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.     

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 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.     

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.     

Multicentric plasma cell variant of Castleman's disease with cutaneous involvement

BACKGROUND: Castleman's disease (CD) is a rare low-grade B-cell lymphoproliferative disorder that can be associated with a variety of antibody-mediated paraneoplastic syndromes. The disease is classified clinically by two forms and three histologic variants. METHODS: We describe the clinical and pathological features of a 44-year-old woman who presented with an autoimmune hemolytic anemia, thrombocytosis, polyclonal gammopathy, axillary lymphadenopathy, hepatosplenomegaly, and several erythematous and violaceous nodules and plaques without scaling involving the trunk and extremities. RESULTS: Histologic examination of the skin lesions revealed a deep dermal and subcutaneous nodular mononuclear infiltrate composed primarily of polyclonal plasmacytoid cells without atypia and an increased vascular proliferation. Additional studies including a bone marrow and lymph node biopsy, serum and urine protein electrophoresis, and computed tomography scans supported the diagnosis of multicentric plasma cell variant of CD with an associated autoimmune paraneoplastic hemolytic anemia. CONCLUSION: Cutaneous involvement in CD is part of the multicentric nature and should be considered in the differential diagnosis of a polyclonal plasma cell-rich lymphoproliferative disorder associated with paraneoplastic autoimmune disease.     

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 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.     

Multiple cutaneous reticulohistiocytosis with T‐cell large granular lymphocyte clonopathy

A 63‐year‐old Caucasian man presented with a 4‐month history of disseminated asymptomatic reddish‐brown papulonodular lesions. A skin biopsy showed dermal infiltration with CD68⁺ histiocytes, predominantly with eosinophilic cytoplasm, some with a ground‐glass cytoplasm, and a small number of giant cells. The diagnosis of multiple cutaneous reticulohistiocytosis was made. Bone marrow immunophenotyping due to peripheral blood lymphocytosis revealed the presence of a monoclonal population of CD3⁺, CD8⁺ CD57⁺ large granular lymphocytes. The present case suggests the coexistence of multiple cutaneous reticulohistiocytosis with an underlying disorder.     

Granuloma faciale: distribution of the lesions and review of the literature

Granuloma faciale (GF) is an uncommon inflammatory condition characterized by reddish brown papules and plaques that usually involve the facial area. Extrafacial lesions are rare. Histologically, the lesions are marked by leukocytoclastic vasculitis and extensive fibrin deposition. There are a variety of treatment options available for GF.     

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 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.     

Clinical and pathological study of lichen-planus-like keratosis in China

Lichen-planus-like keratosis is usually diagnosed pathologically; rarely, a definitive diagnosis can be made grossly in the clinic. Only a few cases of lichen-planus-like keratosis have been reported in China. The purpose of this study was to investigate the clinical and pathological features of lichen-planus-like keratosis in China. Fifty cases of lichen-planus-like keratosis patients diagnosed pathologically during a 5-year period in our clinic were analyzed. Clinical features were recorded. Sectioned specimens were subjected to hematoxylin and eosin staining to observe pathological changes. Results showed that there were 34 males and 16 females (ratio 2:1) with an average age of 61.2 years. Most of the lesions were single papules or plaques with rough surfaces. They were distributed on the face, larger than 1 cm, and dark red to brown in color. Only one case (2%) was considered to be lichen-planus-like keratosis clinically. By hematoxylin and eosin staining, solar lentigo and solar elastosis could be found in 68% and 32% of lichen-planus-like keratosis lesions, respectively. Eosinophil (42%) and plasma cell (36%) infiltration was also found frequently. Exoerythrocytes could be detected in 50% of the cases. Lichen-planus-like keratosis is not uncommon in clinical practice in China, the diagnosis of lichen-planus-like keratosis should be made by a combination of clinical manifestations and pathological changes. It is better to classify lichen-planus-like keratosis as a benign skin tumor. More attention should be paid to lichen-planus-like keratosis in China.     

