Pemphigoid nodularis associated with autoantibodies to the NC16A domain of BP180 and a hyperproliferative integrin profile

Journal of the American Academy of Dermatology
M SchachterL S Chan

Abstract

Pemphigoid nodularis, a rare variant of bullous pemphigoid, has clinical features resembling prurigo nodularis, with blisters arising from normal-appearing or nodular skin. The fine antigenic epitope of the autoantibodies and the mechanism accounting for the nodular phenotype has not been delineated. We describe a patient with pemphigoid nodularis that fulfilled the criteria of bullous pemphigoid by histopathologic examination and direct and indirect immunofluorescence studies. Immunopathologic examination also revealed in situ deposition and circulating autoantibodies of all IgG subclasses, except IgG3, and both light chains to the patient's skin basement membrane. By immunoblotting, the patient's IgG autoantibodies labeled BP180, BP230, and an unidentified 150-kd epidermal protein and mapped the BP180 epitope to the MCW-1, region 2 of the NC16A domain. The nodular plaque skin showed expression of alpha-6 and beta-1 integrin subunits, mediators of matrix-cell signaling and proliferation, at the basal and the suprabasal epidermis, a pattern found in psoriasis, which is the prototype of hyperproliferative dermatoses.

Citations

Apr 4, 2006·Molecular and Cellular Biology·Kevin WilhelmsenArnoud Sonnenberg
Oct 4, 2005·The Australasian Journal of Dermatology·Michael R Lee, Stephen Shumack
Nov 18, 2003·Clinical and Experimental Dermatology·Y Sakuma-OyamaM M Black
Apr 19, 2005·Journal of Nippon Medical School = Nippon Ika Daigaku Zasshi·Hirotsugu TashiroSeiji Kawana
May 22, 2016·Journal of Cutaneous Medicine and Surgery·Björn DangelGisela Metzler
Sep 23, 2018·Clinical and Experimental Dermatology·M HattoriO Ishikawa
May 17, 2005·Drugs·Scott R A WalshP Régine Mydlarski
Jan 27, 2019·Experimental and Therapeutic Medicine·Corina VornicescuLoredana Ungureanu
Jan 11, 2018·International Journal of Dermatology·Kozo YonedaTakashi Hashimoto

❮ Previous
Next ❯

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Basement Membranes

Basement membranes are thin, specialized extracellular matrices surrounding most tissues in all metazoans. Here is the latest research on basement membranes.

Adhesion Molecules in Health and Disease

Cell adhesion molecules are a subset of cell adhesion proteins located on the cell surface involved in binding with other cells or with the extracellular matrix in the process called cell adhesion. In essence, cell adhesion molecules help cells stick to each other and to their surroundings. Cell adhesion is a crucial component in maintaining tissue structure and function. Discover the latest research on adhesion molecule and their role in health and disease here.