Systemic Plasmacytosis: A Case which Improved with Melphalan

Plasmacytosis, a distinctive proliferative disorder of plasma cells, is characterized by peculiar multiple skin eruptions, lymphadenopathy and polyclonal hypergammaglobulinemia. To date there has been no report of such cases showing remarkable responses to therapeutic agents. We herein report a case of plasmacytosis which developed in a 52-year-old Korean man and showed remarkable improvement with melphalan.     

A CASE OF PLASMACYTOSIS WITH MULTIPLE PECULIAR ERUPTIONS

A 32-year-old man had been in good health until he noticed painful swelling of the lymph nodes in the left occipital region in spring of 1975. Half a year later, multiple, infiltrating erythematous or nodular lesions appeared on the anterior chest and then spread over almost his entire body except for the lower extremities. These skin lesions were reddish brown to purplish brown in color, and irregular in shape and size. All of his superficial lymph nodes were palpable, and pain was felt on pressure. No other systemic symptoms were noted. Laboratory findings showed polyclonal gammopathy; total protein, 9.6 g/dl; IgG, 4,900 mg/dl; IgA, 590 mg/dl and an increased erythrocyte sedimentation rate of 92 mm/hr. A skin biopsy revealed prominent perivascular proliferation of plasma cells and follicular hyperplasia in the dermis. Lymph node biopsy also revealed proliferation of plasma cells in both the paracortical area and medulla. No neoplastic growth was recognized. These pathological findings and polyclonal hypergammaglobulinemia were consistent with reactive plasmacytosis. The patient is now under observation, and the skin lesions and systemic lymphadenopathy have not been changed. This peculiar B-cell proliferative disorder was discussed in relation to other diseases.     

Cutaneous and Systemic Plasmacytosis Associated with Renal Amyloidosis

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder of unknown etiology characterized by cutaneous polyclonal plasma cell infiltrates associated with various extracutaneous involvement and polyclonal hypergammaglobulinemia. Here, we report on a 54-year-old male patient with chronic renal insufficiency who presented with disseminated reddish-brown macules and plaques on the face and trunk. In our evaluation, he was found to have lymphadenopathy, polyclonal hypergammaglobulinemia; benign plasma cell infiltration involving the skin, bone marrow, and retroperitoneal area; and renal amyloidosis. To the best of our knowledge, this is the first reported case of CSP associated with renal amyloidosis.     

Extensive hyperpigmented plaques in a chinese singaporean woman: a case of cutaneous plasmacytosis

Cutaneous plasmacytosis is a rare disease entity presenting with multiple extensive red-brown plaques, histopathology showing marked hyperplasia of mature polyclonal plasma cells, and polyclonal hypergammaglobulinemia on serum protein electrophoresis, in the absence of an underlying secondary cause. We report in this article the first case of cutaneous plasmacytosis from Singapore. A 33-year-old Chinese woman presented with mildly pruritic reddish brown papules and plaques over her trunk and arms for 2 years. Physical examination, laboratory investigations, and radiographic examination were negative for systemic involvement and lymphadenopathy. Serum immunoelectrophoresis showed polyclonal hypergammaglobulinemia with immunoglobulin G and immunoglobulin A. Two sets of skin biopsies performed 2 years apart essentially showed similar histopathological findings of a superficial and deep perivascular infiltrate with numerous mature plasma cells and small typical lymphocytes. There were lymphoid follicles with well-formed germinal centers and mantle zones, surrounded by mature lymphocytes. No light chain restriction was present on immunohistochemistry, and polymerase chain reaction for heavy chain gene rearrangement was negative for monoclonality. Despite potent topical corticosteroids and 8 months of phototherapy with narrow band ultraviolet light, there was no improvement. Intralesional triamcinolone injections to a few lesions afforded temporary relief of itch and flattening of lesions.     

Multicentric Castleman disease with cutaneous manifestations: report of 2 cases and comparison with systemic plasmacytosis

We report 2 patients with multicentric Castleman disease. Both presented with multiple, indurated, hyperpigmented plaques, generalized lymphadenopathy and polyclonal hypergammaglobulinemia. Biopsy specimens showed infiltration of mature plasma cells and lymphocytes in the dermis and lymph nodes. Skin specimens were negative for human herpesvirus 8, latent nuclear antigen 1 and Epstein-Barr virus by in situ hybridization. PCR disclosed clonal T-cell receptor gene rearrangement in the bone marrow cells of 1 patient. We discuss the possible relationship between multicentric Castleman disease and systemic plasmacytosis as well as plasma cell proliferation.     

