Primary effusion lymphoma with skin involvement.

Primary effusion lymphoma (PEL) was once defined as a body cavity-based lymphoma without identifiable contiguous tumour mass, but is now recognised as an independent clinicopathological entity. The case of a 67-year-old Japanese woman with PEL is reported, in which the clinical findings showed a pericardial effusion and multiple erythema on the hypogastrium and inguinal region. The histopathological findings showed a diffuse infiltration of large neoplastic B cells from the dermis to the subcutis. After the disappearance of pericardial effusion without any treatment, she received several rounds of chemotherapy to resolve the skin eruption, but she finally died from multiple organ failure. No tumour mass was observed during the course of her disease.

Axillary apocrine carcinoma with benign apocrine tumours: a case report involving a pathological and immunohistochemical study and review of the literature.

BACKGROUND: Apocrine carcinoma is rare and often occurs in the axilla. This is the second apocrine carcinoma arising in bilateral axillae with associated apocrine hyperplasia to be reported. AIMS/METHODS: Because benign apocrine tumours may be precursors of cancer, this case was investigated immunohistochemically and histologically, and a literature (English and Japanese) review undertaken of cases with coexistent malignant and benign apocrine tumours in the axilla to elucidate the relation between apocrine carcinoma and benign apocrine tumours. RESULTS: Only four cases of axillary apocrine carcinoma with benign apocrine tumours were identified in the literature. In each case, benign apocrine hyperplasia was situated within and surrounding the adenocarcinomatous nests. Staining for epithelial membrane antigen revealed three patterns: (1) poorly differentiated tumour cells showing strong cytoplasmic staining; (2) combined luminal surface and cytoplasmic staining of glandular cells; and (3) a strongly positive lineal staining pattern at the luminal membrane surface, comprising one or two apocrine hyperplastic secretory cells. The basal lesions of apocrine hyperplasia were strongly positive for alpha smooth muscle actin, whereas the periphery of adenomatous lesions showed weaker positive staining, even though the periphery of adenocarcinomatous lesions was negative. CONCLUSIONS: All five apocrine carcinomas with benign apocrine tumours occurred in elderly Japanese men who had bilateral benign apocrine tumours even if affected by unilateral axillary apocrine carcinoma. The immunohistochemical results support the notion that apocrine hyperplasia is a precursor of cancer and that apocrine carcinoma, adenoma, and hyperplasia may be successive steps in the linear progression to carcinoma.

A case of exaggerated mosquito-bite hypersensitivity with Epstein-Barr virus-positive inflammatory cells in the bite lesion.

We describe a unique patient with mosquito-bite hypersensitivity who had extremely high titres of Epstein-Barr virus antibodies. For many years he developed intractable ulcers on the sites of mosquito-bite. Epstein-Barr virus infection was detected in almost all inflammatory cells in the ulcers and in the peripheral blood lymphocytes by using in situ hybridization to Epstein-Barr virus-encoded small ribonucleic acids and by polymerase chain reaction to Epstein-Barr virus DNA. The inflammatory cells in the ulcers were positive for T-cell marker. Our results suggest that the Epstein-Barr virus infection in T cells may participate in the pathogenesis of exaggerated mosquito hypersensitivity and in delayed healing of ulcers on the sites of mosquito-bite.

Monoclonality of infiltrating plasma cells in primary pulmonary nodular amyloidosis: detection with polymerase chain reaction.

AIMS: To investigate the relation between localised amyloidosis and immunocytic dyscrasia. METHODS: Open lung biopsy specimens from a 72 year old man with multiple nodules in the right middle and lower lung were stained with haematoxylin-eosin, Congo red, and antibodies against IgG, IgA, IgM, and kappa and lambda light chains. Semi-nested PCR amplification for the immunoglobulin heavy chain (IgH) gene was performed using consensus primers for the VDJ region of the IgH gene, FR3A, LJH, and VLJH. RESULTS: The biopsy specimens contained eosinophilic amorphous material stained with Congro red and anti-kappa light chain, and surrounded by inflammatory cells intermingled with plasma cells. Plasma cells in the adjacent amorphous material showed cytoplasmic staining with anti-kappa. Polymerase chain reaction revealed a discrete amplified band of apparently uniform size with background smear. CONCLUSIONS: Primary AL type localised amyloidosis involves local accumulation of monoclonal plasma cells and their secreted products, as in nodular cutaneous amyloidosis. Localised AL type nodular amyloidosis is a separate entity in amyloidosis.

