epitope Spreading

Author Affiliations
  • 1Department of Dermatology, University Hospital of Heidelberg, Heidelberg, Germany
  • 2Department of Dermatology, University Hospital of Lübeck, Lübeck, Germany
  • 3Department of Neurology, University of Heidelberg, Heidelberg, Germany
  • 4Laboratory for Clinical Immunology, Lübeck, Germany
JAMA Dermatol. 2019;155(5):631-633. doi:10.1001/jamadermatol.2018.5536

Bullous pemphigoid (BP), anti-p200 pemphigoid, and anti–laminin-332 mucous membrane pemphigoid are distinct subepidermal autoimmune bullous diseases (AIBDs).1 Anti–contactin-1 (anti-CNTN1)–positive inflammatory polyneuropathy (IP) is a recently described entity with clinical similarities to chronic inflammatory demyelinating polyneuropathy, which manifests pathologically as a disruption of the paranodal architecture.2,3 The patient in this case initially presented with BP, and after having been successfully treated and living symptom-free for 5 years, he developed an overlap of BP, anti-p200 pemphigoid, and anti–laminin-332 mucous membrane pemphigoid with an anti-CNTN1–positive IP in close temporal proximity. We hypothesize an underlying disease-modifying intermolecular epitope-spreading (ES) phenomenon.

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