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Periostin contributes to fibrosis by enhancing TGF-β signaling via Smad 7 inhibition, which may lead to ECM deposition and periostin generation.

Dermatomyositis (DM) is an autoimmune inflammatory disease characterized by skin eruptions and myositis. Anti-transcriptional intermediary factor 1-c antibody (anti-TIF1-c Ab) is one of the most frequently detected myositis-specific autoantibodies and adults positive for anti-TIF1-c have markedly higher rates of malignancy. Our aim was to determine the clinical associations of anti-TIF1-c levels in 31 Japanese adult DM patients positive for anti-TIF1-c. We determined associations between the anti-TIF1-c index and patient characteristics and disease severities. Sixteen patients with anti-TIF1-c Ab had concomitant malignancies. A mild positive correlation was found between the levels of serum creatine phosphokinase at the first visit and anti-TIF1-c levels. In contrast, there was no significant difference in the anti-TIF1-c Ab index between patients with and without malignancy. Dysphagia tended to be observed in patients with malignancy. On sequential analysis, anti-TIF1-c levels in patients without malignancy were lower or turned negative after treatment for DM. Ab titers tended to be sustained in patients with stage IV malignancies. Interestingly, a re-increase in the Ab titer was observed on recurrence of malignancy or increase in DM activity. Four patients were completely cured of their malignancies, and anti-TIF1-c levels in three patients turned negative with the loss of DM activity. These data suggest that higher anti-TIF1-c titers may not directly indicate the presence of malignancy. Nevertheless, longitudinal changes in the anti-TIF1-c index in individual patients may partially reflect activities of both DM and malignancy.

A number of cases of psoriasis vulgaris developing bullous skin lesions have been diagnosed as either bullous pemphigoid with antibodies to the 180-kDa bullous pemphigoid antigen (BP180) non-collagenous 16a (NC16a) domain or anti-laminin-γ1 (p200) pemphigoid. We report a case of subepidermal bullous disease with psoriasis vulgaris, showing antibodies to both BP180 C-terminal domain and laminin-γ1. A 64-year-old Japanese man with psoriasis vulgaris developed exudative erythemas and tense bullae on the whole body but he did not have mucosal involvement. The blistering lesion showed subepidermal blisters histopathologically. In indirect immunofluorescence of 1 mol/L NaCl-split skin, immunoglobulin (Ig)G antibodies reacted with both the epidermal and dermal side. Immunoblotting showed positive IgG with recombinant protein of BP180 C-terminal domain and 200-kDa laminin-γ1 in normal human dermal extract.

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