Kan Niizuma

Pigmented Dermatofibrosarcoma Protuberans (Bednaf Tumor)

An autopsy case of pigmented dermatofibrosarcoma protuberans (Bednaf tumor) with systemic metastasis is reported. No previous example of this tumor showing widespread metastasis has been reported in the literature. The patient, a 45 year old man, developed a tumor on the right upper arm. The tumor recurred twice and metastasized to other parts of the skin, lungs and brain during the 8‐year clinical course. The primary tumor contained melanin‐laden tumor cells and showed a storiform growth pattern. Autopsy confirmed multiple metastatic lesions in the skin, lungs, brain, thyroid, pancreas, stomach, small intestine and thigh muscles. The recurrent and metastatic tumors lacked both melanin production and the storiform arrangement, and instead revealed “fibro sarcomatous” change with a herring bone or interlacing pattern of growth. Acta Pathol Jpn 40: 935–940, 1990.

Isolated Epidermolytic Acanthoma

A solitary scrotal lesion of isolated epidermolytic acanthoma was studied by optical microscopy. The lesion displayed typical granular degeneration. The tumor parenchyma comprised several foci extendi

Solitary neurofibroma: a case of subungual neurofibroma on the right third finger

We report here the results of an optical and electron microscopic investigation of a subungual neurofibroma of a very rare solitary type. As far as we can ascertain from an extensive search of the literature, no case of this kind has ever been documented in Japan. In the Western literature, Shelly et al. [5] have reported a case of subungual neurofibroma, but give only a photomicrograph, with no histopathological illustrations, and Runne et al. [4], likewise, give only macroscopic photographs, without histopathological information. Unfortunately, the descriptions given by Shelly et al. and Runne et al. offer no indication as to whether, in the cases they refer to, the neurofibroma fortuitously developed subungually as a feature of more general neurofibromatosis (von Recklinghausens disease), or was a purely solitary subungual neurofibroma of the type described here. The subject of the investigation was female, age 13 years 1 month. A slight general protuberance of the nail on the right finger was noticed at about 11 years 1 month. Initially, deformation of the nail proceeded extremely slowly, without pain or other subjective symptoms, but after two years the patient noticed a tumorous lesion beneath the nail in question, and attended our clinic on 7 January 1989. At first examination of the lesion the nail on the right third finger had deformed overall into a flattened dome shape and presented moderate tenderness from the ulnar half only. X-ray examination revelaed mild compression atrophy on the ulnar side of the distal phalanx. Figure I shows a general view of the tumour after ablation of the nail. The tumour was completely

THE MAJOR HISTOCOMPATIBILITY COMPLEX IN PSORIASIS VULGARIS: III. HLA-C ANTIGENS

HLA‐C typing was performed on 40 unrelated Japanese patients with psoriasis vulgaris. The patient group consisted of 22 patients who had psoriatic HLA antigens i.e., B13 in seven cases, B17 in one case and B37 in 14 cases; and 18 patients who had none of these specific HLA antigens. HLA‐C typing was also performed on 40 unrelated healthy Japanese controls. Cw6 was found in 55.0% of the patients as compared with 5.0% of the healthy controls. All patients (21 cases) who had either B13 or B37 were positive for Cw6. Both controls who were positive for Cw6 also had B37. These results suggest the possibility of a linkage disequilibrium between Cw6 from locus C and B13 and B37 from locus B, and/or an association between Cw6 and susceptibility to psoriasis.

AN ELECTRON MICROSCOPIC STUDY OF LIPID DROPLETS IN THE NORMAL HUMAN SEBACEOUS GLAND

Lipid droplet ultrastructure in the normal human sebaceous gland was studied by the conventional method, the lipid retention procedure, and the tannic acid fixation method. The results strongly supported the views that (i) lipid droplets originate from the Golgi apparatus, and (ii) the lipid droplets do not mature by fusion of neighbouring droplets but as a result of direct participation of the smooth endoplasmic reticulum. (iii) A tri‐laminar true limiting membrane was clearly detected around all lipid droplets in certain exceptional acini, but was not otherwise detected in mature sebaceous acini.