Cutaneous presentation of nasal/nasal type T/NK cell lymphoma: clinicopathological findings of four cases.

Epstein-Barr virus (EBV)-associated T/natural killer (NK) cell lymphoma mainly shows nasal lesions, and has recently been shown to be associated with cutaneous T-cell lymphoma (CTCL). The detailed features of CTCL nasal metastasis have yet to be elucidated. We report clinicopathological findings for four cases of cutaneous T/NK cell lymphoma with metastasis to the nose. The four patients presented progressive involvement of nasal lesions of CTCL, an aggressive course and poor outcome. Their pathological and immunohistological findings were consistent with peripheral T/NK cell neoplasm and, in three of four cases, EBER-1 were apparently detected in lymphoma cells by in situ hybridization, and two of four cases were also positive for TIA-1. The polymerase chain reaction (PCR) results showed the identical band from the skin and nasal lesions of the two patients. We also reviewed the cases of similar clinical course and attempted to elucidate clinical, pathological, immunological and genotypic features. The 10 reported cutaneous T/NK cell lymphomas with nasal metastasis revealed a poor prognosis (nine of 10 died at 3-108 months). Six cases of nine showed a positive reaction to EBV, and six cases revealed T-cell receptor beta or -gamma rearrangement. These findings suggest that most cutaneous T/NK cell lymphoma with nasal metastasis are similar to nasal T-cell lymphoma associated with EBV infection. This type of cutaneous T/NK cell lymphoma likely to involve nasal lesions and skin cases seemed to have a poor prognosis.

Nodular localized cutaneous amyloidosis: further demonstration of monoclonality of infiltrating plasma cells in four additional Japanese patients.

Nodular localized cutaneous amyloidosis (NLCA) is a disorder characterized by deposition of amyloid derived from immunoglobulin light chains. We used semi-nested polymerase chain reaction (PCR) to analyse archival paraffin-embedded sections from a previous patient and from four additional, previously reported patients with NLCA to determine whether involvement of monoclonal plasma cells is a universal feature of this condition. The semi-nested PCR analysis revealed one or two amplified bands, around 100-120 bp, for all five cases of NLCA, although the yields varied from case to case. These results suggest that clonal expansion of plasma cells in NLCA may occur locally.

Rudimentary meningocele of the scalp.

A rudimentary meningocele, a variant of primary cutaneous meningioma, was seen on the scalp of a 9-month-old Japanese boy. Clinically, the lesion on the left parietal area was round, about 1.6 cm in diameter, alopecic, and slightly elevated. Histologically, the lesion, located from the dermis to the subcutis, consisted of scattered foci of meningothelial cells, an anastomosing network of empty spaces with psammoma bodies and collagen bodies, and small vessels. Immunohistochemically, the meningothelial cells were positive for vimentin and desmin. Ultrastructurally, they had elongated cytoplasmic processes, intermediate filaments in the cytoplasm, and desmosomal junctions.

The value of genotypic analysis in the assessment of cutaneous plasmacytomas.

We report a 79-year-old woman with primary cutaneous plasmacytoma in whom polymerase chain reaction (PCR) was used to demonstrate the monoclonality of the tumour. Four years after presentation, further skin lesions occurred and PCR again showed evidence of monoclonality despite the histology being non-specific. The reported frequency of multiple primary cutaneous plasmacytoma is increasing, and the mortality rate of patients with multiple lesions is three times that of those with a solitary lesion. PCR may be a useful technique for assessing such patients at presentation.

Nodular localized cutaneous amyloidosis: detection of monoclonality of infiltrating plasma cells by polymerase chain reaction.

We report nodular localized cutaneous amyloidosis arising in a 74-year-old woman as a single nodule at the left angle of the mouth. Histological examination revealed eosinophilic material deposited in the dermis, identified as amyloid by staining with Dylon stain, and antiamyloid P component, and by electron microscopy. The amyloid material was further determined immunohistochemically to be of AL fibril protein type derived from lambda light chains. Infiltrating plasma cells, around blood vessels in the dermis, stained monotypically with anti-lambda antibody, suggesting plasma cell monoclonality. Semi-nested polymerase chain reaction (PCR), for rearrangement of immunoglobulin heavy chain gene using paraffin-embedded sections, yielded two distinct amplified bands, indicating monoclonality of the infiltrating plasma cells. These findings support the hypothesis that primary localized nodular amyloidosis involves local accumulation of monoclonal plasma cells and their secreted products. The two amplified bands observed on PCR may, in the present case, reflect abnormal secretion of immunoglobulin light chains.

