Systemic Plasmacytosis - A Case Report with a Review of the Literature -

Cutaneous and systemic plasmacytosis is a rare disease arising primarily in the Asian population. After its first recognition as a distinctive cutaneous lesion in 1976, since then approximately 60 cases have been reported worldwide, the vast majority of them being from Japan. Previously termed as “cutaneous plasmacytosis,’’ the condition has been subsequently named as ‘‘cutaneous and systemic plasmacytosis’’ to reflect the high frequency of lymphoplasmacytic infiltration of extracutaneous sites. The term cutaneous plasmacytosis is mainly used for a disorder, which accompanies dark brown skin eruption but lacks systemic involvement. Cutaneous and systemic plasmacytosis is characterized by a cutaneous polyclonal plasma cell infiltration accompanied by lymphadenopathy, and polyclonal hypergammaglobulinemia. The most common manifestation of extracutaneous involvement is superficial lymphadenopathy. Involvement of lung is presented as lymphoid interstitial pneumonia or as multiple tiny nodules. Involvement of the liver, spleen, and kidney is of less common occurrence. In our present study, we describe a case of Korean patient, who was initially diagnosed to be suffering from cutaneous plasmacytosis, which later involved systemic involvement when the patient underwent lymph node biopsies on the left axilla and supraclavicular area. To the best of our knowledge, this is the third case to be reported in Korean literature.