Nihon Hifuka Gakkai zasshi. The Japanese journal of dermatology最新文献

Three cases of neurilemmomatosis are reported. A 22-year-old man without any relatives with similar symptoms visited our clinic, complaining of multiple skin tumors since the age of 15 and bilateral acoustic nerve symptoms since 19. Physical examination revealed no pigmented or depigmented spots. Histopathological examination of the eight tumors excised from the skin, acoustic nerve and spinal cord showed that these were all neurilemmomas. A 36-year-old man with a 15 year history of multiple skin tumors and one year history of acoustic nerve symptoms was seen at our clinic, revealing no pigmentary disorders. The tumors excised from the skin and bilateral acoustic nerves were all neurilemmomas histopathologically. A 5-year-old boy, who was the only child of the second case and had had several skin tumors since his birth, visited us after postoperative death of his father. He revealed no pigmentary abnormalities. The histology of the skin tumor was neurilemmoma. The absence of neurofibromas and pigmented spots in these patients with neurilemmomatosis suggests that this disorder might be close to, but distinct from neurofibromatosis. Although familial cases of neurilemmomatosis like our case 2 and 3 reported so far are very few, they support a possibility that neurilemmomatosis might be a genetically determined neurocutaneous syndrome, a kind of phacomatosis.

The basal cells of old and young guinea pigs were separated by use of density gradient centrifugation. DNA contents of basal cells were measured on the smear specimens prepared by Feulgen's stainings. DNA synthetic activities of old guinea pigs and those of young guinea pigs were almost the same. These date suggested that there was no specific relationship between the DNA synthetic activity and aging of epidermis.

A case of Farber's disease (disseminated lipogranulomatosis) is reported. A Japanese boy presented with progressive joint deformity, a hoarse voice, mental retardation and skin granulomatous lesions. He died of bronchopneumonia at the age of 2 years. Biopsied specimen from the perianal granulomatous lesion showed many macrophage contained lipids, and acidic oligo-, or polysaccharides, or, glycoconjugates. Electron microscopic study revealed, in addition to curvilinear tubular bodies (Farber's bodies), numerous granular structures accumulated in the cytoplasm of the macrophage. Biochemical analysis demonstrated a storage of free ceramide in the patient's liver, and confirmed the patient's diagnosis. This is the 5th case reported in Japan.

Inhibitory effect of arbutin (hydroquinone-beta-D-glucopyranoside) on the melanogenesis was studied biochemically using cultured B16 melanoma cells. The maximum arbutin concentration lacking an inhibitory effect on cell growth was 5 X 10(-5) M. At this concentration, melanin content per cell was decreased significantly to about 39%, compared with that of arbutin untreated cells. Also, tyrosinase activity of arbutin treated cells was decreased significantly. When arbutin was added to B16 melanoma cell suspension, arbutin was not hydrolyzed to liberate hydroquinone. Further, tyrosinase activity in crude preparations from B16 melanoma cells was inhibited by arbutin. From these results, it is suggested that arbutin can inhibit the melanogenesis by affecting not only the synthesis but also the activity of tyrosinase rather than by killing melanocytes B16 melanoma cells. Also, it is suggested that hydroquinone is not responsible for the inhibitory effect of arbutin on the melanogenesis.

Abnormalities of triglyceride (TG) metabolism are considered to play an important role in pathogenesis of psoriasis. Two psoriatic patients with hypertriglycemia were treated with 750 mg of oral Clofibrate daily. While they were treated, both patients showed improvement of psoriasis. Upon cessation of treatment the lesions returned. During the treatment, levels of serum TG, apolipoprotein C-III (apo C-III), and apo E were reduced significantly. The analysis of serum fatty acids revealed a change in the level of linoleic acid. The serum linoleic acid level, which had been low in both cases before the treatment, increased in one case and decreased in other during the treatment. In the biopsy specimen from the post-treatment plaque, both capillary proliferation and endothelial swelling in the dermis were less prominent. There was a moderate reduction in the number of lymphocytic cells, and an increase in that of histiocytic cells. Clofibrate treatment improved TG metabolism and the histological and clinical findings in the psoriatic lesion.

