Search

To measure X-ray spectra and to perform photon-counting computed tomography (PC-CT) with high count rates, we developed a zero-dark-counting spectrometer using a short-decay-time scintillator. A method exploiting a YAP(Ce) [cerium-doped yttrium aluminum perovskite] single crystal scintillator with a decay time of 30 ns and an MPPC (multipixel photon counter) has been developed to count X-ray photons. The photocurrent from the MPPC was amplified by a high-speed current–voltage amplifier, and the event pulse was sent to a multichannel analyzer (MCA) to measure X-ray spectra. The MPPC was driven under pre-Geiger mode at a bias voltage of the MPPC of 70.7 V and a temperature of 23 °C. The PC-CT was accomplished by repeated linear scans and rotations of an object, and projection curves of the object were obtained by the linear scan at a tube current of 1.0 mA. The exposure time for obtaining a tomogram was 10 min at a scan step of 0.5 mm and a rotation step of 1.0°. At a tube voltage of 100 kV, the maximum count rate was 200 kcps. In the PC-CT using gadolinium media, we observed image-contrast variations with changes in lower-level discrimination voltage of the event pulse using a comparator.

The aim of this retrospective study was to identify the risk factors associated with the severity characteristics in the Tokyo guidelines for conversion to open surgery in patients with acute cholecystitis (AC) who underwent laparoscopic cholecystectomy.A total of 225 patients were enrolled in the study. The patients were classified into two groups: a conversion group and a no-conversion group. The preoperative characteristics and therapeutic strategy were analyzed as risk factors for conversion to open surgery. The postoperative outcomes were also analyzed.Conversion to open surgery occurred in 29 patients (12.9%), including seven patients (6.7%) with mild AC and 22 patients (18.5%) with moderate AC. A univariate analysis showed that the risk factors for conversion to open surgery included a duration of symptoms longer than 72 h, an elevated C-reactive protein (CRP) value and the Tokyo guidelines 2013 (TG 13) severity classification. The multivariate analysis showed that the risk factors for conversion to open surgery included a duration of symptoms longer than 72 h and a CRP value11.5 mg/dl.A duration of symptoms longer than 72 h, which is included in the criterion for moderate AC severity in the TG 13, was an independent risk factor for conversion to open surgery. In addition, adoption of a high CRP value as an additional criterion for moderate AC may increase the utility of the TG 13.

Merkel cell polyomavirus infects up to 80% of patients with Merkel cell carcinoma. Combined Merkel cell carcinoma and cutaneous tumors occur occasionally. Previous reports have suggested that Merkel cell polyomavirus is absent from combined Merkel cell carcinoma and squamous cell carcinomas. This is the first report that Merkel cell polyomavirus infected in both lesions of a combined Merkel cell carcinoma and basal cell carcinoma. A 92-year-old Japanese man presented with a right thigh small subcutaneous mass. Histologic examination revealed a combined tumor with Merkel cell carcinoma and basal cell carcinoma with ductal differentiation. Both tumors and intermingled Merkel cells in basal cell carcinoma expressed Merkel cell polyomavirus large T antigen, and 17 and 240 copies of Merkel cell polyomavirus/cell were detected in the microdissected Merkel cell carcinoma and basal cell carcinoma specimens, respectively. Mutation analysis of Merkel cell polyomavirus large T antigen revealed a novel truncating mutation in Merkel cell carcinoma and a similar but different mutation in the basal cell carcinoma. These results suggest that each was infected by a different Merkel cell polyomavirus subclone derived from a single Merkel cell polyomavirus.

This report presents the case of a common hepatic artery (CHA) pseudoaneurysm secondary to postoperative pancreatic fistula (POPF) after pancreatoduodenectomy (PD), which was successfully treated using a coronary covered stent. A 70-year-old female underwent subtotal stomach-preserving PD for middle cholangiocarcinoma. POPF was identified on postoperative day (POD) 7, and the patient suddenly lost 500 ml of blood via the abdominal drain on POD 19. Urgent celiac arteriography revealed a CHA pseudoaneurysm. A coronary covered stent was placed to prevent rupture of the pseudoaneurysm and to maintain hepatic arterial flow, instead of performing transarterial embolization. No vascular adverse events were encountered during or after the procedure. Computed tomography and angiography showed a patent stent graft and good hepatic arterial flow 9 months after placement of the stent. Endovascular stent-graft placement not only treated the pseudoaneurysm, but also preserved the arterial blood flow. This report describes the placement of a covered stent graft for delayed hemorrhage after PD.

