ABSTRACT
BACKGROUND: Titres of circulating autoantibodies detected by indirect immunofluorescence (IIF) have been used for the diagnosis and evaluation of disease activity in bullous pemphigoid (BP). In BP, the major pathogenic epitope is known to be the non-collagenous extracellular domain (NC16A) of the 180-kDa transmembrane hemidesmosomal protein (BPAG2). Recently, an enzyme-linked immunosorbent assay (ELISA) kit using the NC16A domain recombinant protein (BP180 ELISA kit) has become commercially available to measure the quantities of pathogenic autoantibodies circulating in BP patients. OBJECTIVE: To investigate the correlation of clinical severity and ELISA indices in BP. METHODS: Fourteen patients with a typical form of BP and one refractory BP patient who died despite extensive treatment were included in this study. Antibody titres in sera from these patients were measured using BP180 ELISA kit and an analysis of ELISA indices with disease activity was performed. RESULTS: ELISA indices were significantly reduced after successful therapy, although IIF titres did not always show apparent correlations. In the patient with refractory BP, ELISA indices also showed a good correlation with disease course. ELISA indices measured using the BP180 ELISA kit were well correlated with the disease activity. CONCLUSION: This commercially available kit more closely followed disease activities than the IIF titres. The BP ELISA system may be a useful tool to evaluate the disease activity and to assess the effectiveness of the treatment of BP.
Subject(s)
Autoantigens/chemistry , Autoantigens/immunology , Chemistry, Clinical/methods , Enzyme-Linked Immunosorbent Assay/methods , Pemphigoid, Bullous/diagnosis , Autoantigens/analysis , Epitopes , Fluorescent Antibody Technique, Indirect , Humans , Male , Middle Aged , Non-Fibrillar Collagens , Pemphigoid, Bullous/metabolism , Protein Structure, Tertiary , Recombinant Proteins/chemistry , Remission Induction , Time Factors , Collagen Type XVIIABSTRACT
Annular lichen planus is a rare, unique subtype of lichen planus. We report a 34-year-old Japanese male who had multiple, dark red to purple, annular macules with slightly raised borders. Histopathological examination of a skin biopsy specimen from the peripheral region of the macule showed the typical features of lichen planus. Immunohistochemical stainings revealed that a number of CD1a-positive, S-100-protein-positive Langerhans cells were present at the border zone of the annular lesion and most of the infiltrating cells were CD4-positive, CD8-negative lymphocytes. Conversely, inside the annular lesion, the number of Langerhans cells was decreased, and an equal number of CD4-positive cells and CD8-positive cells was present in the sparse infiltration. These results suggest that activation of Langerhans cells initiated a lichenoid tissue reaction but a subsequent depletion of Langerhans cells suppressed the lymphocyte infiltration. These sequential events might be involved in the formation of the unique annular structure in this condition.
