Bullous Pemphigoid Associated with Silicosis

Abstract

Bullous pemphigoid (BP) has never before been reported to associate with silicosis, although there are numerous reports of silicosis accompanied by different autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, dermatomyositis or rheumatoid arthritis. We report on a 63-year-old Japanese patient with silicosis who developed tensed bullae, erosions and macular pigmentation on the trunk and extremities. Indirect immunofluorescence revealed anti-basement-membrane-zone antibodies; immunoblotting analysis demonstrated that the patient’s serum reacted with the 230-kD BP antigen in the epidermal extracts, as well as a recombinant protein of the NC16a domain of 180-kD BP antigen. Clinical symptoms improved after treatment with systemic steroids. To the best of our knowledge, this is the first reported case of BP associated with silicosis.

Environmental factors producing autoimmune dysregulation – Chronic activation of T cells caused by silica exposure

“…In addition, we detected anti-topoisomerase I Ueki et al 2001aUeki et al ,b, 2002, anti-desmoglein (Ueki et al 2000(Ueki et al , 2001a, anti-caspase 8 , and anti-CD95/Fas (Takata- Tomokuni et al 2005) antibodies. Particularly for the detection of anti-caspase 8 antibody and anti-topoisomerase I antibody, the HLA (Human Leukocyte Antigen) types of proteins found in the SIL were investigated.…”

Prospective Risk of Rheumatologic Disease Associated with Occupational Exposure in a Cohort of Male Construction Workers

“…Also, we did not include in this analysis certain other even less common autoimmune diseases also associated with silica, for example, ANCA-associated vasculitis, Sjögren syndrome, "overlap" syndromes, and even pemphigus. 10,11,[25][26][27][28] There are other study limitations that should also be noted as well. The job exposure matrix subcategory of other inorganic dusts captures a heterogeneous group of exposures.…”

Antidesmoglein Autoantibodies in Silicosis Patients with No Bullous Diseases

“…In previous papers [5, 6, 7], we reported that pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid were associated with long-term silicosis. Silicosis is well known to be a causative factor in several autoimmune diseases and collagen diseases [8, 9, 10, 11, 12, 13]and pemphigus may also be one of these autoimmune diseases.…”

“…In previous reports [5, 6, 7], we described 2 cases of pemphigus and 1 case of bullous pemphigoid associated with silicosis. As it is well known, some silicosis patients suffer from concomitant autoimmune diseases or collagen diseases, such as systemic sclerosis [8, 9], systemic lupus erythematosus [10, 11], rheumatoid arthritis [12], dermatomyositis [13]or Sjögren syndrome [13].…”