One of identical twin girls was born with ulcers on her leg, and shortly after birth developed a flaccid blister on the leg. Subepidermal blister with vacuolar degeneration of basal cell layer and the heavy infiltration of mononuclear cells in the upper dermis were observed in the blister lesion. She also had generalized livedo. Her identical twin sister did not exhibit ulcers or blisters, but was born with milia on her limbs. Their mother was found to have lupus erythematosus with positive anti-Ro/SSA antibodies and developed Sjogren syndrome. We emphasize neonatal blistering and congenital milia unique manifestations of neonatal lupus erythematosus.

Drug reaction can be one of the triggering factors either for exacerbation of pre-existing psoriasis or precipitation of psoriasis de novo. Herein, we report a case with psoriasiform drug eruption due to pravastatin, but it relapsed after discontinuance of the drug. Serial investigation of the histopathology and immunohistochemistry with anti-signal transducer and activator of transcription 3 (STAT3) revealed drug reaction-associated lesions intermingled with psoriasiform changes, which later predominated, suggesting a conversion into psoriasis de novo.

49歳, 男性。体幹・四肢に自覚症状のない紅斑, 丘疹が生じ増加してきた。病理組織にてCD4陽性を示す異型細胞の浸潤, TCR遺伝子再構成を認め, 皮膚T細胞リンパ腫 (CTCL) と診断した。ステロイドパルス療法後, ステロイドと低用量MTX内服投与で治療していたが, 皮疹が再燃した。病理組織にて大型の異型リンパ球の密な浸潤を認め, CD4陽性を示す異型T細胞とCD20, CD79a陽性を示す異型B細胞が混在していた。免疫グロブリンH鎖に遺伝子再構成を認めた。EBERs陽性細胞を少数認め, 組織学的にEBV感染が証明できた。以上よりCTCL患者にメトトレキセート関連リンパ増殖性疾患 (MTX-LPD) が発症したと考えた。MTX中止のみでは軽快せず, フルダラビンによる化学療法を施行した。MTX-LPDについて原疾患, Epstein-Barr Virusとの関連など過去の報告例と比較検討した。MTX-LPDは重篤な医原性疾患の一つであると考え報告する。

症例は71歳，男性。当科初診の7ヵ月前に右耳朶後面に増殖性局面が出現し，3ヵ月後に自壊した。摘出したところ，2週間後に創は潰瘍化した。外科的処置を繰り返すたびに潰瘍が拡大し，耳朶が下垂した状態になった。増殖性局面の病理組織所見はpseudoepitheliomatous hyperplasiaの像を示し，真皮に好中球，単核球および形質細胞が浸潤していた。増殖性膿皮症と診断し，プレドニゾロン40mg/日を内服投与したところ，潰瘍は上皮化したが，漸減後中止した3日後に発熱とともに潰瘍が再出現した。シクロスポリン3mg/kg/日の内服投与により再び潰瘍の上皮化はすすみ，現在漸減中である。

ステロイドと低用量メソトレキセート (以下MTXと略す) の併用療法が奏功した未分化型大細胞リンパ腫 (Anaplastic large cell lymphoma; 以下ALCLと略す) の1例を経験したので報告する。症例は60歳, 女性。初診の4年前に下肢に出現した皮疹をALCLの診断のもと, 近医および当科でステロイド外用・内服, PUVA療法, IFN-γ注射, エトレチナート内服, 電子線照射などで治療されていたが軽快再発を繰り返していた。平成16年の再発時にCHOP療法施行後に本治療を開始したところ, CHOP療法後に残存していた病変はすべて速やかに消退した。以後38ヵ月間再発はみられない。本治療はCHOPなどの多剤併用化学療法施行前の, あるいは施行後の補助・維持療法として推奨される治療法であると考えられる。

