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.

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.

Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature.

This report describes the case of a 76 year old man who suffered from febrile ulceronecrotic Mucha-Habermann disease (FUMHD). Despite this patient's typical clinical and histological findings, the fulminating course led to death. Polymerase chain reaction (PCR) analysis of the skin lesions showed that the infiltrating cells were monoclonal in origin and were from an aberrant clone. FUMHD is a very rare, febrile variant type of pityriasis lichenoides et varioliformis acuta, and is characterised by necrotic cutaneous ulcerations associated with high fever and systemic manifestations. Including this present case, only 18 cases of FUMHD have been reported. FUMHD can occur in both adults and children, although there are several differences between the manifestations of the disease in the two groups. One major difference is prognosis: all cases resulting in fatality are of the adult type, whereas no fatal cases have been reported among children. The aberrant clone detected by PCR may be responsible for host responses, resulting in the severe symptoms observed in this disorder.

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.

Linear focal elastosis. An ultrastructural study.

We studied an 86-year-old Japanese man with linear focal elastosis. The lesions were asymptomatic yellow striae in the lumbar region, histologically composed of massive, well-demarcated basophilic fibers that stained positively with elastic tissue stains. Electron microscopy revealed fine, reticular or granular electron-dense materials, and elastic fiber microfibril-like materials in the matrix, in addition to numerous mature and immature elastic fibers. These findings suggest that active elastogenesis was occurring in the lesions. The four cases reported so far have the three common features of age, sex, and lesion location.

Posttraumatic occurrence of infantile digital fibromatosis. A histologic and electron microscopic study.

We encountered a patient with infantile digital fibromatosis. The representative lesion was a firm, indurated nodule, which developed in the scar of a previous injury on the skin of the left thigh of an 11-year-old girl. Electron microscopy revealed tumor cells that were strongly reminiscent of myofibroblasts. Histopathologically, the cytoplasmic inclusion bodies in the tumor cells stained positively or negatively with hematoxylin-eosin. Electron microscopically, they were either well-defined dense bodies that often showed a doughnutlike appearance or ill-demarcated dense bodies. Spotty calcified foci were seen in the central area of the tumor mass.

Lichen sclerosus et atrophicus. A histological, immunohistochemical and electron microscopic study.

We studied three cases of genital lichen sclerosus et atrophicus (LSA) using histological, immunohistochemical and ultrastructural techniques to elucidate the characteristics of the collagen fibres, the elastic fibres and the interfilamentous matrix in the upper dermal homogeneous zone. In the early stages of LSA, the homogeneous zone caused elaunin fibres to push downwards and partially disappear, the collagen fibres were presumably phagocytosed by fibroblasts, and there were peculiar cells containing numerous vacuoles in the cytoplasm. In the advanced stages of LSA, the homogeneous zones showed newly formed collagen fibres and amorphous, dispersed, medium electron-dense substances that were probably composed of elastin or elastin-like substances and microfilaments originating from the collagen fibres. Immunohistochemically, there were numerous anti-aortic alpha-elastin-positive substances, but there was no positivity for anti-amyloid P component. These findings suggest that in advanced LSA a hybrid substance (elastocollagenous mass) between collagen fibres and elastic fibres may be formed in the homogeneous zone.

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.

Psoriasis verrucosa showing peculiar histologic features.

A 60-year-old man had a 10-year history of linearly arranged verrucous papules on his left fingers in addition to psoriatic erythematosquamous papules and plaques on his trunk and extremities. The verrucous papules were clinically classified into two types: dome-shaped papules with a keratotic plug and crater-shaped papules with a central depression. Histopathologically, the former showed cup-shaped acanthosis with a parakeratotic column containing Munro's microabscesses, while the latter showed a cup-shaped epidermal invagination with Kogoj's spongiform pustule-like changes. Both types of papules were clinically diagnosed as psoriasis verrucosa, but their histologic features differed from those reported previously.

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.

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.

Cutaneous sarcoidosis showing multiple papular eruptions with keratotic plugs.

A 26-year-old woman developed 249 discrete papules on her lower extremities. Twenty-three of them had keratotic plugs. Histologic examination demonstrated granulomatous foci consisting of epithelioid cells and a epidermal invagination which was disrupted at the base and lateral sites. This invagination was considered to represent transepithelial elimination.

Paget's disease of the male breast--report of a case and histopathologic study.

We report a 61-year-old male with mammary Paget's disease. Physical examination revealed a slightly exudative erythema at the areola and a reddish, enlarged left nipple. No tumor or left axillary lymph nodes was palpable. He underwent a left modified radical mastectomy. Histologically, there was an intraductal carcinoma in the upper portion of the mammary ducts. The axillary lymph nodes that were examined were free of metastasis. Paget cells had neither estrogen nor progesterone receptors. We speculated that the histogenesis of Paget cells involved carcinoma cells that invaded the epidermis of the nipple.

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.

Linear focal elastosis associated with striae distensae in an elderly woman.

We report the case of a 73-year-old woman with linear focal elastosis (LFE) associated with striae distensae. Yellow palpable striae were found extending horizontally in the lumbar region. The yellow striae appeared to be joined to striae distensae in the lateral sides of the yellow striae. Striae distensae were also present in regions other than the back but were not associated with yellow striae. Histologic examination of a yellow stria revealed a focal increase in elastic fibers in the dermis. Although there have been some unusual cases of LFE arising in women or in young persons, this disorder still predominantly affects aged men. A fairly uniform feature of LFE is its occurrence on the dorsal skin, suggesting involvement of this specific anatomical site in its pathogenesis. We suspect that LFE usually arises de novo in the skin, although striae distensae may also be a cause of it.