Plasma cytosis cutaneous and systemicD47.9
Synonym(s)
HistoryThis section has been translated automatically.
Yashiro, 1976
DefinitionThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
> 50 years, very occasionally also with children.
LocalizationThis section has been translated automatically.
face, neck, neck, trunk
Clinical featuresThis section has been translated automatically.
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.
LaboratoryThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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.
Differential diagnosisThis section has been translated automatically.
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.
TherapyThis section has been translated automatically.
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.
Note(s)This section has been translated automatically.
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".
LiteratureThis section has been translated automatically.
- Brezinski EA et al (2014) N. Cutaneous plasmacytosis with perineural involvement. Case Rep Dermatol Med PubMed Central PMCID: PMC4006542.
- Chen H et al (2012) Cutaneous and systemic plasmacytosis showing histopathologic features as mixed-type Castleman disease: a case report. At J Dermatopathol 34:553-556
- Kato K et al (2013) IgG4-positive cells in skin lesions of cutaneous and systemic plasmacytosis. Eur J Dermatol 23:255-256
- Oka M et al (2013) Cutaneous plasmacytosis in an 88-year-old woman successfully treated with low-dose oral corticosteroid. Int J Dermatol. 52: 1412-1414
- Shiba Y et al (2014) Isolated benign primary cutaneous plasmacytosis. Int J Dermatol 53: 397-398
- Song HS et al (2014) Congenital form of isolated benign primary cutaneous plasmacytosis in a child. Ann Dermatol 26:121-122
- Takeuchi M et al (20123) Cutaneous multicentric Castleman's disease mimicking IgG4-related disease. Pathol Res Pract 208:746-749
- Wagner G et al (2013) Cutaneous and systemic plasmocytosis. J Dtsch Dermatol Ges 11:1161-1167
- Xia JX et alo. (2013) A case of cutaneous plasmacytosis. Exp Ther Med 5:1211-1213
- Yamaguchi H et al (2013) Cutaneous plasmacytosis as a skin manifestation of IgG4-related disease. Eur J Dermatol 23:560-562