Search

症例1: 57歳，男性。幼少時から日光曝露により露光部に紅斑，小水疱が出現するようになった。原因が不明のまま，皮疹が出現した時のみ近医で外用療法を受けていた。症例2: 7歳，男児。2，3歳頃から露光部に被刺激感とともに浮腫性紅斑が出没していた。2000年9月，長時間の日光曝露で同様の症状が出現し，湘南皮膚科を受診した。ポルフィリン症を疑われ，当科を紹介された。両症例とも尿中ポルフィリン体は認められなかったが，光溶血試験は陽性で，赤血球プロトポルフィリンの高値を認めた。臨床症状，病理組織所見および赤血球蛍光現象で蛍光赤血球も認められたことから，両症例を骨髄性プロトポルフィリン症と診断した。症例1ではGOT，GPT，γ-GTPの軽度上昇を認めた。症例2では肝機能に異常は認めなかったが，腹部超音波検査で胆石症を指摘された。2症例とも誘発テストでは皮疹の再現はできなかったので，本症の本邦報告例における誘発テストについても考察した。また，骨髄性プロトポルフィリン症に合併する肝障害や胆石症について文献的考察を加えて報告した。

We have developed a new technique of adjusting propagation delay time of Multi-Wire Boards (MWBs) . MWBs use polyimide insulated copper wires for signal connection. The wires will intersect each other. Therefore, in high-density wiring, improper variation in the delay time will be caused by intersections of wires, adjacent through holes, folded wires, and via holes in the middle of wiring. A new estimation method was proposed and the influence of these factors on the variation of the delay time was investigated. It was found that about 73% of the variation is caused by the intersection of wires. With this new technique, it is possible, after finishing the routing design, to control the length of the wiring, and adjust the propagation delay time so that it is equal to that calculated by a newly developed software. We confirmed that the variation time of the boards designed in this way decreases to one-third or less of that of the conventional MWBs.

In the treatment of skin diseases, antihistamines and antiallergic agents are among the most frequently used oral medications, and are available as a wide variety of products. We investigated what criteria dermatological specialists use to select antihistamines and antiallergic agents, as well as the use objectives and expected benefits. The present investigation was conducted in a total of 1448 patients, including 336 patients with urticaria, 944 patients with eczema/dermatitis, and 199 patients with atopic dermatitis, in 6 dermatological clinics and 1 university hospital. A Case Card to record the prescription motives and clinical evaluation was used, and the results were tabulated and analyzed. As a result, it was found that the expected result was obtained in more than 80% of cases prescribed for by dermatologists based on the prescription motives for individual cases.

Patients showing coexistent linear IgG and IgA deposition along the basement membrane zone on direct immunofluorescence have been described as either bullous pemphigoid, epidermolysis bullosa acquisita, linear IgA bullous dermatosis, or cicatricial pemphigoid, depending on the clinical features and laboratory findings. In the present report, we describe two cases showing atypical clinical features distinct from those of other known bullous diseases. No circulating antibodies were detected by indirect immunofluorescence of normal human skin. Indirect immunofluorescence of 1 M NaCl split skin revealed IgG and/or IgA antibodies reactive with the dermal side of the split. Immunoblotting of normal human epidermal and dermal extracts showed no apparent reactivity with known autoantigens. The results suggest that there may be a unique and distinct bullous disease with linear IgG and IgA deposition at the basement membrane zone.

Three cases of granuloma annulare which did not exhibit a self-limited course were treated with tranilast at the dose of 300 mg/daily. The treatment resulted in the resolution of skin lesions within three months of administration. Although spontaneous resolution is often observed in granuloma annulare, tranilast may provide an alternative therapy for the treatment of cases resistant to spontaneous healing.

Using complement immunofluorescence, the capability of complement fixation by bound antibodies in pemphigus lesional skin was compared with circulating intercellular antibodies of complement-fixing type in the same patient. Five lesional skin samples from 6 patients, containing bound IgG, gave positive binding of complement (C3) supplied from fresh guinea pig serum, while only one serum sample showed complement-fixing pemphigus antibodies in the circulation. These results support the view that complement-fixing pemphigus antibodies are rapidly localized to the skin and play a role on the production of acantholysis.

Complement-fixing intercellular antibodies were detected in 10 of 17 sera from untreated pemphigus patients. The role of complement in the organ culture system was investigated using these sera. Ten sera possessing complement-fixing intercellular antibodies showed IgG binding to the intercellular substance in the organ-cultured skin and acantholysis-like changes were observed in eight cases. C3 deposition was not seen in any case. However, after treatment of the sections of cultured skin with fresh normal human serum, complement fixation of the intercellular substance by bound IgG was revealed in all the ten cases. No significant differences in the grade of acantholysis-like changes between the complement-depleted system and the complement-supplied system were observed. Complement does not appear to be necessary in the acantholytic process in the in vitro organ culture system, even though we considered the presence of complement-fixing intercellular antibodies.

