Takae Hirone

Annular Elastolytic Giant Cell Granuloma: Response to Cyclosporin A

A case of annular elastolytic giant cell granuloma with a good response to cyclosporin A is reported. A 62‐year‐old man developed multiple annular patches on the trunk with elevated and indurated borders. Biopsy specimens from the border showed granulomatous inflammation in the mid dermis with phagocytosis of elastic fibers by giant cells. Biopsy specimens from the center showed dense collagen formation without inflammation. Immunological investigation of perivascular infiltrating cells in the lesions revealed a predominance of CD4+ cells over CD8+ cells. Our case showed a good response to cyclosporin A (5 mg/kg/day) for eight weeks. There were no adverse effects and no recurrences for one month after discontinuation of cyclosporin A.

T CELL LYMPHOMA PRESENTING CLINICAL AND MORPHOLOGICAL FEATURES RESEMBLING POLYMORPHIC RETICULOSIS AND LYMPHOMATOID GRANULOMATOSIS

A male, 53 years old, complained of a mass on the left side of neck. The biopsy specimens revealed extensive necrotizing lesions with polymorphonuclear leukocyte Infiltration. About three years later a recurrence from the same site was noted and the reblopsy specimens showed features of malignant lymphoma of pleomorphic type. Subsequently multiple cutaneous nodules and then ulcerative lesions of the midfacial region developed and repeated biopsies were done. Immunological examination indicated that the lymphoid cells obtained from the skin lesion had predominantly T‐lymphocyte marker. He died of massive hemorrhage from the nasopharyngeal lesion 51 months after the first admission. Autopsy revealed multiple organ Involvements, including the nasopharynx, lungs, subcutaneous tissue, and adrenal gland. The morphological features In the present case were compatible with a diagnosis of lymphomatoid granulomatosis or polymorphic reticulosis (midline malignant reticulosis), and were thought to be best designated as T cell lymphoma.

Tape stripping induces marked epidermal proliferation and altered TGF-α expression in non-lesional psoriatic skin

Psoriasis is a chronic inflammatory disorder characterized by epidermal hyperproliferation. Although recent evidence suggests that T cell activation is a primary trigger for psoriasis lesions, there may be alterations in the keratinocyte growth regulatory pathways which induce epidermal hyperproliferation in psoriatic patients. To test this hypothesis, we investigated the proliferative activity of epidermal keratinocytes 48 h after tape stripping, one of the standard mechanical ways to stimulate the epidermis, in 20 psoriasis patients and in 18 controls. Epidermal cell kinetics were assessed with DNA flow cytometry, the mitotic index, bromodeoxyuridine incorporation, Ki-67 antigen expression and DNA polymerase alpha expression. The expression of TGF-alpha and EGF receptors, critical mediators of keratinocyte proliferation, were also investigated immunohistochemically. The results of multiparameter assays showed that the baseline proliferative activity in uninvolved skin was the same in psoriasis patients and normal controls. After tape stripping, although both psoriasis patients and the normal controls showed significant increases in epidermal cell proliferation, the values of all the parameters investigated were significantly greater in the psoriasis patients than in the normal controls. EGF receptors were overexpressed in basal and suprabasal keratinocytes after tape stripping in both the psoriasis patients and the normal controls. In contrast, overexpression of TGF-alpha was only observed in the patients with psoriasis, which may explain their increased proliferative response to trauma.

Factor-XIIIa-positive cells in normal peripheral nerves and cutaneous neurofibromas of type-1 neurofibromatosis

Cutaneous neurofibromas consist of heterogenous cell populations including Schwann cells, perineurial cells, and fibroblastlike cells. However, the histogenesis of neurofibromas, particularly the origin and nature of the fibroblastlike cells, is still controversial. Recently, cells containing blood coagulation factor XIIIa have been reported in cutaneous neurofibromas, although their identity is uncertain. In this report, by the combination of double immunohistochemical straining and immunoelectron microscopy, we demonstrate that factor-XIIIa-positive cells are distinct from Schwann cells, perineurial cells, endothelial cells, mast cells, and conventional macrophages, but correspond to the fibroblastlike cells in cutaneous neurofibromas. Such factor-XIIIa-positive cells in cutaneous neurofibromas, however, differ from conventional fibroblasts in the strong expression of HLA-DR antigen and lack of prolyl 4-hydroxylase. Similarly, so-called endoneurial fibroblasts and, occasionally, connective tissue cells within perineurium and epineurium in normal peripheral nerve fibers express factor XIIIa as well as HLA-DR antigen. The results suggest that fibro-blastlike cells in cutaneous neurofibromas are probably derived from factor-XIIIa- and HLA-DR antigen-positive connective tissue cells in peripheral nerves. The role of such factor-XIIIa-positive cells in the growth and development of cutaneous neurofibromas is discussed.