76歳, 女性。外陰部から大腿に境界明瞭な紅斑と黒褐色斑と脱色素斑の混在を認め, 紅斑上に紅色結節を認めた。結節部の病理組織所見はPaget細胞が真皮全層に浸潤していた。右鼠径リンパ節を触知し, CTでは右鼠径と右外腸骨動脈領域のリンパ節腫大を認めた。CEAは正常範囲内であった。病変境界部から2cm離して拡大切除し, 両側浅深鼠径リンパ節と右外腸骨動脈領域リンパ節郭清術を施行した。右鼠径, および右外腸骨動脈領域リンパ節にPaget細胞の浸潤を認め, Stage III(T3N1M0)と診断した。手術5ヵ月後に腹部大動脈分岐部にリンパ節転移を認めた。ドセタキセル(タキソテール®)療法を開始したところ, 3ヵ月半後(9回投与後)には画像診断上complete responseを得た。治療開始13ヵ月後の現在, 再発および転移を認めていない。

Background: The histological resemblance between extramammary Paget disease and Bowen disease has been described since Bowen’s original article was published in 1912. Methods: We herein describe a case of vulval primary extramammary Paget disease in a 61-year-old women with the histological features of Bowen disease. Results: Histological examination of a biopsy specimen showed acanthosis with full-thickness cellular atypia, focal hyperkeratosis and parakeratosis in the epidermis, and no characteristic Paget cells were observed. However, histological examination of an operative specimen revealed areas characteristic of Paget disease and Bowen disease. Overall, the areas characteristic of Bowen disease and Paget disease occupied 6% and 32% of the total operative specimen, respectively. The two areas were sharply separated. Immunohistochemical findings showed carcinoembryonic antigen to be expressed in areas containing Paget cells, but not in the areas characteristic of Bowen disease. Cytokeratin 7 (CK7) (OV-TL 12/30) and CK8 (35βH11) were strongly expressed in both of these areas. The staining for high-molecular-weight cytokeratins was negative in both of these areas. Conclusions: Our findings indicated that primary extramammary Paget disease and squamous cell carcinoma in situ arose multifocally from a common cell in the epidermis.

Oxidized LDL has been obtained by incubation with copper ions (Cu-LDL) or various kinds of cells. LDL incubated with xanthoma tissues (x-LDL) is considered a model of in vivo oxidized LDL that has extravasated into xanthoma lesions. To investigate the mechanism of x-LDL formation, we studied the effects of various enzyme inhibitors or antioxidants on the oxidation process of LDL. Thiobarbituric acid-reactive substance (TBARS) levels, electrophoretic mobility and spectrophotometric pattern of the oxidized LDL were examined. Antioxidants suppressed TBARS formation in both x-LDL and Cu-LDL. Enzyme inhibitors inhibited TBARS levels in x-LDL, but not in Cu-LDL. All the enzyme inhibitors and antioxidants, except for the cyclooxygenase inhibitor, inhibited the anodic electrophoretic mobility of x-LDL. The anodic electrophoretic mobility of Cu-LDL was suppressed only with antioxidants. Spectrophotometry indicated that an increase in the absorbance at 240 nm was observed in Cu-LDL, but not in x-LDL. x-LDL oxidation is primarily catalyzed by phospholipase A 2 , and subsequently generated polyunsaturated free fatty acids propagate the peroxidation. Fatty acid hydroperoxides conjugated with dienes are not synthesized in x-LDL. On the other hand, non-enzymatic oxidants, such as superoxide anion and hydroxyl radicals generate Cu-LDL with diene-conjugated fatty acid hydroperoxides.