A new method for obtaining the self-diffusion coefficients of A in the AY–BY solid solution has been proposed. In this method, the tracer diffusion of A* and the interdiffusion between AY and BY are carried out simultaneously, and the self-diffusion coefficients are determined as a function of composition over the whole range of composition in a similar way as the Boltzmann-Matano analysis. Since this method employs a diffusion couple consisting of single crystals of AY and BY, one can avoid troubles and errors due to segregation and/or phase separation which are often encountered in experiments using solid solutions as specimens. In order to confirm the validity of the proposed method, Dcl* in the TlCl–TlBr system and DAg* and DNa* in the AgBr–NaBr system have been determined at 400 °C by this method and compared with the results obtained by the conventional method. A good agreement has been found between them.

Summary Skin biopsy was performed in five cases of pemphigus; direct immunofluorescence (IF) studies demonstrated intercellularly bound IgG and C3 in each case. Sections of lesional skin, containing in vivo bound IgG were, in two cases, capable of further binding C3in vitro from normal human serum, an effect most marked in relation to areas of acantholysis. This work provides additional evidence that the complement system is involved in the process of pemphigus acantholysis.

固定内の自己拡散定数 (D*) を求めるのによく用いられている順次切削法では, 実験的に得られたトレーサーの濃度分布は, 通常, ガウス曲線C=M/√πD*t exp(-x2/4D*t) に一致するとして解析されている. ガウス曲線は, 拡散方程式を初期条件にデルタ関数状のトレーサー分布を仮定して解いた解である. しかし, 試料表面に付着されるトレーサーを含む化合物の厚さは, 実際には有限であり, また, その化合物が試料と化学形を異にし, 表面付着層と試料中での拡散定数が異なっている場合もしばしばある. 従来はこうした事情を考慮して拡散方程式が解かれていないので, 本研究では付着層の厚さ (h) が有限値をとり, 付着層中での拡散定数 (DA) と試料中での拡散定数 (DB) が違うとして拡散の偏微分方程式を解き厳密解を得た. その結果, 一定のDB/DA値に対して, r値 (r=h/√DAt) がゼロから無限大に増加するにつれ, 濃度分布はガウス曲線から誤差補関数曲線へと変化していく挙動が定量的に明らかになった. 濃度分布が両曲線のいずれかに一致している場合には, その曲線から正確なD*の値を得るための数学的に厳密な解析法があるが, いずれにも一致しない中間的な分布の場合には, 正確なD*を求めることは困難である. したがって, 自己拡散定数を正確に求めるためには, 濃度分布が両曲線のいずれかに一致するように, あらかじめ実験条件を設定しておく必要がある. こうした実験諸条件の許容範囲を示すために, (r, DB/DA) 平面を濃度分布が1%以内の誤差で, ガウス曲線に一致する領域, 誤差補関数曲線に一致する領域, 両曲線に共に一致しない領域の3領域に分けて図示した. TlCl単結晶にNaClの形でClのラジオアイソトープ, 36Clを付着させ, 36Clの拡散を行なわせて得られる濃度分布を測定し, これらの議論を実験的に例証した.

In order to compare the observed interdiffusion coefficients with those calculated by theoretical formulae proposed for ionic crystals, self-diffusion coefficients of Tl, Cl, and Br in TlClTlBr solid solutions and interdiffusion coefficients of the TlClTlBr system were determined at 400°C over the whole composition range. It was found that D∗ Cl :D∗ Br :D∗ Tl ≒ 18:9:1 , and that the maximum difference between observed interdiffusion coefficients and those calculated by a formula originated by Lindstrom is 13%.

The redox reaction between thin wustite foil and CO2 or CO was studied by means of a microbalance in a temperature range 900–1075°C. It was found that the reaction at the gas-wustite interface is rate-controlling and that the reaction rate is expressed asV_ox=k_OP_CO2a_O^-m/3 and V_red=k_O’P_COa_O^n/3where aO is the oxygen activity of wustite, and m and n are 2.1–2.2 and 0.8–1.2, respectively. On the basis of the theory by Kobayashi and Wagner, the most predominant mechanism is concluded to beCO_2+2\ominus\ightleftarrowsCO+O^2-Actigation energies for kO and kO′ were 35+3 and 32+3 kcal/mol, respectively.

By in vitro complement immunofluorescence, 6 sera from pemphigus with intercellular antibodies were tested for their capability to fix C1q, C4, C3, and properdin. All 6 serum samples yielded positive reaction for C3 staining. Three serum samples gave positive staining for C1q, 5 serum samples for C4, and 3 serum samples for properdin, respectively. Substitution of C2 deficient serum as a complement source inhibited C3 and properdin staining but not positive C1q and C4 staining. These results are best explained by the concept that complement activation in vitro by intercellular antibodies occurs via the classical pathway followed by assembly of the C3 amplification mechanism.

