Cutaneous and systemic plasmacytosis showing histopathologic features as mixed-type Castleman disease: a case report

Cutaneous and systemic plasmacytosis (CSP) is a rare disorder characterized by disseminated reddish brown plaques and polyclonal hypergammaglobulinemia. The lesions of CSP are histologically characterized by an infiltration of mature polyclonal plasma cells, which display similar pathological features to the plasma cell-type Castleman disease (CD). The relationship between CSP and CD is controversial. Herein, we described a 43-year-old man from China with disseminated reddish brown plaques and nodules on the cheek and temple. The serum level of immunoglobulin G and immunoglobulin A were higher than normal. In addition to mature plasma cell perivascular infiltrate in the dermis, the biopsy of the lesions showed small to medium-sized germinal follicles with hyalinized vessels and a concentrically arranged mantle zone. The patient had clinical features of CSP, but the biopsy revealed changes resembling mixed-type CD. To the best of our knowledge, this is the first case of CSP with the pathological features of mixed-type CD reported from 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.     

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.     

皮肤及系统性浆细胞增多症一例

患者男,46岁。6年余前背部出现散在红褐色斑块和结节,逐渐发展至腋窝及前胸部,质地变硬,冬季稍瘙痒。实验室检查发现高丙种球蛋白血症。电子计算机断层扫描（CT）发现双肺多发结节、斑片灶,纵隔、腋窝及腹股沟淋巴结肿大,脾脏增大。皮损组织病理检查：真皮中下层可见大量淋巴细胞、组织细胞、成熟浆细胞形成的肉芽肿性浸润,并可见淋巴滤泡样结构,未见细胞异形;免疫组化染色：CD38、CD138、CD79a、κ轻链、λ 轻链均阳性。结合临床及实验室检查,诊断为皮肤及系统性浆细胞增多症。     

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 plasmacytosis: A report of five cases with immunohistochemical evaluation for HHV-8 expression

Cutaneous plasmacytosis is a rare disorder that typically affects middle-aged to older individuals of Asian, particularly Japanese, descent. Clinically, it is characterized by multiple asymptomatic red-brown plaques and nodules on the trunk. Lymphadenopathy and hypergammaglobulinemia may be present. Histologically, the lesions show a moderately dense superficial and deep perivascular infiltrate composed predominantly of mature plasma cells without atypia or light chain restriction. We report our experience with five additional cases, including results of immunohistochemical studies for human herpes virus 8.     

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 plasmocytosis

Cutaneous 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.     

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.     

皮肤浆细胞增多症

报告1例皮肤浆细胞增多症.患者男,51岁.右下肢股外侧浸润性红斑4年余,口周和腹部皮损1年.皮损组织病理检查:真皮深层及部分皮下组织可见以成熟浆细胞为主的大量炎性细胞浸润,其间有少量淋巴细胞及多核巨细胞.免疫组化染色示:浸润的浆细胞CD79a、CD138阳性.诊断:皮肤浆细胞增多症.     

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.     

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.     

Hyperviscosity syndrome associated with systemic plasmacytosis

Systemic plasmacytosis is characterized by plasma cell proliferation in multiple organs including skin, and by polyclonal hypergammaglobulinaemia. Hyperviscosity‐related retinopathy has never been described with this condition, to our knowledge. We report a case of systemic plasmacytosis in a 49‐year‐old Japanese woman, who presented with fever, multiple erythematous plaques, hypergammaglobulinaemia, renal failure and bilateral retinal haemorrhage. Reduction of immunoglobulin with oral steroid reversed the retinopathy related to hyperviscosity syndrome. When marked hypergammaglobulinaemia is found in a patient with systemic plasmacytosis, funduscopic examination should be performed to reveal early asymptomatic retinal changes, because the retinopathy is treatable by control of the underlying disease.     

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.     

Systemic and cutaneous plasmacytosis with multiple skin lesions and polyclonal hypergammaglobulinaemia: significant serum interleukin-6 levels

We report two patients who developed benign plasmacytosis with multiple skin lesions. The cases were characterized by hyperplasia of mature plasma cells, and polyclonal hypergammaglobulinaemia. One patient had hyperplasia of mature plasma cells not only in the skin, but also extensively in lymph nodes and the retroperitoneal areas around the ureters. The other had plasma cell hyperplasia limited to the skin. Extensive investigations failed to reveal any clinical or laboratory evidence suggesting the presence of any underlying disease accompanying the hypergammaglobulinaemia and/or plasma cell proliferation, such as chronic infectious disease, collagen disease or other chronic inflammatory disorder. Clinically and histologically, the first patient showed features compatible with a diagnosis of systemic plasmacytosis and the second with a diagnosis of cutaneous plasmacytosis. Significant serum interleukin-6 (IL-6) levels were detected in both patients, suggesting that IL-6 may be involved in the pathogenesis of these conditions.     

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.     

Angioimmunoblastic T‐cell lymphoma with a clonal plasma cell proliferation that underwent immunoglobulin isotype switch in the skin,coinciding with cutaneous disease progression

Plasma cell proliferations in specific cutaneous lesions of angioimmunoblastic T‐cell lymphoma(AITL) are very uncommon. Here, we report a case of clonal plasma cell proliferation in skin with heavy‐chain‐immunoglobulin‐isotype‐switch after cutaneous disease progression. Histopathologically, initial plaque lesions were suggestive of marginal‐zone B‐cell‐lymphoma. Nevertheless, this 77‐year‐old lady was diagnosed with AITL after the progression of skin lesions from plaques to nodular tumors. A lymph node biopsy confirmed the diagnosis. Both cutaneous specimens showed a polymorphic cellular infiltrate with atypical T‐cell‐lymphocytes arranged in a pseudonodular pattern that expressed CD3, PD1 and BCL6, with patchy expression of CD30. Interestingly, a slight IgG‐Lambda plasma cell component was seen at the periphery of the infiltrate in the first specimen which increased in number in the later nodular lesion, showing not only Lambda light chain restriction and IgG but also IgG4. PCR studies for IgH and TCR genes showed an IgH clonal peak on both skin lesions but not on lymph node biopsy. On the contrary, the same clonal TCR peak was found in the three specimens. Neoplastic follicular helper T‐cells within cutaneous‐specific microenvironment could be responsible for the modulation of the immunoglobulin isotype class switch change. Further studies are needed to support this hypothesis.     

Cutaneous plasmacytosis associated with lung and anal carcinomas

Cutaneous plasmacytosis is a rare disorder characterized by a benign proliferation of mature plasma cells that appears as multiple dark-brown to purplish skin lesions, often associated with polyclonal hypergammaglobulinaemia. We present the case of a 55-year-old Caucasian man who suffered from a cutaneous plasmacytosis associated with two different carcinomas. Cutaneous plasmacytosis seems to be a reactive process because most cases reported are not associated with any apparent underlying disease. Nevertheless, because few reported cases were associated with malignancies, screening of additional neoplasms would be justified.     

Successful treatment with topical PUVA of nodular cutaneous plasmacytosis associated with alopecia of the scalp

We obtained a successful response to PUVA therapy in a 51-year-old Japanese man who had multiple nodules of the trunk, extremities and scalp along with polyclonal hypergammaglobulinaemia; the scalp lesions were associated with extensive alopecia. Examination of skin biopsies showed a dense infiltrate of mature plasma cells in the dermis and hair follicles; no systemic disease or functional involvement of other organs was detected. The clinical and histological findings were compatible with cutaneous plasmacytosis and treatment with topical PUVA gradually reduced the size and number of the lesions.