Plasma cytosis cutaneous and systemic D47.9

Yashiro, 1976

Rare polyclonal lymphoproliferative disease of unknown aetiology with characteristic reddish-brown nodules, plaques and nodules on the face, neck and nape of the neck and also on the trunk. Preferred occurrence in Japan (few cases from China and other countries are described). The disease can occur with or without systemic symptoms. Increases in serum immunoglobulins IgG and IgA are not uncommon.

> 50 years, very occasionally also with children.

face, neck, neck, trunk

Multiple, 0.5 - 3.0 cm large, red or brown, less symptomatic, papules, plaques and/or nodules consisting of mature oligoclonal plasma cell infiltrates. At the same time a "superficial" lynphadenopathy is found in about 50% of the patients.

The usual blood analyses are generally normal. BSG accelerations of > 50 the first hour are frequently observed. Electrophoresis: hypergammaglobulinemia, with increases in immunoglobulins IgG and IgA. No monoclonality.

The skin histological picture shows a nodular superficial, intermediate and/or also deep nodular dermatitis with evidence of mainly plasma-cell shaped infiltrates with individual, small, well definable germ centres. Often "onion ring" type proliferates are found in the mantle zone.

The extent to which "cutaneous and systemic plasmocytosis" is not identical with the plasma cell type of Castleman's lymphoma must be conclusively clarified, since there is a large overlap between the two clinical pictures.

Associations or overlaps with POEMS syndrome should be discussed.

Prednisolone 30-50 mg/day p.o.; possibly in combination with thalidomide 150 mg/day p.o. over a period of several months Local therapy can be carried out with Tacrolimus 0,1 % 2x/day.

Cutaneous and systemic plasmocytosis (CSP) was first described by Yashiro in 1976 as a "kind of plasmacytosis". Later (1980), Kitamura defined the clinical picture more precisely and extended it by the term " contaneous -and systemic- plasmocytosis".

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