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A case of diffuse neonatal hemangiomatosis is described. At birth, a giant tumor, 10 cm in diameter and 6 cm in height, was present on the left axilla and associated with thrombocytopenia. Numerous cherry-red papules were present on the skin and buccal mucosa. On the second day of life, the giant tumor was excised. Histological examination of the tissue revealed masses of endothelial cells embedded in fibrous tissues. Plain and enhanced computed tomography of the brain revealed a hemorrhage and two hemangiomas. The associations of intravascular papillary endothelial hyperplasia were discussed.

The allotypes of C6, C7, factor B (BF) and factor I (IF) of the human complement system were studied in 11 Japanese patients with pemphigus (5 with pemphigus vulgaris and 6 with pemphigus foliaceus) and 17 with bullous pemphigoid (BP) to investigate the genetic background of these diseases. The allotypes were detected by using isoelectric focusing and immunoblotting. The frequency for IF* A allele in the pemphigus patients was significantly higher (p = 0.009) than that in healthy controls (n = 60). A significant association of IF A allotype with pemphigus was also observed (p = 0.027), with a relative risk of 6.3. There was no association between the C6, C7, BF or IF allotypes and BP. These data suggest that IF A allotype may be an etiological genetic factor in the development of pemphigus.

Decay-accelerating factor (DAF) is a 70-kD membrane glycoprotein that regulates autologous complement activation, by preventing assembly of alternative or classical C3/C5 convertases, and has been shown to have a wide tissue distribution. In this study, DAF antigen has been demonstrated at the intercellular spaces of normal human epidermis with monoclonal antibody against DAF using the peroxidase- anti-peroxidase method. The amount of DAF was greater at the granular layer than the basal cell layer as judged by intensity of the staining. Western blot analysis of DAF of the epidermis showed a 55-kD band, whereas that of buffy coat cells was approximately 67 kD. When DAF of the epidermis was treated with ncuraminidase, the molecular weight was reduced to 53 kD, whereas that of buffy coat cells was 56 kD. These results indicated that the content of sialic acid of DAF in the epidermis was different from that of buffy coat cells. In phosphatidylinositol-specific phospholipase C (PIPLC) – treated normal human skin, DAF was not demonstrated in the epidermis, whereas DAF remained unchanged on the elastic fibers. After the treatment of the epidermis by PIPLC, DAF was released into the buffer shown by Western blot analysis. These results suggested that DAF on the epidermis was anchored to keratinocyte via phosphatidylinositol (P1), whereas the anchoring mechanism of DAF on the elastic fibers was not through PI.

A 77-year-old man was seen with florid, vegetating nodules in the umbilical, axillary and inguinal areas, scaly erythema in the trunk and fragile nails. Histologically, marked to moderate acanthosis and papillomatosis with intraepidermal abscesses composed of acantholytic cells eosinophils and neutrophils were seen in the vegetating lesions which showed intercellular IgG deposit by the direct immunofluorescence technique. There were no circulating pemphigus antibodies. The lesions were successfully treated by topical corticosteroids. However, flaccid bullae with hypopyon showing subcorneal acantholysis and intercellular IgG deposits continued to develop. After 31/2 years, circulating pemphigus antibody of IgG2 subclass was demonstrated.

We have employed a successful therapy for epidermolysis bullosa with high-dose oral tocopherol acetate. Two siblings with dominant dystrophic epidermolysis bullosa of the Cockayne-Touraine type were reported. Both siblings suffered from tense blisters and erosions healing with scars and transient milia on the extremities. Electron microscopic study of the blisters revealed a cleavage beneath the lamina densa in both siblings. High-dose oral tocopherol acetate therapy was administered to them with satisfactory clinical reduction of the blister formation.

A 37-year-old male presented with a cauliflower-like tumor in the right buccal mucosa, leukoplakia of the tongue and esophagus, and keratotic papules or plaques on the extremities including bilateral palms and soles, associated with borderline diabetes mellitus and a repeated history of infections. A multi-system keratinizing abnormality was suspected, although no distinct disease category has been found showing similar involvements.

Membrane co-factor protein (MCP; CD46) is an integral membrane protein with molecular weight (MW) of the two species of 63 kD and 55 kD, and regulates autologous complement activation, with the activity of factor I cofactor. The quantity of each species is genetically regulated, and two codominantly inherited allelic variants account for the three phenotypic patterns. By immunohistochemical study, MCP was found both in the intercellular spaces of the epidermis and on the endothelial cells in the dermis of normal human skin in vivo. The intensity of the staining pattern was higher in the basal layer than in the granular layer. By Western blot analysis with use of a monoclonal antibody, MCP in the epidermis appeared as several bands ranged from 60-50 kD, with a major band of 56 kD, which was different from those in either polymorphonuclear cells, platelets, and cultured keratinocytes. No other variants were found in the epidermis obtained from skin of 20 normal humans. Complement activation in human skin may be regulated at several steps, including DAF and HRF20, thereby protecting cells from autologous complement attack.