免疫母细胞性淋巴结病样T细胞淋巴瘤伴皮肤损害1例

免疫母细胞性淋巴结病样Ｔ细胞淋巴瘤（ＩＢＬ－ＴＣ）是特殊类型的周围Ｔ细胞淋巴瘤。临床特征为发热;淋巴结、肝脾肿大;皮疹及高γ球蛋白血症。皮疹呈多种形态,随其发展主要为二型：丘疹结节型和红皮病型。淋巴结病理示淋巴结结构破坏,有异形淋巴样细胞、免疫母细胞和浆细胞样的所谓淡染细胞（ｐａｌｅｃｅｌｌｓ）浸润。免疫学示Ｔ细胞标记。     

Cutaneous plasmacytosis limited to the extremities in a white patient: an unusual clinical picture

Cutaneous plasmacytosis is an uncommon disease characterized by a cutaneous polyclonal plasma cell infiltrate usually associated with polyclonal hypergammaglobulinemia. It has predominantly been found in Japanese patients and it is rare in white patients. Clinically, this condition manifests as multiple red to dark brown skin lesions that mainly are located on the trunk. We report the case of a 66-year-old white woman who presented with reddish brown to violaceous macules and plaques restricted to the extremities. The histopathologic findings, laboratory data, and systemic studies led us to the diagnosis of cutaneous plasmacytosis.     

Cutaneous and systemic plasmacytosis: a Chinese case

Cutaneous and systemic plasmacytosis (CSP) is an exceedingly rare condition arising primarily in patients of Japanese descent. Herein, we describe a patient of mainland Chinese origin suffering CSP. A 49-year-old Chinese male had asymptomatic brownish-red plaques and papules of the face and trunk for 6 years. Physical examination revealed innumerable symmetric red-brownish macules on face and trunk with fewer red-brownish papules scattered among the macules. Chemical analysis revealed hypergammaglobulinemia. Computerized tomography scan discovered some lymphadenopathy in the axillary, paratracheal and pulmonary regions. Histological examination showed focal perivascular and periadnexal infiltrate of mainly plasma cells in the superficial and deep dermis. Immunohistochemical study showed that a great number of the infiltrating cells were CD20-positive. The infiltrated polyclonal plasma cells expressed both kappa and lambda light chains. Topical therapy with tacrolimus 0.1% ointment for 2 months reduced the thickness and pigmentation of the facial skin lesions. The lesions resumed the original appearance 3 weeks after discontinuing the therapy. To the best of our knowledge, this is the first case of CSP from mainland China.     

Cutaneous T‐cell Lymphoma of 12 Years Duration Initially Presenting in a Two Year Old

A 67‐year‐old Chinese man presented with a longstanding history of asymptomatic, brown‐red macules that diffusely involved his trunk and the extremities and was associated with generalized lymphadenopathy. His serum protein electrophoresis revealed a polyclonal hypergammaglobulinemia. No monoclonal Bence Jones protein was detected in the urine. Flow cytometry of his peripheral blood revealed a normal polyclonal population of B and T cells. Histopathologic examination demonstrated a dense nodular infiltrate of lymphocytes and numerous mature plasma cells in the reticular dermis. Immunoperoxidase studies for kappa and lambda light chains failed to demonstrate clonality. In addition, immunoglobulin gene rearrangement studies failed to reveal a monoclonal band. A bone marrow biopsy showed no abnormality. Cutaneous and systemic plasmacytosis is a rare lymphoproliferative disorder that is an important consideration in the differential diagnosis of cutaneous infiltrates in which plasma cells predominate.     

皮肤窦性组织细胞增生症

报告1例皮肤窦性组织细胞增生症（CSH）。患者女,48岁,仅表现为左面颊部有一斑块。皮损的组织病理特征为真皮及皮下有以组织细胞和淋巴细胞为主的浸润,可见吞噬现象。免疫组化染色组织细胞S-100蛋白和CD68阳性,CD1a标记阴性。阿维A试验性治疗有效。     

Cutaneous Rosai-Dorfman disease

Presented is a case of a 31-year-old woman with cutaneous Rosai-Dorfman disease, which is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. It primarily involves lymph nodes but can affect extranodal sites such as the skin, where it typically appears as indistinct erythematous papules and nodules. On histopathologic examination, emperipolesis is a consistent finding. The etiology is unknown, and treatment is based on clinical manifestations.     

CD56-Positive cutaneous lymphoma with multicentric Castleman's disease-like systemic manifestations.

