Scarring autoimmune bullous disease in a Ugandan patient with autoantibodies to BP180, BP230, and laminin 5

Grace K. Mulyowa, Gerold Jaeger, Cassian Sitaru, Eva B. Bröcker, Detlef Zillikens, Enno Schmidt*

*Corresponding author for this work
16 Citations (Scopus)

Abstract

We report on a 24-year-old, male Ugandan patient with a 2-week history of itchy papules, vesicles, erosions, and crusts distributed on the entire body, accompanied by minor erosions on the palate, tongue, and lower lip. Conjunctivae and genital mucosa were not involved. Circulating IgG and IgA autoantibodies were found against recombinant full-length BP180, BP180 4575, and the C-terminus of BP230. In addition, IgG reactivity was observed against the 16th noncollagenous region of the BP180 ectodomain, the cell-derived soluble ectodomain of BP180 (linear IgA disease antigen 1), and the α3 and γ2 chains of laminin 5. No reactivity was detected with type VII collagen, α6β4 integrin, and the p200 protein. Oral prednisolone and dapsone led to clearance of lesions that mostly healed with scarring and milia formation. Here, we describe a scarring mucocutaneous variant of an autoimmune blistering skin disorder that extends the current clinical and immunopathologic spectrum of this group of diseases.

Original languageEnglish
JournalJournal of the American Academy of Dermatology
Volume54
Issue number2 SUPPL.
Pages (from-to)S43-S46
ISSN0190-9622
DOIs
Publication statusPublished - 02.2006

Research Areas and Centers

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

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