Identical twin sisters developed generalized erythema and bullae on skin and mucous membranes at 18 and 19 years of age. Atrophic scars and milia were formed later. Indirect immunofluorescence (IF) study of the separated skin by incubation in 1.0M NaCl showed antibasement membrane zone (BMZ) antibodies bound to the dermal side. The HLA-DR typing demonstrated DR2/DW11. Pulse therapy resulted in marked clinical improvement. This is the first report of epidermolysis bullosa acquisita (EBA) in identical twins and suggests the possibility that the disease may have a genetic component.

A 38-year-old Japanese man was seen for severe palmoplantar keratoderma, extending to the dorsal aspects with red rims. He had had spontaneous amputations of the toes and surgical amputation of the right lower leg because of squamous cell carcinoma of the right sole. The clinical symptoms suggested those of mal de Meleda, except for the absence of consanguinity and of granular layers in the epidermis. The keratoderma improved with oral etretinate treatment.

To the Editor.— A 65-year-old Japanese woman was first seen in January 1979 because of a slowly enlarging, extensive, well-demarcated erythematous plaque on the right side of her face (Fig 1, panel A), which had started as a red papule 30 years before. No history of preceding trauma was offered. The general condition of this patient was not impaired. Her family history showed no apparent or chronic infections. Histologic examination of the lesion revealed an epithelioid cell granuloma with periodic acid-Schiff-positive fungal elements in the dermis. Based on the culture findings, a diagnosis of infection with Fusarium species was suggested, but could not be confirmed at the time. The patient was treated with oral griseofulvin and flucytosine with no favorable response. Topical 0.1% to 0.5% amphotericin-B injections were then tried weekly or monthly for 1 year, with partial regression of the plaque. The patient failed to appear for further

苔癬型薬疹の原因には従来, 砒素, 金製剤, 抗マラリア剤, 抗結核剤, サイアザイド系降圧利尿剤, スルフォニール·ウレア系糖尿病剤が知られていたが, 最近では欧米に比べ脳代謝改善剤, 末梢血管改善剤によるものがはるかに増加しており全報告数の66.7%を占めていた。教室で経験した苔癬型薬疹のうち, 定型的な扁平苔癬の組織像を呈する症例は37.5%であつた。苔癬型組織反応を呈する疾患は扁平苔癬, LE, graft vs host反応, 苔癬様日光角化症など多数あるが, 扁平苔癬では発症初期にランゲルハンス細胞の増加, Tリンパ球の表皮·真皮内浸潤, ついで基底層ケラチノサイトの破壊と再生が起ることからこれらの疾患の発生に遅延型アレルギーが関与していると考えられる。治療には, 原因薬剤の中止, 抗ヒスタミン剤, 副腎皮質ホルモン剤の外用のほかPUVA療法, エトレチネート, グリセオフルビンなどが有効である。

This experiment was carried out in order to investigate the time sequence of tissue fibrinolytic activity in human skin following UVB or UVA after topically applicated psoralen using Todd's technique of fibrinolytic autography. Tissue fibrinolytic activity increased in the upper dermis for one to six hours after exposure to psoralen and subseqent UVA, when erythema responses were absent. The return to a normal pattern of fibrinolysis occured in the upper dermis 12–18 hours after treatment with psoralen and UVA, and then psoralen-UVA-induced erythema responses began. These responses were maximal 96 hours after treatment with psoralen and UVA, although no fibrinolytic activity was found in the upper dermis. Little change was observed in the lower dermal activity throughout the course of these responses. After UVB irradiation, a transient increase in fibrinolytic activity occured, with a normal pattern of lysis returning in the lower dermis at 12–18 hours. In the lower dermis an increase was observed at one to 6 hours although the activity in the upper dermis had already decreased. UVB erythema responses appeared within this period, were maximal at 6–12 hours, and declined gradually after 12 hours concomitantly with the activity in the upper and lower dermis. The upper dermal activity disappeared at 18 hours, while the lower dermal activity was not found after 72 hours.

A slowly growing, subcutaneous mass in the left eyebrow region of a 54-year-old Japanese woman showed numerous small epithelial cell nests surrounded by a dense desmoplastic stroma. The epithelial cell nests showed partially keratinizing cystic structures containing a small amount of glycogen. Immunoperoxidase staining for carcinoembryonic antigen was positive only in the lumina or on the ductal lining surface of tumor cells; staining for S-100 protein was positive within almost all of the tumor nests. Birbeck granules indicating Langerhans cells were found within some of the cells.