Primary cutaneous meningioma on the scalp: report of two siblings.

Primary cutaneous meningioma (PCM) is a rare tumor whose pathogenesis is quite obscure. We reported PCMs occurring on almost the same occipital region of two siblings studied by histology, immunohistochemistry, and electron microscopy. Both lesions were attached to duras, but extracranial. One lesion was histologically diagnosed as meningothelial meningioma; its tumor cells showed electron microscopically interdigitating cytoplasmic processes with junctional complexes. The other was interpreted as fibroblastic meningioma; its tumor cells were arranged linearly in a stepping-stone arrangement and had small dense bodies in the cytoplasm. The tumor cells stained positively with anti-vimentin antibody. Both lesions had adenomatous hyperplasia of the eccrine glands. Although the histologic and electron microscopic features of these two lesions slightly differed from each other, their pathogenesis was essentially considered to be acoelic or rudimentary meningocele.

Association of tumor necrosis factor-alpha gene expression and apoptotic cell death with regression of Shope papillomas.

The objective of this study was to test the hypothesis that spontaneous regression of Shope papillomas involves tumor necrosis factor-alpha and apoptotic cell death of the papilloma cells. In situ hybridization using RNA probes of rabbit tumor necrosis factor-alpha revealed tumor necrosis factor-alpha mRNA in most of the numerous mononuclear cells infiltrating the upper dermis of regressing papillomas and at the dermoepidermal junction. Such cells in progressing papillomas were much fewer in number and were located in the deeper dermis. In situ terminal deoxynucleotidyl transferase assay demonstrated DNA strand breaks in many scattered epidermal keratinocytes of regressing papillomas but in only a few thin layers just beneath the horny layer in progressing papillomas. Electron microscopy demonstrated that regressing papillomas contained many apoptotic bodies and keratinocytes showing apoptotic changes such as chromatin condensation, degradation of condensed nuclei, surface protuberances, and a filamentous degeneration, as well as infiltrating lymphocytes and macrophages. We propose that tumor necrosis factor-alpha produced by infiltrating mononuclear cells probably plays a role in the papilloma regression.

Detection of human papillomavirus type 2a DNA in verrucae vulgares by electron microscopic in situ hybridization.

Electron microscopic in situ hybridization (EMISH) of common warts (verrucae vulgares) of the hands was performed using a biotinylated human papillomavirus type 2a (HPV-2a) DNA probe and immunogold labelling of ultrathin sections of 2% glutaraldehyde-fixed, Lowicryl K4M-embedded tissues. It was first established that the warts contained HPV-2a DNA by light microscopic in situ hybridization. The HPV-2a probe chiefly labelled cells in the horny, granular and upper spinous layers of the epidermis, and labelling decreased towards the basal cell layer. The gold particles were located precisely on the viral particles in the nuclei of granular cells. The lower limit of detection by EMISH was found to be the keratinocytes of the third cellular layer above the basal cells. These keratinocytes showed evidence of a viral cytopathic effect, suggesting that vegetative DNA replication in infected keratinocytes occurs at least as early as this level of the epidermis.

Acquired tufted angioma showing spontaneous regression.

We report a 62-year-old woman with acquired tufted angioma. Several scattered reddish nodules were present on the neck and upper chest. During a follow-up period of 6 months, some of the lesions showed transient spontaneous regression and one disappeared completely. Electron microscopy revealed that a few tumour cells contained Weibel-Palade bodies in their cytoplasm.

Proteus syndrome: report of the first Japanese case with special reference to differentiation from Klippel-Trenaunay-Weber syndrome.

This is the first report of a Japanese girl with Proteus syndrome. She presented with growth acceleration and precocious development of the left breast as well as macrodactyly, hemihypertrophy, a subcutaneous preaxillary mass, portwine stains, connective tissue nevi, and a depigmented macule. All these abnormalities were confined to the left side of her body. Although most of the manifestations fit those of Proteus syndrome, the presence of the portwine stains and hemihypertrophy also suggested Klippel-Trenaunay-Weber syndrome. The findings in our patient suggest that the most important characteristic distinguishing Proteus syndrome from Klippel-Trenaunay-Weber syndrome is the presence of functional abnormalities such as a growth spurt and precocious breast development. Proteus syndrome may be genetically different from the Klippel-Trenaunay-Weber syndrome.