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Pemphigus vulgaris has never before been associated with silicosis, although there are many reports of silicosis accompanied by several autoimmune diseases such as progressive systemic sclerosis, systemic lupus erythematosus, dermatomyositis or rheumatoid arthritis. We observed a patient with pemphigus vulgaris accompanied with silicosis. The patient was a 75-year-old man with a 2-month history of repeated oral erosions and blisters on the back, thighs and axillas. Histological examination showed suprabasal cleavage with acantholysis. Immunoblotting analysis demonstrated binding of the patient’s serum to the 130-kD pemphigus vulgaris antigen (desmoglein 3) and the 160-kD pemphigus foliaceus antigen (desmoglein 1). The patient has radiographically been diagnosed as having silicosis. An elevated serum IgG, antinuclear antibody, anti-ssDNA, antimicrosomal antibodies and a biologically false-positive reaction to the Wassermann test were also detected. Although the clinical symptoms improved after treatment with systemic steroids, the patient died due to pneumonia. This is the first reported case in which the characteristics of both pemphigus vulgaris and silicosis could be detected.

We describe a case of Sjögren's syndrome who repeatedly developed annular erythema on her extremities. Her anti-nuclear antibody, anti-SSA/Ro antibody, and anti-SSB/La antibody were all negative. Characteristics of the annular erythema included a tendency to appear on the extremities especially in summer, spontaneous regression after 1-2 weeks, and residual slight pigmentation. The histological findings revealed dermal perivascular lymphocytic infiltration admixed with some neutrophils. Slight exsudative changes were found in the upper dermis. There were no epidermal changes. This case suggests the existence of annular erythema which may not be related to the anti-SSA/Ro or anti-SSB/La antibody. Unknown factors other than those antibodies may be involved in the pathogenesis of the annular erythema.

Bullous pemphigoid (BP) is an autoimmune blistering skin disease caused by autoantibody against the hemidesmosomal protein BP180. B regulatory cells (Bregs) are crucial in maintaining self-tolerance and suppressing autoantibody production. Blood samples were collected from thirty-six BP patients and thirty healthy volunteers. The number of CD19 + CD24 hi CD27 + Bregs was analyzed by flow cytometry. Elisa assay was performed to detect the level of anti-BP180 autoantibodies produced by patient-derived peripheral blood mononuclear cells (PBMCs) with Bregs deletion or without. In addition, T cells were cultured with autogenous Bregs from BP patients or healthy controls respectively, then the proliferation and cytokine productions in these T cells were detected by flow cytometry. Finally, the expressions of IL-10 and TNF-a in Bregs were further examined. Then etanercept was added into the culture of patients-derived PBMCs to investigate the role of TNF-a in autoantibody production. The frequency of circulating CD19 + CD24 hi CD27 + Bregs increased in BP patients compared with healthy controls. However, patient-derived Bregs were impaired in suppressing the production of specific anti-BP180 autoantibodies. These patients-derived Bregs were also defective in suppressing CD4 + T cell activation. Further, patient-derived Bregs produced comparable level of IL-10, while aberrantly secreted TNF-a. Etanercept could inhibit the autoantibody production in patient-derived PBMCs. Our results suggested that BP patient-derived Bregs were impaired in suppressing CD4 + T cell activation in a manner of aberrant secretion of TNF-a, rather than IL-10, thus contributing to the autoantibody production.

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