妊娠10ヵ月前期に発症した25才初産婦例を経験し, 免疫学的検索を加えて報告した。(1)皮疹の形態は類天疱瘡様であつた。(2)免疫血清学的所見ではIgM抗体産生よりIgG抗体産生へのconversionがみられ, CH50は高値であつた。(3)免疫組織学的所見では皮疹部, 無疹部に免疫グロブリン (IgG, IgM, IgA) 補体成分C1qの沈着はなくβ1C/β1Aグロブリン (C3) の沈着が表皮真皮間基底膜部にみられた。また血清中にはHG因子を認めた。(4)治療はステロイドが有効であつた。

四肢に緊満性水疱をともなう腫脹, 浮腫とそう痒のある多形紅斑様皮疹を生じ, 水疱中の好酸球が目立ち, 真皮, 皮下組織にも好酸球が浸潤し, 末梢血, 骨髄中の好酸球の増多と補体価, 皮疹, 発熱の消長が並行した8才女児例を経験し, disseminated eosinophilic collagen diseaseと考えた。なおIgEは高く, ステロイド投与により, IgEの著明な減少につれて皮疹は改善した。

徳島大学皮膚科教室において経験したスポロトリコーシス25例につき概略と解析を試みた。25例中男子12例, 女子13例と男女差なく, 10才以下の小児7例, 40才以上17例と中高年令, 小児に多発している。外傷が誘因と思われる例は44%, 病型はリンパ管型40%, 限局型60%, 播種型, その他の例はみられず, 初発部位は顔面が52%, 上肢44%, 下肢4%, 躯幹, その他0%であつた。治療はヨードカリの内服, 温熱療法により著効をおさめた。

A Statistical survey of viral disease in Department of Dermatology, Tokushima University, for the last 14 years from 1964 to 1977 was reported. The proportion of viral disease to the outpatients was 4.40% and the ratio between male and female was 1 to 1.45. Viral disease were classified to herpes zoster (25.98%), verruca vulgaris (25.74%), verruca plana juvenilis (17.21%), herpes simplex (11.71%), molluscum contagiosum (9.59%), varicella (3.56%), condyloma accuminatum (1.78%), rubeolla (1.54%) and others (2.84%).

58才農夫の頭部熱傷瘢痕に, 受傷後56年して発症したケラトアカントーマの1例を報告した。われわれが集めえた本症の本邦報告は157例におよぶが, 熱傷瘢痕に生じた報告例は2例目であつた。記載の明らかな先行病変は色素性乾皮症, DLE, sunburn, 老人性色素斑, 熱傷, 紅皮症, 湿疹病変などであつた。

To know the pathophysiology of erythematodes, the serum complement level (CH50), its diurnal changes, the titers of nine components of the complement and kinin-like substance were measured. 1) Normal level of CH50 was 37.7±5.8 u. 2) CH50 in various skin diseases was within 29 to 70 unit. There was no specificity on CH50 level in DLE, but CH50 in SLE was under 30 u. in almost cases. 3) Diurnal variation of CH50 assayed every 4 hours was no significant differences between normal control and SLE. It was round 6 u. in both. 4) An acute decrease of CH50, C1, C4, C2, C3, C5, C6, IA and kinin-like substance was observed before the clinical severe condition.

A 32-year-old Japanese man was seen because of a crusted and oozing plaque on his scalp of 3 years' duration. Histologically, suprabasal clefts and bullae containing acantholytic cells as well as intercellular IgG and C3 deposits were found by the direct immunofluorescent technique. There were no circulating pemphigus antibodies. The lesion was controlled by the topical application of 0.05% clobetasol propionate ointment and remained localized for the following 2 years.

In our present study, impairment of tissue fibrinolytic activity was observed in the affected skin of patients with progressive systemic sclerosis (PSS) by fibrinolytic autography. We treated PSS with a fibrinolytic agent, urokinase, based on the idea that the impairment of tissue fibrinolytic activity is implicated in the development of PSS. Two patients with PSS have responded favorably well to administration of Urokinase.

A 61-year-old female with extensive subcutaneous epithelioid cell granuloma in the back and extremities had negative tuberculin reaction, positive anti-nuclear factor (ANF) and elevated serum angiotensin-converting enzyme (ACE) level while on oral clofibrate for one-and-a-half years because of hyperlipemia. She also suffered from ischemic heart disease with pericardial effusion and dependent edema. The skin surface temperatures over the lesions were about 2°C higher than those of the surrounding normal skin. The subcutaneous lesions cleared spontaneously within 4 months after clofibrate was discontinued. Tuberculin reaction became positive and serum ACE returned to the normal level.