Background: Although it has been reported that papuloerythroderma of Ofuji (PEO) often occurs in association with internal malignancy, the true incidence of malignancy in patients with PEO is unknown. Objective and Methods: To ascertain the incidence of and relationship with internal malignancy in patients with PEO, 11 patients with PEO diagnosed at our dermatology clinic between September 2005 and June 2011 were retrospectively reviewed. Results: Internal malignancy was found in 6 (54.5%) of the 11 PEO patients, and 5 cases were idiopathic PEO. In the 6 cases with associated malignancy, PEO preceded the malignancies, and the diagnosis of malignancy was made just before or shortly after the diagnosis of PEO, but the malignant process and PEO did not always run a parallel course. Conclusions: Although the limitations of this study included a relatively small sample size, the present findings show a high incidence of internal malignancy in patients with PEO.
The clinical features of adult-type IgA vasculitis have not been well characterized. To analyze the characteristics of IgA vasculitis in adults, patients diagnosed with IgA vasculitis based on EULAR/PRINTO/PRES criteria (2012) in our institution between 2003 and 2012 were studied, comprising 85 adults (age ≥ 21 years) and 37 pediatric patients (≤ 20 years). Compared with pediatric cases, adult disease showed significantly higher serum C-reactive protein and IgA values, a lower percentage of cases was associated with infections (56.5% vs 89.2%, P < 0.001) but there was a greater range of infections affecting different tissues and organs, and there was occasional cases with malignancy (8.2%) including four cases of lung carcinoma and three with hematological disorders. The skin lesions in adults tended to be widely distributed on the abdomen and waist (15.3% vs 2.7%, P = 0.045). Adult cases were associated with greater renal involvement, as evidenced by proteinuria, hematuria and/or urinary casts, compared with the pediatric group (76.2% vs 48.6%, P = 0.003) and disease recalcitrance was also significantly higher (38.8% vs 18.9%, P = 0.031). Examination of the serum levels of immunoglobulins in adults showed that a sole increase in IgA was associated with renal and gastrointestinal manifestations, but this was not seen in cases with concurrent increases of IgA and IgG or IgA, IgG and IgM. Although the retrospective nature of the study is a limitation, it identified possible associations with the wide range of infections, more severe renal damage, and malignancy in adult IgA vasculitis.
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