Eight out of 12 serum samples from patients with bullous pemphigoid having basement membrane zone antibodies gave positive binding not only to the basement membrane zone but also to the basal cell membrane and/or cytoplasm as observed by complement immunofluorescence. Reaction of fluorescein labeled pemphigus vulgaris gamma-globulins, binding mainly to the lower intercellular spaces of the epidermis, was greatly reduced by the prior incubation of high-titered bullous pemphigoid sera having the reactivity to the basal cells, while that of fluorescein labeled pemphigus foliaceus gamma-globulins binding mainly to the upper and middle intercellular spaces, was not influenced by the prior application of these bullous pemphigoid sera. These results indicate that bullous pemphigoid antibodies are heterogenous and can be classified into 2 types and that some cross reaction is present between pemphigus antibodies and bullous pemphigoid antibodies having the reactivity to the basal cells.

• The serum of a patient with a typical case of pemphigus vulgaris contained complement-fixing intercellular autoantibodies (pemphigus antibodies). Complement-fixing pemphigus antibodies were demonstrated only during the untreated active stage of the disease and titrated lower than corresponding IgG autoantibodies, which paralleled well with the disease activity. Pemphigus lesional skin, which contained in-vivo-bound IgG, showed the capability of further binding C3 in vitro from normal human serum. It was suggested from these findings that the complement system may play an active role in pemphigus acantholysis through complement-fixing pemphigus antibodies. ( Arch Dermatol 114:1191-1192, 1978)

The capability of complement fixation of pemphigus antibodies was tested using combined in vitro complement immunofluorescent (IF) staining methods. Three sera out of 25 serum samples from 22 pemphigus patients revealed positive reactions, while all other sera gave negative results. Specificity control tests confirmed the positive reactions to be specific for complement staining. Complement fixing pemphigus antibodies were titrated lower than corresponding IgG antibodies and were demonstrable only in the extensive stage of the disease. Thus, the present work supplied evidence that pemphigus antibodies fix complement in vitro. However, the discrepancy still remains between the in vivo deposition of complement in most cases of pemphigus and in vitro capability of complement fixation in only few cases. More investigations should be needed to explain the exact role of complement in pemphigus acantholysis.

Summary A 79-year-old female developed a generalized pruritic annular erythematous eruption without bullae. Histologically there was eosinophilic spongiosis but no associated acantholysis. Using direct immunofluorescence, the deposition of IgG and C3 was detected in the intercellular space, but circulating anti-intercellular-antibodies were not demonstrated. We considered this case to have atypical pemphigus. The eruption responded well to oral administration of Diaminodiphenyl sulphone 75 mg daily.

A new method for obtaining the self-diffusion coefficients of A in the AY–BY solid solution has been proposed. In this method, the tracer diffusion of A* and the interdiffusion between AY and BY are carried out simultaneously, and the self-diffusion coefficients are determined as a function of composition over the whole range of composition in a similar way as the Boltzmann-Matano analysis. Since this method employs a diffusion couple consisting of single crystals of AY and BY, one can avoid troubles and errors due to segregation and/or phase separation which are often encountered in experiments using solid solutions as specimens. In order to confirm the validity of the proposed method, Dcl* in the TlCl–TlBr system and DAg* and DNa* in the AgBr–NaBr system have been determined at 400 °C by this method and compared with the results obtained by the conventional method. A good agreement has been found between them.

A positive correlation between complement-fixing pemphigoid antibody titre and disease activity was observed in 4 of 8 patients with bullous pemphigoid using complement immunofluorescence. The fact that this correlation cannot always be demonstrated seems to contradict the active role of complement-fixing pemphigoid antibodies in the pathogenesis of bulla formation. The similarity of bullous pemphigoid and herpes gestationis is discussed.

To the Editor.— According to the recent report by Kumar et al in the July 1980Archives(116:812-814), complementfixing (CF) intercellular (IC) antibodies in titers comparable to those seen by the ordinary indirect IgG test are a characteristic of pemphigus-like antibodies. Recently, 51 serum samples from untreated patients with pemphigus (clinically, histologically, and immunopathologically confirmed) were tested in our laboratory for the presence of CF IC antibodies. Six serum samples were found to have positive CF IC antibodies in titers greater than 1:20. In one case diagnosed as herpetiform pemphigus, the titer of CF IC antibodies (1:20) did not differ substantially from the titer of ordinary IgG antibodies (1:40). In addition, we could demonstrate IgG deposition in the IC spaces of organ-cultured human skin using pemphigus serum samples including CF IC antibodies; no C3 deposition was found. These bound IC antibodies were able to fix complement. We believe that the