Background Activated mast cells enhance the uptake of mast cell–derived proteoglycan–low-density lipoprotein complexes by macrophages. Objective We sought to investigate mast cell contribution to the pathogenesis of xanthoma. Methods Twenty cases of xanthelasma palpebrarum and 6 cases of tuberous xanthoma lesions were analyzed using immunohistochemical staining. Results Xanthelasma lesions contained up to 5-fold more tryptase-stained mast cells than tuberous xanthoma lesions. Tuberous xanthoma lesions especially showed extensive staining of tryptase around mast cells and within some macrophages and foam cells. More than 99% of mast cells in xanthelasma lesions contained both tryptase and chymase. Approximately 60% of mast cells represented only tryptase in tuberous xanthoma lesions where the ratio of macrophages to tryptase-stained mast cells was extremely high (15:1) as compared with xanthelasma lesions (2:1). Limitations A change in mast cell phenotype has not been necessarily proven. Conclusion Mast cells are activated under the microenvironment in which macrophages predominate rather than mast cells, which thus reflects the clinical phenotypes of xanthoma lesions.

7例の落葉状天疱瘡，1例の尋常性天疱瘡に対して血漿交換療法とステロイドパルス療法の併用療法を施行した。有用であった7例では臨床症状の改善，抗体価の減少，ステロイド投与量の減量が可能であった。併用療法を早期より積極的に施行することで臨床症状を速やかに改善させ，ステロイド剤投与量の減量が容易となり寛解維持にも好影響が得られた。併用療法は，血漿交換療法による直接的な病因抗体の除去に加え，ステロイドパルス療法によりさらに臨床症状を効果的に改善したと考えた。この併用療法は天疱瘡に対する優れた導入初期治療であり長期的予後にも好結果を与えると結論した。

Summary Background The source of accumulated lipids in the foam cells of xanthoma is primarily the lipoproteins existing in the lesional dermis. Objective This study was designated to clarify the contribution of low density lipoprotein (LDL) density substances existing in xanthoma tissue to foam cell formation. Methods An LDL density fraction was obtained from homogenized rabbit experimental xanthoma tissue. Biochemical and functional characteristics of xanthoma-extracted LDL density substance were examined. The in vivo foam cell-inducing ability of xanthoma-extracted LDL density substance was examined microscopically at the intradermal injection site. Results Xanthoma-extracted LDL density substance showed more negatively charged mobility on agarose gel electrophoresis than plasma native LDL. A small amount of aggregated material remained at the origin on agarose gel electrophoresis. Xanthoma-extracted LDL density substance contained much higher level of lipid peroxides than native LDL. Mouse peritoneal macrophages internalized xanthoma-extracted LDL density substance extensively and transformed into foam cells by incubation with xanthoma-extracted LDL density substance. Intradermal injection of the xanthoma-extracted LDL density substance induced foam cell infiltration in the skin of a normolipemic rabbit. Conclusion LDL density substances prepared ex vivo from experimental xanthoma tissue contained lipid–protein complexes that have physiochemical properties of oxidized LDL. The lipid–protein complexes were incorporated into foam cells. The substances were considered to contribute to foam cell recruitment during the persistence of xanthoma lesions.

Summary Background Xanthoma is a foam cell infiltrating lesion similar to atherosclerosis. Glycosaminoglycans and proteoglycans have long been considered to play a role in atherogenesis. Objective The purpose of this study is to investigate the role of hyaluronan, the main dermal glycosaminoglycan, in xanthoma formation. Methods The complex formation of low density lipoprotein (LDL) with hyaluronan was investigated by assaying the cholesterol level of precipitates that were formed by incubating LDL, hyaluronan and cetylpyridinium chloride in the presence of Ca 2+ . The uptake of LDL by mouse peritoneal macrophages was studied by assaying the cellular cholesterol esterification activity. The responsible receptor for the LDL internalization was examined by saturating hyaluronan receptor and blocking class A macrophage scavenger receptor (CD204). Hyaluronan was injected into the dorsal skin of diet-induced hypercholesterolemic rabbits to reveal the xanthoma inducing activity of hyaluronan. Results Cetylpyridinium chloride precipitated hyaluronan, which had formed complexes with LDL. The macrophages incorporated hyaluronan–LDL complexes and oxidized LDL via CD204. Foam cell infiltration and cholesterol accumulation were induced by intradermal injections of hyaluronan in diet-induced hypercholesterolemic rabbits. Conclusion Hyaluronan, like other sulfated glycosaminoglycans, retains LDL by forming a complex. Via macrophage scavenger receptors, macrophages incorporate not only LDL–hyaluronan complexes, but also oxidized LDL, which has been oxidized during the retention time.