We examined alpha-actin and an angiogenic factor, basic fibroblast growth factor (bFGF), by immunohistochemistry and Western blot analysis in four angiofibromas (AFs) and a connective tissue nevus (CTN) obtained from two patients with tuberous sclerosis (TS). There was an increase of alpha-actin-positive microvessels in the papillary and the upper reticular dermis of AFs and a CTN as compared to those in normal skin. The main localization of alpha-actin in the microvessels of AFs was considered to be pericytes. Many microvessels and a few interstitial fibroblast-like cells in frozen sections of AFs and a CTN were positively stained for bFGF, and most of the bFGF-positive microvessels corresponded to those containing alpha-actin as determined by double immunostaining. These data suggested a possible role of increased bFGF in stimulating angiogenesis and/or mainitaining vessels in AFs, although augmentation of mitotic activity was not noted by staining with Ki-67. Further investigations on the identification of the bFGF-producing cells and biological function of bFGF in AFs may be a clue to elucidate pathomechanisms of AFs.

We sent out a questionnaire to 47 patients of SLE and its similar diseases as to their past histories of chilblain. The results of the patients were compared to those of 141 cases of control. Although the percentage of cases who had revealed chilblain frequently (40.0%) and the age of chilblain onset (mean: 10.8 years old) in SLE group were not significantly different from those of control (28.4% and 12.4 years old, respectively), the chilblain which SLE patients developed had some characteristics compared to that of control, (1) higher incidence of chilblain episodes, (2) longer duration until cure, (3) more liability that chilblain leads to erosion or ulceration and (4) frequent occurrence of chilblain in the other seasons than winter. Especially SLE patients with the characteristic of (4) had higher association rates of Raynaud's phenomena, chilblain LE and livedo, suggesting disorder of peripheral circulation. It was also revealed that females are generally more liable to develop chilblain than males (females: 40.0%, males: 13.1%). Those results suggest some important relationship between chilblain and LE lesions. It is supposed that chilblain with the characteristics described above may possibly be transformed into LE lesions.

The patient is a 5-year-old boy. There was no family history of the similar skin eruptions. The erythema with scales appeared on his head, face and neck at 1 month of birth. The erythematous hyperkeratotic plaques spread symmetrically. There was no follicular components. The eruption was chronic and persistent. In histopathology, hyperkeratosis with focal parakeratosis, acanthosis and intact granular layer were observed. In electron microscopy, a large number of mitochondria that frequently appeared to be swollen and lipidlike vacuoles in corneocytes were observed. On account of the features, we diagnosed this case as progressive symmetric erythrokeratodermia (PSE). PSE is a rare disease, and besides, it is difficult to define PSE because of contradictions in existing literatures, the variability of clinical expression and the lack of histologic, hereditary, and biochemical criteria. Nearly 70 cases were diagnosed as PSE and its variants in Japan i.e. 4 cases with PSE, 8 cases with progressive erythrokeratodermia, and 58 cases with Asahi-Ijiri disease and its variants. However, only 2 cases diagnosed as PSE and 4 cases nominated as others satisfied the recently established criteria by Ruiz-Maldonado, et al. In this paper, reported cases of PSE and its variants were reviewed and reassessed.

A 39-year-old woman had numerous keratotic papules on the volar surface of the right palm, both soles, fingers and toes. There were some spiny plugs on the edges of her toes. Histologic examination revealed a cornoid lamella. "Punctate porokeratotic keratoderma" "porokeratosis punctata palmaris et plantaris" refer probably to related conditions "keratosis punctate palmaris et plantaris". We prefer the term "punctate porokeratotic keratoderma".

To know the variation of DNA contents of seborrheic keratosis, keratoacanthoma, actinic keratosis, and Bowen's disease, cytophotometric assay was used. As the results, following findings were obtained. 1. DNA index of actinic keratosis was higher than those of seborrheic keratosis and keratoacanthoma. 2. DNA index of Bowen's disease was higher than that of seborrheic keratosis. 3. Polyploid cell population (greater than 6C) of keratoacanthoma was higher than that of seborrheic keratosis. 4. Polyploid cell populations (greater than GC) of carcinoma in site (actinic keratosis, Bowen's disease) were higher than that of keratoacanthoma . These date suggested that polyploid cell population and DNA index reflect grade of malignancy of tumor developed from keratinocytes. A clinic keratosis and Bowen's disease revealed almost the same DNA pattern. And the difference of polyploid cell population of keratoacanthoma++ ++ and seborrheic keratosis suggested the difference of the biological activity of them.