Colloid degeneration of the skin--a case report.

A patient with multiple nodules on the chin and scalp is presented. Histologically, the nodules were composed of amorphous materials which also completely filled up the whole dermis. Histochemical observation indicated that the amorphous material in the nodule was a kind of glycoprotein. Electron microscopic examination revealed that the material consists of short wavy fibrils, 60 to 100 A in diameter, and of a low electron‐dense amorphous substance. Biochemical examination supported the view that the amorphous material is so‐called sialomucoid. The nodules were finally diagnosed as colloid degeneration of the skin (nodular type).

An Adult Case of Histiocytosis X with a Vulvar Ulcer and Multiple Bone Lesions

A 62‐year‐old female with histiocytosis X presented with a vulvar ulcer. Multiple osteolytic lesions were later detected. Histological examination of the ulcerated skin showed diffuse proliferation of histiocytic cells with folded nuclei and pale eosinophilic cytoplasm. Immunohistochemistry revealed S100 protein and vimentin as well as CD1a, CD4, and HLA‐DR antigens in the proliferating cells. Electron microscopy demonstrated Birbeck granules in the cytoplasm of the cells. The patient was successfully treated by complete surgical excision of the ulcer followed by radiotherapy for recurrent vulvar erythema.

Action Spectrum for Bergamot-Oil Phototoxicity Measured by Sunburn Cell Counting

The present study investigated the phototoxic effect of bergamot oil and its photosensitive component, bergapten, on sunburn cell (SBC) production in guinea pig skin. The back skin was pretreated with bergamot oil or bergapten and exposed to monochromatic light under various conditions. After irradiation, skin specimens were excised, and histological sections were prepared. The number of sunburn cells in the interfollicular epidermis was counted. The SBC formation by bergamot oil or bergapten plus UVB radiation was the same as that without pretreatment with any photosensitizer. In contrast, a significant number of SBCs were induced by bergamot oil or bergapten plus UVA radiation, but no SBCs were found after the treatment with UVA alone. The result indicates that bergamot oil or bergapten was photosensitized by UVA irradiation. The SBCs were linearly increased in a UV‐dose dependent manner. On the basis of the regression lines, an action spectrum and spectral peak for the photosensitizers plus UVA were obtained. The action spectrum for bergamot oil‐ and bergapten‐induced SBC formation was in the ranges of 325–365 nm and 325–350 nm, and their spectral peaks were at 335–345 nm and 335–350 nm, respectively. The data are in good accordance with those estimated from skin erythema reactions. Therefore, counting SBCs is a very useful parameter for quantitative evaluation of phototoxicity.

SERUM LEVELS OF ETRETINATE (RO 10-9359) AND ITS METABOLITE (RO 10-1670)

Serum levels of Etretinate (ET, Ro 10‐9359) and its metabolite (AM, Ro 10‐1670) were monitored during and after cessation of ET treatment in 12 patients with acquired disorders of keratinization who underwent the ET therapy at initial doses of 30 to 60 mg/day. Studies were made of the possible correlations between ET and AM serum levels, the appearance and disappearance of side effects, and elimination time profiles. Side effects appeared 1 to 2 weeks after the initiation of ET administration in all cases, although serum ET and AM levels at this time varied considerably, indicating individual differences in ET and AM susceptibility in the appearance of side effects. Serum ET and AM levels at the time of disappearance of side effects were lower than those at the time of appearance, suggesting some correlation of side effects with serum levels of ET, AM, or both. Serum level curves for ET and AM after cessation of ET administration were diverse. The maximum ET and AM elimination times from serum were 3 and 6 months, respectively.

Complication cases of senile keratosis, Bowen disease and squamous cell carcinoma.

A 74-year old man developed 2 squamous cell carcinomas, 2 Bowen carcinomas, 4 Bowens disease and multiple senile keratoses on his lower legs and genitalia. Immunohistochemistry for p53 protein revealed that 20 out of 30 (67%) excised lesions expressed mutant p53 protein. Although the patient had no history of arsenic ingestions, tar was suspected as a carcinogenic agent.

A case of trichilemmal carcinoma for which electron irradiation was effective.

患者は91歳の女性。額に原発性trichilemmal carcinomaがあり, X線CTにより, 左の耳前部リンパ節・耳下腺・咬筋への転移が検出された。外科的手術は困難と考えられたので放射線治療を行った。原発巣に対して8MeV電子線を総照射量5500rad照射, 転移巣に対して12MeV電子線を総照射量6400rad照射した。照射後原発巣も転移巣も消退した。しかし, 4ヵ月後左頸部リンパ節に, さらにその2ヵ月後右頸部リンパ節に転移が認められた。これらの転移にも放射線治療は有効であった。