Facial granuloma. On the clinic-histologic extent of variations of finding in 5 patients

The clinical and histopathological findings are presented in five patients with granuloma faciale. The lesions most often occur on the face and are characterized by single or multiple soft, elevated, well-circumscribed nodules or plaques ranging in color from reddish purple to brown. The sites most commonly affected are the nose, temple, checks and forehead. The etiology of granuloma faciale is unknown. The condition is extremely persistent and may last for many years. Histologically, a narrow zone of uninvolved dermis is usually observed between the epidermis and the dermal, dense, polymorphous infiltrate at all levels of the corium. The infiltrate is usually diffuse or shows a nodular perivascular pattern, consisting of lymphocytes, plasma cells, neutrophils and histiocytes together with a varying number of eosinophils. In older lesions the formation of the fibrous tissue may be seen, accompanied by capillary proliferation. Differential diagnosis includes sarcoidosis, discoid lupus erythematosus, erythema elevatum et diutinum, angiolymphoid hyperplasia with eosinophilia and histiocytosis X. The diagnosis of granuloma faciale requires a synopsis of clinical and histological findings.     

CD56+ blastic transformation of chronic myeloid leukemia involving the skin

We report on two patients with chronic myeloid leukemia (CML) who presented blastic transformation involving the skin, with leukemic infiltrates showing unusual morphologic and immunohistologic characteristics. Both patients were elderly men with a 36-month and a 40-month history of CML, respectively. They presented with disseminated, reddish to violaceous papules and plaques (case 1), and with localized reddish nodules on the left temporal area (case 2). Concurrent features of blastic transformation in the bone marrow were observed in one patient (case 1). Histopathologic examination of skin lesions revealed similar features in both cases. There was a moderate to dense dermal infiltrate composed mainly of medium-sized atypical mononuclear myeloid precursor cells with only few relatively well-differentiated cells of the granulocytic series. Histochemical staining for naphthol-ASD-chloroacetate esterase revealed strong positivity (>50% of neoplastic cells) in case 2 and only scattered positivity (< 10% of neoplastic cells) in case 1. Immunohistologic analysis performed on paraffin-embedded sections showed in both cases variable reactivity of neoplastic cells for leucocyte common antigen (CD45), lysozyme, myeloperoxidase, CD11c, CD15, CD43, CD66, CD68, HLA-DR, and the neural cell adhesion molecule (NCAM) CD56. A negative reaction was observed for CD3, CD34, and TdT. The immunohistologic findings were remarkably similar to those reported for acute myeloid leukemia (AML) with monocytic differentiation (French-American-British [FAB] classification, subtype M4). Examination of blasts from the bone marrow performed in one patient (case 1) revealed a similar phenotype also with CD56 expression. In conclusion, our observations show that specific cutaneous infiltrates in CML may show morphologic and immunohistochemical characteristics similar to those observed in AML with monocytic differentiation. Moreover, specific cutaneous manifestations of CML may express CD56.     

免疫表型为CD8~+,CD4~-,CD56~+的蕈样肉芽肿1例

报告1例免疫表型为CD4-,CD8+,CD56+的蕈样肉芽肿(MF)。患者男,21岁。双侧腋窝、躯干及腹股沟大片状灰红色至棕红色斑8年,呈渐进性发展,无任何自觉症状。腹部皮损组织病理示表皮轻度角化不全,真皮内致密淋巴细胞,部分细胞入侵表皮。免疫组化示CD4-,CD8+,CD3+,CD43+,CD68灶状+,CD30灶状+,CD56+,TIA-1-,EBV-。诊断:蕈样肉芽肿。给予阿维A胶囊30mg/d,顿服,糠酸莫米松乳膏及多磺酸粘多糖乳膏外用。皮损缓解,临床随访4个月,病情稳定。     

Cutaneous involvement in multiple myeloma: a clinicopathologic,immunohistochemical, and cytogenetic study of 8 cases