We report the case of an 85-year-old man who presented with an enlarging tumor on the right temple. Histologically, spindle-shaped cells were proliferating continuously within well-differentiated squamous cell carcinoma that was immunohistochemically negative for vimentin and alpha-smooth muscle actin (ASMA) but positive for cytokeratin (AE1/3). These spindle-shaped cells expressed vimentin and ASMA but not cytokeratin (AE1/3). Intermediate cells with round nuclei and small amounts of cytoplasm displayed slight expression of vimentin, ASMA, and cytokeratin. This case illustrated spindle cell squamous cell carcinoma with mesenchymal metaplasia of malignant epithelial cells.

A neuronal system dedicated to itch consists of primary afferent and spinothalamic projection neurons. Histamine is thought to be one of the main mediators for the transmission of itch sensation. However, there are little available information on the role of histamine in scratching behaviour and sensory transmission of atopic dermatitis and chronic eczema. In the present study, the role of histamine in scratching behaviour and neural conduction of sensation in the chronic eczema model was investigated by using l-histidine decarboxylase (HDC) gene knockout mice lacking histamine. The chronic contact dermatitis was induced with daily application of diphenylcyclopropenone (DCP) on a hind paw of HDC (+/+) and HDC (-/-) mice for 2 months. The observation of scratching behaviour and the hot-plate test were performed in both mice. Histological studies were performed in the skin and spinal cord tissues. Histological examination revealed that both HDC (+/+) and HDC (-/-) mice displayed the similar extent of inflammatory cell infiltration, hyperplastic epidermis and newly spreading of neuronal processes in the skin tissue. Scratching behaviour was exclusively induced in HDC (+/+) mice, whereas it was barely observed in HDC (-/-) mice. The expression of c-Fos was specifically upregulated in HDC (+/+) mice in lamina I of the spinal dorsal horn following repeated DCP application. Scratching behaviour in chronic contact dermatitis in mice was thought mainly mediated with histamine. The afferent pathway of sensation in chronic contact dermatitis model may connect with the central nervous system through lamina I of the spinal dorsal horn.

著明な色素沈着を伴う疣状扁平苔癬を生じた58歳の男性例を報告する。初診の2年前より血液透析を受けており，7ヵ月前より数種類の薬剤を内服していた。5ヵ月前より背部を主体に全身性，左右対称性に紫紅色丘疹が多発し，次第に黒色丘疹と疣状の灰白色小結節および局面が擦過部位に目立つようになった。病理組織像は扁平苔癬に合致し，黒色丘疹部では表皮内メラニンの増加とメラノファージ浸潤が著しく，灰白色局面では表皮肥厚が顕著で，表皮内メラニンは乏しく，メラノファージも少なかった。薬剤誘発性と考え，血液透析は継続しつつ，内服薬を中止または他剤に変更し，プロピオン酸クロベタゾール軟膏の外用を行うことにより皮疹は消退し，再発しなかった。パッチテストおよび内服誘発試験では原因薬剤は特定できなかった。著明な色素沈着は，血液透析で除去されず体内に高濃度に蓄積されたβ-lipotropinがメラニン産生を著しく増強させた結果と考えた。

A 15-year-old boy first noticed multiple firm papules on his right upper chest two years before presenting to our clinic. These papules were densely distributed and showed epidermal nevus-like linear arrangement at some sites. The number, size, and distribution of these papules remained unchanged for one year of our observation. The papules flattened leaving scars and did not recur within a year after one shaving abrasion using a scalpel. Histopathologically, epidermis of the papule displayed acanthosis with elongated rete ridges. Accumulation of mucin was apparent in the papillary and subpapillary dermis. In mucinous nevus, the origin of cells with nevoid proliferation is obscure. In contrast with common collagenous connective tissue nevus, it is hard to define the localized persistent mucin accumulation as a nevoid manifestation. The present case of mucinous nevus might be caused by significantly stimulated glycosaminoglycan synthesis in a kind of epidermal nevus without extreme hyperkeratosis.

