Detection of Type VII Collagen Autoantibodies Before the Onset of Bullous Systemic Lupus Erythematosus

Abstract

Importance Anti-type VII collagen autoantibodies are often detectable in patients with bullous systemic lupus erythematosus (BSLE); however their timing of appearance preceding onset of disease is unknown. Observations We report the case of a 50-year-old female with a history of systemic lupus erythematosus who presented with vesicles and bullae around her lips, trunk, axillae, arms, and thighs. Histologic analysis as well as immunofluorescence and immunoblot studies confirmed the diagnosis of BSLE. Immunoblotting and ELISA studies of the patient’s serum obtained three months prior to the onset of BSLE showed presence of anti-type VII collagen autoantibodies. Levels of anti-type VII collagen IgG increased after bullous lesions appeared. Within one month after initiating dapsone and increasing the dose of prednisone, skin lesions promptly resolved. A year after onset of BSLE, her anti-type VII collagen IgG decreased below levels observed prior to the inception of her bullous lesions. Conclusions and Relevance This study shows that anti-type VII collagen autoantibodies can precede the clinical appearance of BSLE. The subsequent increase and decrease in the levels of circulating anti-type VII collagen autoantibodies, which mirrored skin disease activity, support a potential role in their initiation of disease.

Recurrent oral ulcers and blisters in a young woman

“…BSLE is caused by autoantibodies to the dermoepidermal junction, mainly the NC1 domain of type VII collagen, which is also a target antigen of epidermolysis bullosa acqusita (EBA). Anti‐type VII collagen autoantibodies can precede the clinical appearance of BSLE . IgG is found most frequently, followed by IgA and IgM.…”

“…Anti-type VII collagen autoantibodies can precede the clinical appearance of BSLE. 5 IgG is found most frequently, followed by IgA and IgM. Blisters are primarily present on sun-exposed areas, and show a predilection for the trunk, supraclavicular area, arms, face, vermillion border and oral mucosa.…”

Basement membrane collagens and disease mechanisms

“…These anchoring fibrils mediate dermal–epidermal adhesion via binding of collagen VII to laminin-332 and collagen IV in the BM, and collagen I in the interstitial matrix [8]. Auto-antibodies against epitopes in the NC-1 domain cause the autoimmune skin blistering disorders epidermolysis bullosa acquisita (EBA) [85] and bullous systemic lupus erythematosus [86].…”

Systemischer Lupus erythematodes

“…Ein "u-serrated" Muster wird bei Bindung von AntiTyp VII Kollagen Antikörpern gefunden; dieses zeigt vermutlich den Verlauf der Ankerfibrillen von den Ankerplatten in die Lamina densa an bran verankert. Sie bilden daher einen wesentlichen funktionellen Mechanismus der dermoepidermalen Adhäsion [27,28]. Nur ganz selten werden beim bullösen SLE andere Autoantigene der Basalmembran als Zielstrukturen von zirkulierenden Autoantikörpern nachgewiesen, dazu zählen Laminin-332, Laminin-311 (früher als Laminin 6 bezeichnet) oder das bullöse Pemphigoidantigen II (BP-230-Antigen) [29].…”