Dermal dendritic cell number correlates with serum autoantibody titers in Brazilian pemphigus foliaceus patients

M.P.V Chiossi,R.S Costa,A.M.F Roselino

doi:10.1590/s0100-879x2004000300008

Abstract

Pemphigus foliaceus (PF) is an autoimmune bullous disease endemic in Brazil. Since serum IL-12 is increased in patients with PF and Langerhans cells (LC) produce IL-12, we titrated serum autoantibodies by indirect immunofluorescence, and quantified epidermal dendritic cells, known as LC, and dermal dendritic cells (DC). Biopsies of blistering lesions were obtained from 22 patients, 13 of whom were submitted to biopsy of both injured and of apparently healthy skin. The control groups consisted of skin from 8 cadavers and from 12 women submitted to breast plastic surgery. LC and DC were identified with anti-CD1a antibody and quantified by morphometric analysis. LC number in the lesion and in apparently healthy skin from PF patients was similar to that of both control groups. DC number in the injured skin (median=0.94 DC/mm basement membrane) was higher than that of the cadaver group (median=0.13 DC/mm basement membrane). In the 13 patients with biopsies of both injured and apparently healthy skin, LC and DC were present in larger numbers in the lesion. There was a direct correlation between DC number in the lesion of the PF group and serum autoantibody titers. This correlation was not observed for LC number. The increased number of DC in the lesion, as well as its direct correlation with serum autoantibody titers suggest the participation of DC in the pathogenesis of PF. The relationship between increased DC number and IL-12 in PF needs to be clarified.

Highlights

Pemphigus foliaceus (PF) is an autoimmune bullous disease characterized by acantholysis in the lower layer of the stratum corneum of the epidermis, deposition of immunoglobulin G (IgG) in the intercellular space of the epidermis, and circulating IgG against the intercellular substance, as demonstrated by indirect immunofluorescence (IIF)
PF is endemic in Brazil and we recently demonstrated that the northeastern region of the State of São Paulo is a remaining focus of PF [3,4]
The Langerhans cells (LC) population is reduced in the epidermis of PF patients and the number of dermal dendritic cells (DC) is higher in the lesional skin than in the perilesional skin [7]

Summary

Introduction

Pemphigus foliaceus (PF) is an autoimmune bullous disease characterized by acantholysis in the lower layer of the stratum corneum of the epidermis, deposition of immunoglobulin G (IgG) in the intercellular space of the epidermis, and circulating IgG against the intercellular substance, as demonstrated by indirect immunofluorescence (IIF). The LC population is reduced in the epidermis of PF patients and the number of dermal DC is higher in the lesional skin than in the perilesional skin [7]. Since serum IL-12 is increased in PF [10] and DC, which produce IL-12, have an antigen-presenting function, the purpose of the present study was to quantify LC and DC in the skin of patients with PF and to attempt to correlate their numbers with circulating serum autoantibody titers.

Objectives

Results

Conclusion