Systemic Plasmacytosis: A Case which Improved with Melphalan

Lee, Jun Young; Choi, Seok Keun; Park, Jong Won; Cho, Baik Kee

doi:10.1111/j.1346-8138.1995.tb03372.x

Cited by 16 publications

(10 citation statements)

References 18 publications

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“…In the review of Shimizu et al [1], the typical course of cutaneous plasmacytosis was chronic with a mean duration of 7.7 years, and this disease did not respond to any treatment. One patient with systemic plasmacytosis improved after treatment with melphalan [9]. Topical PUVA therapy reduced the size of the lesions in a patient who had plasmacytosis of the trunk, extremities and scalp along with alopecia [10].…”

Section: Discussionmentioning

confidence: 99%

Targeting T Cells to Hit B Cells: Successful Treatment of Cutaneous Plasmacytosis with Topical Pimecrolimus

Hafner

Hohenleutner²,

Babilas³

et al. 2006

Dermatology

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Cutaneous plasmacytosis is a rare disease predominantly found in Japanese patients. We describe the case of a 75-year-old white female with cutaneous plasmacytosis of the face and involvement of the bone marrow. In contrast to other cases of cutaneous plasmacytosis, the patient revealed hypogammaglobulinemia and elevated levels of free light chains in the urine. Treatment with topical pimecrolimus 1%, which primarily targets T cells, led to almost complete clinical and histological remission of the skin lesions. Our report indicates that the therapeutically induced disappearance of the plasma cells was due to indirect T-cell-mediated effects.

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Section: Discussionmentioning

confidence: 99%

Targeting T Cells to Hit B Cells: Successful Treatment of Cutaneous Plasmacytosis with Topical Pimecrolimus

Hafner

Hohenleutner²,

Babilas³

et al. 2006

Dermatology

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“…A few cases have followed an aggressive clinical course as lymphoma [15], lymphoid interstitial pneumonia [10], or renal failure [8,9]. Treatment has included topical and systemic corticosteroids [16], cyclophosphamide [4], topical tacrolimus [17], psoralen-UV-a, and other chemotherapy [6,9,[18][19][20].…”

mentioning

confidence: 99%

High ratio of IgG4-positive plasma cell infiltration in cutaneous plasmacytosis—is this a cutaneous manifestation of IgG4-related disease?

Miyagawa‐Hayashino¹,

Matsumura²,

Kawakami³

et al. 2009

Human Pathology

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Cutaneous plasmacytosis is a rare condition affecting middle-aged individuals, characterized by multiple red-brown papules and plaques over the trunk. It has been reported mainly in Japan. The condition is accompanied by polyclonal hypergammaglobulinemia and superficial lymphadenopathy. Lung or retroperitoneal involvement occurs rarely. In the present study, 3 consecutive cases of cutaneous plasmacytosis were observed histologically to have abundant infiltration of IgG4-bearing plasma cells. All 3 were associated with superficial lymphadenopathy, one with interstitial lung involvement showing ground-glass opacity on computed tomography and the others with bone marrow plasmacytosis, showing histologic evidence of more IgG4-positive plasma cells. All 3 had polyclonal hypergammaglobulinemia, one had high serum concentration of IgG4, and all had elevated serum IL-6. The ratios of IgG4+ to IgG+ plasma cells were assessed using skin biopsy specimens with pemphigus (n = 7), discoid lupus erythematosus (n = 5), and morphea (n = 2) (mean ratios, 19%, 0%, and 0%, respectively); we noted the proportion of IgG4-positive plasma cells in cutaneous plasmacytosis (mean, 48%). IgG4-related sclerosing disease is a newly recognized systemic disorder characterized by lymphoplasmacytic infiltration and fibrosis and by a high serum IgG4 level and increased IgG4-positive plasma cells in the tissues. Skin manifestations of this disorder have not been described. Although cutaneous plasmacytosis could be a chronic allergic hypersensitivity reaction, our findings raise the possibility of a relationship in pathogenesis between cutaneous plasmacytosis and IgG4-related sclerosing disease.

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“…Eine therapeutische Wirkung ließ sich nicht feststellen [4,38]. Als wirksam erwies sich Melphalan und die Kombination von Prednisolon, Immunglobulinen und Cyclophosphamid [22,39].…”

Section: Therapieunclassified

Kutane und systemische Plasmozytose

Wagner

Rosé²,

Klapper

et al. 2013

J Deutsche Derma Gesell

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SummaryCutaneous and systemic plasmacytosis is a rare disorder observed mainly in Japanese that features an infiltration of mature plasma cells in various organ systems. In addition to the skin, lymph nodes and bone marrow are regularly affected. Laboratory tests show a polyclonal hypergammaglobulinemia. The cutaneous morphology is characterized by red to dark brown macules, papules and plaques a few centimeters in diameter, usually distributed symmetrically on the face, neck and back. Etiology and pathogenesis are not known. It is speculated that a reactive dysfunction of plasma cells may be triggered by various stimuli, such as interleukin 6. Treatment of cutaneous and systemic plasmacytosis is difficult. A standardized treatment concept does not yet exist. Topical corticosteroids and calcineurin inhibitors are mainly used.

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Systemic Plasmacytosis: A Case which Improved with Melphalan

Cited by 16 publications

References 18 publications

Targeting T Cells to Hit B Cells: Successful Treatment of Cutaneous Plasmacytosis with Topical Pimecrolimus

Targeting T Cells to Hit B Cells: Successful Treatment of Cutaneous Plasmacytosis with Topical Pimecrolimus

High ratio of IgG4-positive plasma cell infiltration in cutaneous plasmacytosis—is this a cutaneous manifestation of IgG4-related disease?

Kutane und systemische Plasmozytose

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