We report a 55-year-old Japanese male with CD56+ cutaneous lymphoma. The patient had multiple cervical lymphadenopathy, a red nodule on his neck, and parotid gland nodularity. Histologic features of the biopsied cervical lymph node showed follicular hyperplasia with numerous plasma cells. A biopsied skin specimen of the nodule on his neck demonstrated dense infiltration of atypical large lymphocytes into the dermis. Immunohistochemical study of this specimen revealed CD3+, CD4+, and CD56+ expression in the majority of neoplastic cells. Polymerase chain reaction assays for the detection of Epstein-Barr virus sequences were positive for lymph node and skin DNA. Laboratory examinations showed polyclonal gammopathy, pancytopenia, and high serum interleukin-6 levels. These clinical and histological findings resembled those of multicentric Castleman's disease.     

Primary Cutaneous Plasmacytosis: Masquerading as Hidradenitis Suppurativa

Isolated cutaneous plasmacytosis (CP) is a rare entity with few cases reported in world literature. CP masquerading as hidradenitis suppurativa like presentation is a unique case with some features differentiating it clinically from it which were further confirmed by histopathology and immunostaining. Our case showed hyperplasia of mature plasma cells and polyclonal hypergammaglobulinemia, immunostaining for CD138 positivity and kappa: lambda ratio more than 3:1. Extensive clinical and laboratory investigations failed to reveal any underlying pathology, presence of any underlying disease accompanying the hypergammaglobulinemia and/or plasma cell proliferation.     

Clinicopathologic Study of Cutaneous Plasmacytoma

Eight patients with skin tumor lesions composed of dense, predominantly plasma cell infiltrates were studied. Primary cutaneous plasmacytoma can be reactive (polyclonal) or neoplastic (monoclonal). In four of the patients skin lesions were associated with multiple myeloma. Specific skin lesions usually consisted of reddish or purple nodules located on the trunk. In one case the cutaneous lesions developed at the site of previous herpes zoster. Histologically, the cutaneous plasmacytic infiltrate was mainly diffuse and monomorphous. Most of the plasma cells were mature, but in some cases immature immunoblasts and mitoses were observed. Serum immunoelectrophoresis findings correlated with the monoclonality or polyclonality of the plasmacytoma. Presence or absence of systemic involvement cannot be predicted from the appearance of clinical lesions or from maturity of plasma cell infiltration in the skin.     

Successful treatment of Rosai–Dorfman disease with low-dose oral corticosteroid

We present herein a Japanese case of Rosai–Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. A 69-year-old Japanese man presented with a 1-year history of enlarged submandibular lymph nodes and subsequent nasal and pharyngeal bleeding. RDD was diagnosed based on biopsy results from a lymph node in the left parotid region. The patient had also noted several skin eruptions that repeatedly appeared and disappeared on the face and arms. Biopsies were taken from skin eruptions on the face and cuboidal fossa. Both biopsy specimens showed dense, well-demarcated infiltration of histiocytes, lymphocytes and multinucleated giant cells from just under the epidermis to the subcutaneous tissue. These histiocytes were positive for CD68 and S-100, but negative for CD1a, and some displayed emperipolesis. Given the histopathological findings and the fact that the patient was suffering from RDD, skin lesions were diagnosed as cutaneous manifestations of RDD. Cutaneous lesions gradually began to persist concomitant with enlargement of extranodal lymphadenopathy in the nasopharyngeal area. Increasing respiratory obstruction prompted a trial with oral prednisolone commencing at 0.4 mg/kg per day. Both the lymphadenopathy and skin lesions responded quickly. Within 3 months, all his skin lesions disappeared completely with almost complete resolution of lymphadenopathy. Twelve months after the beginning of oral prednisolone therapy, slight recurrence of mucosal and cutaneous lesions appeared, but disappeared quickly with an increase in prednisolone to 0.3 mg/kg per day. Low-dose prednisolone appeared very effective in the case of RDD.     

Cutaneous and systemic plasmacytosis in a patient of Asian descent living in the United States

Cutaneous and systemic plasmacytosis is a rare disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. Previous reports have been largely restricted to the Japanese literature. We present the first documented case of cutaneous and systemic plasmacytosis in a patient residing in the United States. This 49-year-old man, who had immigrated from Korea 19 years earlier, developed innumerable persistent pink-to-brown macular lesions over his trunk and face. Initial and repeat skin biopsy specimens revealed dense perivascular and periadnexal infiltrates of mature plasma cells, and polyclonal plasmacytosis noted on two different biopsy specimens of mildly enlarged lymph nodes. Multiple tiny pulmonary nodules were found to be of the same histologic appearance. No evidence of clonal immunoglobulin gene rearrangements or human herpesvirus type 8 infection was noted in these biopsy specimens. Treatment with antibiotics, systemic chemotherapy, and anti-CD20 antibody therapy failed to eradicate these lesions, which have persisted for 6 years. This case demonstrates that cutaneous and systemic plasmacytosis can arise in a patient of Asian ancestry, even many years after emigration to the United States.     

