U-serrated immunodeposition pattern differentiates type VII collagen targeting bullous diseases from other subepidermal bullous autoimmune diseases

Abstract

Using this pattern recognition by direct immunofluorescence microscopy we discovered several cases of EBA which would otherwise have been erroneously diagnosed as a form of pemphigoid or linear IgA disease.

Systemischer Lupus erythematodes

“…Auch wir können hier nicht eindeutig beweisen, dass Kollagen Typ VII das Autoantigen darstellt, allerdings ist das "u-serrated-Muster" der direkten IF ein sehr starker Hinweis darauf. Für manche Autoren gilt diese diagnostische Methode als Goldstandard der Epidermolysis bullosa acquisita/ des bullösen SLE [30][31][32][33] Autoimmundiagnostik: ANA positiv (1:2560), gesprenkeltes Fluoreszenzmuster, SS-A/Ro-Antikörper >240 U/ml (<10 U/ml), SS-B/La-Antikörper>320 U/ ml (<10 U/ml), Komplementfaktoren im Normbereich.…”

Clinical features and diagnosis of epidermolysis bullosa acquisita

“…The u-serrated pattern is pathognomonic for skin-bound autoantibodies against type VII collagen as found in EBA and bullous systemic lupus erythematosus [26,124]. In all other pemphigoid diseases, a n-serrated pattern characterized by arches closed at the top giving a 'snake-like' appearance [110,124,125]. However, in mucosal biopsies and a number of skin samples, no u-or n-serrated pattern can be seen.…”

“…Importantly, by close inspection of thin sections of 4-6 µm, Vodegel et al could show that IgG/IgA linear deposits at the BMZ show a subtle but well remarkable pattern described as u-serrated [110]. This pattern is characterized by arches that are closed at the bottom giving the appearance of 'growing grass' or 'upstanding arms' (Figure 6).…”

Estudio retrospectivo de las características clínicas, histológicas e inmunológicas en una serie de 9 pacientes con epidermólisis ampollosa adquirida

“…Si los depósitos se sitúan en la sublámina densa (EAA) el patrón serrado adopta una configuración en u (u-serrado). Esta técnica requiere que los cortes sean de menos de 4 m de grosor 23 .…”