53歳，女性。下顎では色素沈着を残し，下腿では色素沈着を残さない固定薬疹が繰り返し出現した。パッチテスト(皮疹部および無疹部)およびスクラッチパッチテスト(無疹部)は陰性。ミノサイクリン5mg投与による内服誘発テストでは，下顎と下腿に皮疹が再燃し，前額にも紅斑が新生した。病理組織像では下腿に比べ下顎の真皮にメラノファージが著明に多く認められた。免疫組織化学では，トリプターゼ陽性肥満細胞が下腿と比較して下顎の特に真皮乳頭層において有意に多く認められた。肥満細胞由来のケミカルメディエーターやサイトカインが，メラノサイトのメラニン合成やメラニンのケラチノサイトへの輸送を促進することも色素沈着を増強させる因子であると考えた。

53歳, 女性。D-ペニシラミンの内服開始1年4カ月後, 肉眼的には落葉状天疱瘡, 病理組織学的には尋常性天疱瘡の所見を示す皮疹を生じた。免疫ブロット法で, デスモグレイン (Dsg)-1抗体が証明されたが, Dsg-3抗体は認められなかった。ELISA法では, Dsg-1およびDsg-3抗体ともに陽性だった。内服中止後も水疱の新生は続いた。プレドニゾロンの内服減量中に水疱が再燃した際には, Dsg-3抗体は陰性化していたがDsg-1抗体は陽性であったことから, Dsg-3抗体は薬剤によって修飾されたDsg-3に対する抗体であり, Dsg-1抗体は持続的に産生されていると考えた。抗核抗体と抗細胞質抗体が陽性であったことから, 自己抗体を産生しやすい素因があると考えた。水疱蓋および水疱内の多くの棘融解細胞が変性していたことも特徴であった。

20歳，インドネシア人男性。2001年1月に来日し，同年4月より船上研修を受けていたところ，8月頃より顔面に皮疹が出現した。同年12月初診時，顔面に両側対称性に淡紅色から紅褐色の一部境界不明瞭な斑·局面·丘疹·結節が多発していた。両側大耳介神経の肥厚を認めた。知覚·発汗の異常はなかった。結節部皮膚スメア検査でbacterial index 4+のZiehl-Neelsen染色陽性桿菌を認めた。鼻汁スメア検査でも菌陽性であった。生検皮膚HE標本では，菲薄化した表皮直下に薄いclear zoneを残し，真皮全層から皮下組織にかけて，泡沫状や空胞状を呈する組織球よりなる肉芽腫を認めた。リンパ球の浸潤は乏しく，類上皮細胞や巨細胞は認めなかった。Ziehl-Neelsen染色陽性桿菌は，真皮の組織球内に多数認めるとともに，毛包上皮内および汗腺上皮内にも存在した。Dharmendra抗原反応は陰性であった。LL型ハンセン病と診断し，ジアフェニルスルフォン，リファンピシン，クロファジミンによる多剤併用療法を開始したが，約1ヵ月後，行方不明になった。残存するハンセン病に対する偏見や無理解，言葉の障壁による信頼関係構築の困難，外国人患者に対する医療体制の不備など，在日外国人患者治療の問題点についても言及した。