Cutaneous involvement by angioimmunoblastic T-cell lymphoma with remarkable heterogeneous Epstein-Barr virus expression

INTRODUCTION: Initially described as an abnormal immune reaction, most cases of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD)-like T-cell infiltrates are now regarded as a peripheral T-cell lymphoma (AILD T-NHL). AILD T-NHL is characterized clinically with constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin rash, and polyclonal hypergammaglobulinemia. Epstein-Barr virus (EBV) is frequently detected in involved lymph nodes, but the presence of EBV in cutaneous infiltrates of AILD T-NHL has rarely been examined. We present a patient with AILD T-NHL with cutaneous involvement that shows marked heterogeneity of EBV expression in the lymph node and skin biopsies, and review the histological findings of AILD T-NHL in the skin. METHODS: Two skin biopsies of a diffuse maculopapular rash and a lymph node were examined and immunophenotyped. In situ hybridization for detection of EBV in the lymph node and skin biopsies was utilized. In order to attempt to delineate which lymphocytes were EBV positive, skin biopsies were dual labeled with CD3, CD45RO, CD20 and EBV. The skin biopsies and lymph node were submitted for gene rearrangement studies by polymerase chain reaction (PCR). Capillary electrophoresis of fluorescently labeled PCR products was utilized for PCR product quantitation. RESULTS: The histological features of the lymph node were diagnostic of AILD T-NHL and a T-cell clone was identified by PCR. The skin biopsies showed an atypical superficial and deep perivascular polymorphous infiltrate consistent with cutaneous involvement by AILD T-NHL. Both skin biopsies showed the same clonal T-cell receptor gene rearrangement as the lymph node. In situ hybridization of the lymph node and one skin biopsy showed a few scattered EBV-positive lymphocytes (<1% of the infiltrate). A second skin biopsy revealed 40-50% of the lymphocytes as EBV positive. Dual staining for CD20 and EBV identified a minority of EBV-infected lymphocytes as B-cells, but most of the EBV-positive cells lacked staining for CD3 and CD45RO. CONCLUSIONS: In our patient, the same T-cell receptor gene rearrangement was found by PCR in all three biopsy sites. Most cases of AILD T-NHL contain only a few EBV-positive cells, but in our patient the extent of EBV expression ranged from <1% to 40-50% of the AILD T-NHL cutaneous infiltrate. To our knowledge, this case is the most extensive and heterogeneous expression of EBV in cutaneous AILD T-NHL to date.     

Cutaneous involvement in angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin's nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia, and cutaneous involvement. We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression.     

Primary cutaneous plasmacytosis in a child. Is this a new entity?

Plasma cell proliferations represent a heterogeneous spectrum of disorders. A 7-year-old Caucasian female had suffered an asymptomatic eruption on the trunk for 4 years. Physical examination revealed a plaque with scattered red-brown papules and nodules. Chemical analysis revealed normal proteinaemia. Histological examination of biopsy specimens showed dense perivascular and periadnexal infiltrate, consisting largely of plasma cells, in the superficial and deep dermis. Immunohistochemical study showed that many cells of the infiltrate were CD20 positive. The plasma cells expressed kappa and lambda light chains. The girl's status (age; absence of hypergammaglobulinaemia, lymphadenopathy and hepatosplenomegaly; presence of an infiltrate of mature polyclonal plasma cells restricted only to the skin) differed from those generally seen in diseases with plasma cell proliferation reported in the literature. This case seems unlike any other described up to the present time.     

Epstein-Barr (EB) virus infection in homosexual men in London

Twenty five homosexual men from London, 14 of whom had persistent lymphadenopathy and 11 of whom did not, were tested for immunity to Epstein-Barr (EB) virus. All yielded positive results to serological tests for the viral capsid antibody, and 11 had antibodies to the early antigen. Thirteen out of 17 were excreting virus into the saliva; culture of peripheral blood mononuclear cells from two of these patients showed no detectable regression induced by T cells that was specific to EB virus. No differences were found between the patients with and without lymphadenopathy. Peripheral blood B cells from six patients with hypergammaglobulinaemia were double stained for cytoplasmic immunoglobulin and EB viral nuclear antigen, and in all cases the activated B cells producing immunoglobulin did not contain EB nuclear antigen. Similarly, lymph node biopsy specimens from five patients showed no cells with EB nuclear antigen. These results indicate that although homosexual men have a high incidence of reactivated infection with EB virus, this viral infection is not the cause of the polyclonal activation of B cells seen in peripheral blood and is not implicated in the aetiology of the lymphadenopathy found in these men.