XXXX : a clinicopathologic study of 12 cases in Taiwan revealing heterogeneous underlying causes.

@entity7255 @entity1 @entity1576 @entity3329
BACKGROUND: @entity7255 is clinically characterized by multiple pigmented papules and plaques that occur mainly on the trunk and many plasma cells in the lesional skin. Most of the cases reported have occurred in Japan. Whether @entity7255 is a unique reactive disease or a neoplastic condition has not been established. METHODS: We collected 12 cases fulfilling the criteria for a diagnosis of @entity7255 at our institution from 2001 to 2013. We analyzed clinicopathologic features and performed immunohistochemical and immunoglobulin gene rearrangement studies. RESULTS: All 12 @entity1 presented with characteristic cutaneous lesions mainly on the trunk and proximal extremities. Many plasma cells were observed in the lesional skin, and the basal layer was hyperpigmented. Four @entity1 had numerous plasma cells in lymph nodes, and one @entity1 also had increased plasma cells in bone marrow. One @entity1 fulfilled the criteria for immunoglobulin G4 (IgG4)-related @entity3329 . Elevated serum IgG and IgG4 concentrations were found in seven patients. Monoclonal immunoglobulin gene rearrangements were detected in four @entity1 . The median duration of follow-up was four years. One @entity1 was lost from follow-up. Partial remission of the cutaneous lesions was observed in five @entity1 , and progressive disease was noted in five @entity1 . One @entity1 died of @entity1576 . CONCLUSIONS: Our study suggests that the so-called " @entity7255 " has heterogeneous underlying causes with or without systemic involvement and may be associated with clonal immunoglobulin gene rearrangements and IgG4-related @entity3329 . No effective treatment is available for this condition.