Human low density lipoprotein (LDL) was incubated with rabbit xanthoma tissue or non-lesional dermis. The xanthoma tissue-modified LDL (x-LDL) was oxidized showing a 12-fold higher level of thiobarbituric acid-reactive substances (TBARSs) and a faster anodic electrophoretic mobility than native LDL (n-LDL). The LDL treated with non-lesional dermis (d-LDL) had a twofold higher TBARS level compared with n-LDL, but the electrophoretic mobility of d-LDL and n-LDL was similar. Cholesterol esterifying activity in mouse peritoneal macrophages, an indicator of LDL uptake, was up-regulated 5-fold and 1.8-fold by incubation with x-LDL and d-LDL, respectively, compared with n-LDL. Macrophages transformed into foam cells in incubation with x-LDL, and intradermal injections of x-LDL induced infiltration of great many foam cells in the normolipemic rabbit dermis. d-LDL had much less effects on the foam cell formation and foam cell infiltration than x-LDL. Cholesterol:protein ratio was higher in x-LDL than in n-LDL and d-LDL, suggesting that x-LDL-induced foam cells accumulated the lipids by incorporating the cholesterol-rich x-LDL. In conclusion, extravasated LDL receives oxidation and contributes to foam cell recruitment in xanthoma lesions. On the other hand, extravasated LDL in non-lesional dermis receives limited oxidation and additional promoting factors are necessary for initiation of xanthoma development.

Repeated ultraviolet (UV) irradiations have been shown to induce keratinocyte proliferation with acanthosis, stimulate the cutaneous nerve proliferation, and increase the synthesis of calcitonin gene-related peptide (CGRP). In the current study, we examined the role of CGRP in the UVB-induced proliferation of murine keratinocytes. UVB irradiation increased the number of bromodeoxyuridine (BrdU)-labeled basal keratinocytes and caused acanthosis. In addition, CGRP expression was up-regulated in the peripheral nerves of the upper dermis and lower epidermis. Repeated intradermal injections of CGRP increased the number of BrdU-labeled basal cells and caused acanthosis. Intradermal injections of capsaicin prior to UVB-irradiation inhibited the UVB-induced CGRP expression, BrdU labeling in basal keratinocytes and epidermal thickening. Intradermal injections of anti-CGRP antibody inhibited the UVB-induced BrdU labeling in basal keratinocytes, but epidermal thickening was not significantly inhibited. These results indicate that CGRP is one of the stimulators to UVB-induced keratinocyte proliferation. On the other hand, expression of substance P, another neuropeptide in the peripheral nerve, was not up-regulated by UVB irradiation.

65歳，女性。高齢発症の多彩な臨床像を示した原発性Sjogren症候群の1例を報告した。顔面と手掌の滲出性紅斑の臨床所見からはSjogren症候群とSLEの区別は困難であった。背部の掻破痕を伴う浮腫性紅斑および筋生検所見からは皮膚筋炎を疑った。各症候はプレドニゾロン30mg/日の内服治療で速やかに消退し，5mg/日の投与で寛解を維持しており，原発性Sjogren症候群と診断した。傾眠，見当識障害を示したが，これも原発性Sjogren症候群の症状と判断した。原発性Sjogren症候群においても，重複症候とは限らず，SLEや皮膚筋炎の症候を示しうることを報告した。

We studied the pathogenetic role of iminodipeptides, and the effects of corticosteroids on the skin lesions of two adult female siblings with prolidase deficiency. The elder sister had had severe skin ulcers and mental retardation since childhood, while the younger sister had shown milder clinical manifestations since late adolescence. The ulcers showed vascular wall thickening and neutrophil infiltration. Oral prednisolone at moderate doses was not effective, but corticosteroid pulse therapy followed by a moderate dose of prednisolone improved the preulcerative indurated lesions and ulcers. A 2-year follow-up of the younger patient indicated that N-formyl methionyl leucyl phenylalanine-induced neutrophil superoxide generation was elevated, in parallel with an increase in the serum iminodipeptide level, when the skin ulcers and preulcerative indurated lesions were most active. Corticosteroid pulse therapy downregulated the superoxide generation by neutrophils. The serum iminodipeptide level, however, did not decrease during 25 days after pulse therapy. These findings suggest that iminodipeptides may play an important part in aggravating the skin lesions by priming neutrophil superoxide generation, and that high-dose corticosteroids improve the skin lesions, probably by inhibiting the infiltration, and superoxide generation by, neutrophils. Neutrophil superoxide generation was more prominent in the elder sister, suggesting that clinical severity may depend on the response of neutrophils to the iminodipeptides. Chronic stimulation by superoxide may cause thickening of cerebral blood vessels and eventual mental retardation.

