Anti‐ <scp>p200</scp> Pemphigoid in an Infant Clinically Mimicking Linear <scp>IgA</scp> Disease

We report a 10-month-old boy with annular/polycyclic vesicles on his face, neck, trunk, and extremities that clinically and histomorphologically indicated linear IgA disease (LAD)/chronic bullous disease of childhood (CBDC); however, the immunofluorescence findings and serum positivity for anti-laminin β4 antibodies were consistent with anti-p200 pemphigoid. His condition improved with prednisolone but recurred during tapering; remission was achieved following dapsone administration. Anti-p200 pemphigoid often resembles bullous pemphigoid clinically and is characterized similarly by subepidermal bullae with linear IgG antibody reactivity and C3 basement membrane zone (BMZ) localization, but differs in its target antigen.

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Publication Details

Journal
Pediatric Dermatology
Published
2026-04-13
DOI
https://doi.org/10.1111/pde.70212
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
Field-Weighted Citation Impact
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article

Anti‐ p200 Pemphigoid in an Infant Clinically Mimicking Linear IgA Disease

Elek Wellman, Kristin M. Leiferman, Morgan L. Wilson, Joseph Conlon
Pediatric Dermatology
Autoimmune Bullous Skin Diseases
article

Anti‐ p200 Pemphigoid in an Infant Clinically Mimicking Linear IgA Disease

Elek Wellman, Kristin M. Leiferman, Morgan L. Wilson, Joseph Conlon
article en

Abstract

We report a 10-month-old boy with annular/polycyclic vesicles on his face, neck, trunk, and extremities that clinically and histomorphologically indicated linear IgA disease (LAD)/chronic bullous disease of childhood (CBDC); however, the immunofluorescence findings and serum positivity for anti-laminin β4 antibodies were consistent with anti-p200 pemphigoid. His condition improved with prednisolone but recurred during tapering; remission was achieved following dapsone administration. Anti-p200 pemphigoid often resembles bullous pemphigoid clinically and is characterized similarly by subepidermal bullae with linear IgG antibody reactivity and C3 basement membrane zone (BMZ) localization, but differs in its target antigen.

Pediatric Dermatology
Southern Illinois University School of Medicine (US), University of Utah (US), ARUP Laboratories (United States) (US), Springfield Clinic (US)
Openalex Percentile: Top 24%
Autoimmune Bullous Skin Diseases
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