Scarring autoimmune bullous disease in a Ugandan patient with autoantibodies to BP180, BP230, and laminin 5 - 09/08/11

Doi : 10.1016/j.jaad.2005.03.054

Grace K. Mulyowa, MD ^a,^b, Gerold Jaeger, MD ^b, Cassian Sitaru, MD ^c, Eva-B. Bröcker, MD ^a, Detlef Zillikens, MD ^c, Enno Schmidt, MD, PhD ^a,^⁎
^a From the Departments of Dermatology, University of Würzburg
^b Mbarara University of Science and Technology
^c University of Schleswig-Holstein, Campus Lübeck

Reprint requests: Enno Schmidt, MD, PhD, Department of Dermatology, University of Würzburg, Josef-Schneider-Str 2, 97080 Würzburg, Germany.

Würzburg and Lübeck, Germany, and Mbarara, Uganda

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 ⍺3 and γ2 chains of laminin 5. No reactivity was detected with type VII collagen, ⍺6β4 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.

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Abbreviations used : BP180, BP230, CP, FL, IF, LAD-1, MMP, NC, NC16A

Plan

Case report

Discussion

Supported by Stiefel Laboratories.
Supported by grants from the Germany Academic Exchange Service (DAAD A/01/20559 to G. K. M.), Wilhelm Sander-Stiftung (98. 078. 2 to D. Z.), and the German Society for Dermatology in the Tropics (to E. S.).
Conflicts of interest: None identified.
Presented at the Annual Meeting of the German Society of Tropical Medicine (DGT), Würzburg, Germany, September 23-25, 2004.