Histochemistry in psoriasis

SummaryThe 5′-nucleotidase activity in psoriatic and normal human epidermis was studied in comparison to acid phosphatase activity. The optimum pH in normal human epidermis was about 5.0 at room temperature. The activity of both enzymes was found to be high in the transitional zone.Acid phosphatase (non-specific) activity was strongly positive in the psoriatic parakeratotic horny layers whereas 5′-nucleotidase activity in that area was completely absent.The results suggest that the enzyme which degrades nucleoside-5′-phosphate to nucleoside and inorganic phosphate is not acid phosphatase but 5′-nucleotidase. Nuclear preservation in psoriatic hyperkeratosis was attributed to absence or inactivation of specific enzymes of nuclear degradation, such as 5′-nucleotidase, rather than acid phosphatase.ZusammenfassungDie 5′-Nukleotidaseaktivität der psoriatischen und normalen menschlichen Epidermis wurde in Verbindung im Vergleich mit der sauren Phosphataseaktivität untersucht. Das optimale pH der normalen menschlichen Epidermis war um 5,0 bei Raumtemperatur. Die Aktivität beider Enzyme wurde erhöht in der transitionalen Zone gefunden.Die saure Phosphataseaktivität (nicht spezifisch) war ausgeprägt positiv in der psoriatischen parakeratotischen Hornschicht, während die 5-Nukleotidaseaktivität in dieser Zone völlig fehlte.Diese Ergebnisse weisen darauf hin, daß das Enzym, welches Nukleosid-5-phosphatat zum Nukleosid und anorganischen Phosphat abbaut, nicht die saure Phosphatase, sondern die 5-Nukleotidase ist. Der Bestand nuklearer Strukturen in der psoriatischen Hyperkeratose wird auf das Fehlen oder die Inaktivierung spezifischer Enzyme des Kernabbaus, wie 5-Nukleotidase zurückgeführt. Die saure Phosphatase kommt dafür weniger in Betracht.

Juvenile melanoma—A case report and histogenetic

SummaryA case of juvenile melanoma which appeared to be completely intradermal was studied by optical and electron microscopy. The histological dopa reaction was negative and melanin granules could not be detected with Fontana-Masson stain, but detailed electron microscopic examination revealed melanosomes very similar to those found in intradermal nevus cells. The tumor was therefore thought to be composed exclusively of melanogenic nevus cells, and participation of schwannian nevoblasts could be excluded. This view would support the concept of the unitary origin of such nevus cells.ZusammenfassungEin völlig intradermal liegendes juveniles Melanom wurde histologisch und elektronenmikroskopisch untersucht. Die Dopa-Reaktion war negativ. Melaningranula konnten nicht mit der Fontana-Masson-Färbung nachgewiesen werden. Elektronenmikroskopisch wurden aber Melanosomen aufgefunden, die denen der intradermalen Naevuszellen ähneln. Der Tumor dürfte aus diesen Gründen völlig aus melanogenen Naevuszellen aufgebaut sein. Hinweise auf Schwannsche Neoblasten wurden vermißt. Die Naevuszellen dürften damit einen einheitlichen Urstamm haben.

Lipid Droplet of Sebaceous Carcinoma

SummaryThe ultrastructure of lipid droplets of sebaceous carcinoma (Meibomian gland carcinoma) was studied by conventional methods and a lipid-retention procedure. Two different structures were clearly differentiated by the latter technique. The peripheral part of the lipid droplet was composed of whirled lamellar structures (myelin-like structures) while the interior appeared to be continuously homogenous. The lamellar structures were presumed to have come from smooth-surfaced endoplasmic reticulum, and to transform to the amorphous material—triglyceride—during maturation of the lipid droplets.A true limiting membrane or membranous shell around the lipid droplet was not deceted.ZusammenfassungDie Ultrastruktur der Lipidtropfen eines Carcinoms der Meibomianschen Drüsen wurde mittels konventioneller Methoden und einer Lipid-Retentionstechnik untersucht. Zwei unterschiedliche Strukturen ließen sich durch die letzte Technik klar herausarbeiten. Der periphere Teil der Lipidtropfen bestand aus wirbelförmigen lamellären Strukturen (myelinähnlichen Strukturen), während das Zentrum des Tropfens homogen erschien. Es wird angenommen, daß die lamellären Strukturen sich von dem endoplasmatischen Reticulum ableiten und sich während der Reifung der Lipidtropfen zu einem amorphen Material (Triglyceride) transformieren. Ein abschließendes Membran um die Lipidtropfen wurde nicht beobachtet.