BACKGROUND: Specific cutaneous involvement in patients with multiple myeloma (MM) is very uncommon. It usually occurs in late stages of MM as a reflection of increased tumor cell burden. We studied 8 patients with cutaneous involvement of MM without underlying bony lesions and reviewed the literature on this rare dermatologic manifestation. DESIGN: We were particularly interested in the clinical course of patients with MM and cutaneous metastases, including survival once metastases were detected and the possible influence of various forms of therapy. Our goal was also to identify the immunoglobulin and the light-chain type in these cases, with emphasis on any possible association between a particular immunoglobulin class and cutaneous involvement, as well as the histopathologic, immunohistochemical, and cytogenetic features of the neoplastic plasma cells involving the skin. SETTING: University department of dermatology, university hospital, and private practice. PATIENTS: Medical records and biopsy specimens from 8 patients with MM and specific cutaneous lesions were reviewed. RESULTS: Cutaneous lesions consisted of multiple erythematous or violaceous nodules or plaques with a wide anatomical distribution. Histopathologically, 2 different patterns were identified: nodular and diffuse interstitial. Neoplastic plasma cells showed atypical features, and in 1 case they displayed a spindle shape, giving a sarcomatoid appearance to the lesion. Immunohistochemical studies demonstrated that neoplastic plasma cells were strongly positive for CD79a, CD138, and epithelial membrane antigen, and variably positive for VS38c and CD43. In each case the immunoglobulin profile and the light-chain type expression of the neoplastic cells were the same as those identified in the serum of the patients: 5 cases were IgA lambda; 2 cases were IgG kappa; and 1 case was IgA kappa. In cases 2, 3, and 4, polymerase chain reaction investigations revealed monoclonal rearrangement for IgH genes, whereas the investigations for human herpesvirus 8 and Epstein-Barr virus yielded negative results. Fluorescent in situ hybridization investigations in these 3 cases demonstrated that the cutaneous neoplastic plasma cells showed the deletion of the rb-1 (retinoblastoma) gene. Despite aggressive chemotherapy, all 8 patients died a few months after the development of cutaneous involvement. CONCLUSIONS: In our series, there was a perfect correlation of immunoglobulin and light-chain type between the serum electrophoresis and the cutaneous plasma cells. Patients with MM showed a short survival once cutaneous metastases appeared independently of the therapy. The deletion the rb-1 gene may provide prognostically relevant information to identify a high-risk subset of patients with MM.     

Plaque‐like dermatofibroma: A distinct and rare benign neoplasm?

Unusual large dermatofibromata are reported in a 40-year-old man and a 48-year-old man, who both presented with plaques on a lower limb. The largest plaque in each case was well-defined, reddish brown, indurated and measured 50 mm x 30 mm and 70 mm x 40 mm, respectively. Several satellite lesions were present around the large plaques. Dermoscopic examination showed diffuse homogenous pigmentation in the absence of other diagnostic criteria for dermatofibroma. Light microscopy of biopsies from each patient displayed similar features. There was a dermal proliferation of fibrohistiocytic cells that entrapped intervening thickened collagen fibres. The overlying epidermis was acanthotic, and in some instances this showed basal hyperpigmentation. There was no evidence of malignancy. Immunohistochemical staining was positive for Factor XIIIa and negative for CD34. Based on the histological findings, a diagnosis of dermatofibroma was made for each of these cases. Fewer than 20 adult cases of large dermatofibroma of this scale, designated giant dermatofibroma, have been reported to date; and only two have shown a plaque-like appearance, the remainder being pedunculated. The authors propose plaque-like dermatofibroma as a variety of large dermatofibroma distinct to pedunculated giant dermatofibroma.     

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.     

Kutane Varianten der Langerhans-Zell-Histiozytose im frühen Kindesalter

The term Langerhans cell histiocytosis (LCH) has been endorsed to describe a group of rare entities, a highly variable spectrum of clinical presentations which are all characterized by localized or generalized proliferation of pathological Langerhans cells. We describe two infants with LCH who had very distinct cutaneous lesions. A two month-old infant developed discrete, disseminated red-brown nodules and plaques. In contrast, a second child at the age of nine months presented with brown aggregated scaling, crusted and ulcero-necrotic papules mainly restricted to the abdomen. In both patients, dermatohistopathology showed characteristic proliferation of pathologic Langerhans cells, and staging procedures revealed involvement of lung and bones. These two cases illustrate the marked variability of clinical presentation and disease course of LCH. In addition, important aspects and current concepts of LCH are discussed reviewing relevant and recent literature.     

Palpable arciform migratory erythema in an HIV patient, a CD8+ pseudolymphoma

BACKGROUND: Palpable arciform migratory erythema (PAME) is characterized by large, elevated, reddish annular lesions localized on the upper trunk. As its infiltrate consists predominantly of dense infiltrates of CD4+ lymphocytes with polyclonal T-cell receptor (TCR) gene rearrangement, it has been grouped as a rare member of the T-cell pseudolymphomas. METHODS: We performed histology, immunophenotyping, and TCR-gamma gene rearrangement studies in an human immunodeficiency virus (HIV)-positive patient, CDC stage IIIB, who showed a clinically typical PAME. RESULTS: While TCR-gamma gene rearrangement studies showed a polyclonal infiltrate confirming a pseudolymphoma, 85% of skin-infiltrating lymphocytes were CD8+ T cells. CONCLUSION: PAME may also occur in HIV-positive patients with CD4+ deficiency. Our case demonstrates that regular CD4 counts and immunocompetence are not necessary for its pathogenesis.