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.     

Treatment of cutaneous plasmacytosis with mask‐bath PUVA: A therapeutic option

Cutaneous plasmacytosis (CP) is an uncommon chronic disease of unknown aetiology, reported mainly in middle‐aged patients of Asian descent. It is diagnosed by a constellation of physical, laboratory, radiological and histopathological findings. We report a patient with CP who demonstrated a favorable and promising response to mask‐bath PUVA.     

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.     

Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease

Cutaneous and systemic plasmacytosis is a rare condition primarily affecting middle‐aged individuals of Japanese descent and has principally been reported in cases originating from Asia. Its relationship, if any, to Castleman disease, is controversial. The authors report a case of cutaneous and systemic plasmacytosis involving a 36‐year‐old man of Chinese ancestry born in North America. The biopsy of an involved lymph node revealed changes resembling Castleman disease. Significantly, this case to our knowledge represents the first case of cutaneous and systemic plasmacytosis presenting in a patient of Chinese ancestry born on the North American mainland. Shadel BN, Frater JL, Gapp JDG, Hurley MY. Cutaneous and systemic plasmacytosis in an Asian male born in the North American continent: A controversial entity potentially related to multicentric Castleman disease.     

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: 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.     

Targeting T cells to hit B cells: successful treatment of cutaneous plasmacytosis with topical pimecrolimus

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.     

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 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 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.     

Case of isolated benign primary cutaneous plasmacytosis in a child

A collection of plasma cells in the skin can represent a broad spectrum of disease entities. Secondary syphilis, primary cutaneous plasmacytoma, primary cutaneous plasmacytosis, cutaneous lymphoid hyperplasia and nodular amyloidosis are considered possible differential diagnoses. We present a case of a 7-year-old girl with an erythematous scaly plaque on her right buttock that had been present for approximately 5 years. Prior to her visit to our department she had been treated at a local dermatology clinic with topical methylprednisolone acetate and topical calcitriol without significant improvement. Histopathological examination revealed psoriasiform hyperplasia, hyperkeratosis, parakeratosis and a band-like or dense perivascular infiltration of plasma cells with a few lymphocytes and histiocytes. Other laboratory tests were within the reference ranges. At our department, the patient was given oral prednisolone along with an intralesional injection of triamcinolone and application of topical methylprednisolone acetate and tacrolimus hydrate to the affected area. The lesion improved significantly but recurred 3 months later. We present a rare case of isolated benign primary cutaneous plasmacytosis in a female pre-adolescent child.     

Cutaneous plasmacytosis and multinucleate cell angiohistiocytoma‐like lesion in a patient with hepatitis B: A fortuitous triad?

A 28‐year‐old woman of Chinese descent, with congenital chronic hepatitis B presented with a 7‐year history of erythematous‐brown papules and plaques on her groins, axillae, and forehead. A first skin biopsy showed findings consistent with two concomitant, yet highly uncommon cutaneous diseases. The presence of lymphoid nodules with germinal centers and clustered polyclonal plasma cells was consistent with cutaneous plasmocytosis. Second, a diffuse proliferation of non‐atypical small vessels (CD31+, CD34+, and HHV8?) in a hypercellular stroma peppered with angulated giant cells (CD163+, CD68?) was suggestive of multinucleate cell angiohistiocytoma (MCAH). Interestingly, the second biopsy of a different plaque on the forehead showed only plasmacytosis and the clinical appearance of both plaques and papules alluded to the distinct presence of both concurrent entities. We speculate the immune modulating effects of chronic hepatitis B may have led to a polyclonal plasmacytic proliferation within the dermis. Furthermore, MCAH has been reported in conjunction with other inflammatory skin diseases such as hidradenitis suppurativa and as such we propose that the MCAH lesion in our case may have arisen as a secondary, reactive process to the cutaneous plasmacytosis.     

Two cases of chronic oral ulcers effectively treated with systemic corticosteroid therapy: Circumorificial plasmacytosis and traumatic ulcerative granuloma with stromal eosinophilia

Chronic oral ulcers are induced by various causative factors. Biopsy from an active site around ulceration is critical for both the definitive diagnosis and proper treatment. We report two cases of chronic oral ulcers, circumorificial plasmacytosis (CP) and traumatic ulcerative granuloma with stromal eosinophilia (TUGSE). A 65‐year‐old man presented with a mucosal ulcer on the right half of the lower lip. The dense plasmacytic inflammatory infiltration was histologically consistent with CP. A 32‐year‐old woman presented with a mucosal ulcer on the right mouth commissure. The dense mixed inflammatory cell infiltrates composed of eosinophils, lymphocytes and histiocytes extending from the submucosal tissue to underlying striated muscle fibers were histologically consistent with TUGSE. p.o. administration of corticosteroid was effective in both cases. A broad differential diagnosis is required for chronic oral ulcers. If the oral ulcer did not respond to the first therapy, clinical re‐evaluation and biopsy is essential.     

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.     

Cutaneous plasmacytosis: an unusual presentation sharing features with POEMS syndrome?

Cutaneous plasmacytosis is a recently described skin disorder consisting of brown to red papules and nodules containing polyclonal plasmacytes. In this particular case, leg ulcers developed but also a diffuse patchy hyperpigmentation coexisting with a primary hypothyroidisim. The last two signs have only been described to date in POEMS syndrome, which is linked to monoclonal plasmacytic proliferation, and might suggest an overlap between these two entities.     

Mucous membrane plasmacytosis: a case report and review of the literature

Mucous membrane plasmacytosis is a rare, idiopathic condition consisting of a dense plasma-cell infiltrate of the mucous membranes. Zoon first described a plasma-cell infiltrate occurring on the glans penis and plasmacytosis involving other body orifices have been reported under a variety of different terms. White et al. simplified the terminology by suggesting all plasma-cell infiltrates of the mucous membranes of body orifices be termed plasma-cell orificial mucositis. The differential diagnoses and treatment for mucous membrane plasmacytosis are summarized. A middle-aged female with an unusual plasma-cell proliferation disorder of the upper aerodigestive tract is reported for its rarity.     

Papular mucinosis: is the inflammatory cell infiltrate neoplastic? The presence of a monotypic plasma cell population demonstrated by in situ hybridization

Papular mucinosis is a condition reported to be associated with abnormal serum paraproteins and plasma cell dyscrasias. We report a patient with papular mucinosis, without a serum paraprotein or bone marrow plasmacytosis, in whom the affected skin contained a prominent perivascular plasma cell infiltrate. Using in situ hybridization, for k and λ light chain mRNA, these plasma cells were demonstrably monotypic for λ light chain and, therefore, presumably monoclonal and putatively neoplastic. We suggest that the absence of a serum paraprotein and marrow plasmacytosis does not exclude the existence of a plasma cell neoplasm in patients with papular mucinosis. Such plasma cell populations may exist in the affected skin, although their true nature and behaviour remains to be determined.     

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.     

干燥综合征并发反应性浆细胞增生症

报告1例伴反应性浆细胞增生症及全血细胞减少的干燥综合征。患者女，53岁。口、眼干燥3个月。实验室检查示全血细胞减少，抗SSMSSB抗体阳性，双侧泪流量2mm／5min，角膜荧光染色阳性，下唇黏膜活检示涎腺间质内见多灶淋巴细胞浸润，骨髓活检示浆细胞比例增高，偶见幼稚浆细胞。诊断为干燥综合征并发反应性浆细胞增生症。