A 53-year-old female developed erythema elevatum diutinum (EED) twelve years after the onset of rheumatoid arthritis. The arthritis had been well controlled for the last several years. Annular purpuric macules were characteristically complicated by common nodular and plaque lesions. Both leukocytoclastic vasculitis and fibrosis were observed in the macular lesions, indicating that the lesions were a manifestation of an early phase of EED. Both types of skin lesions disappeared with treatment with dapsone. They have not relapsed for two years after stopping the dapsone. The leukocytoclastic vasculitis was thought to have developed independently of the rheumatoid arthritis. She had noticed sicca symptoms two years before the appearance of EED, but she did not satisfy the diagnostic criteria for Sjögren's syndrome.

38歳, 男性。深部静脈血栓症による下腿潰瘍を呈した。抗カルジオリピンβ2グリコプロテインI抗体, ループスアンチコアグラントおよび抗核抗体陽性で, 原発性抗リン脂質抗体症候群と考えた。通常の治療では難治であった潰瘍は, ワーファリン®とブコローム内服により短期間で治癒した。ワーファリン®単独投与では効果が不十分な場合には, トロンボテストを指標にブコロームを併用することが重要である。潰瘍再発の4ヵ月前に小腸イレウスを発症し, Degos病類似の皮疹が出現したが, 腸穿孔はなく, 皮疹の病理組織像には特徴的な所見は認められなかったことから, 抗リン脂質抗体症候群に伴うDegos病様症候と考えた。

H2レセプター拮抗剤である塩酸ロキサチジンアセタート(アルタット®)による著明な鱗屑を伴う剥脱性皮膚炎型の薬疹を報告した。病理組織学的にsatellite cell necrosisを認め, 表皮内に浸潤しているリンパ球はヘルパーT細胞であり, Langerhans細胞は消失していた。異常角化細胞は積極的に表皮外に排出されていた。スクラッチパッチテストおよび内服誘発試験で原因薬剤を特定できた。シクロスポリンが著効を呈した。

Generalized eruptions associated with radiotherapy such as erythema multiforme (EM), Steven-Johnson syndrome and toxic epidermal necrolysis are uncommon reactions. A few cases of generalized eruptions during and after radiotherapy have been reported with the use of anticonvulsants and anticancer drugs. However, no reports have described mucocutaneous reactions associated with radiotherapy and concurrent use of anastrozole, an aromatase inhibitor. This report describes EM occurring after radiotherapy performed during breast-conserving treatment for breast cancer in a patient who was taking oral anastrozole.

A 52-year-old man with squamous cell carcinoma (SCC) complicated with pseudoepitheliomatous hyperplasia on the tongue is reported. Six times biopsies obtained from the tumors of the dorsal aspect of the tongue revealed no malignant changes in the hypertrophic epidermis. Histological findings of lichen planus were observed on a part of the tumors. Dental crowns containing patch test-positive mercury were irritating the ulcerating portion of the tumors. Serum SCC antigen level was elevated. The tumors regressed by administration of etretinete, but serum SCC antigen level was unchanged. After stopping the administration, the tumors proliferated again. Histological findings of SCC were observed in a biopsy sample obtained from the floor of the tongue. Serum SCC antigen was decreased after the tumors were resected. Lichen planus or irritations by dental crowns might have caused the SCC. Tongue carcinoma preferentially appears on the side and floor of the tongue. We have to be nervous to the biopsy site of tongue tumors.

For the purpose of treating intractable decubitus and skin ulcer, streptokinase (SK) jellies containing different types of thickening agents were prepared. Adhesion to the affected sites, the spreading ability of the jelly preparation, and the properties of the SK in the jelly, which are all considered to greatly affect the clinical efficacy, were examined pharmaceutically using the viscosity, spreading area, and release and stability of SK as indexes. Regarding the viscosity and spreading area which are thought to affect both adhesion and spreading greatly, when sodium polyacrylate (PANa) was used as a thickener, higher viscosity and smaller spreading area were obtained in comparison with carboxymethylcellulose sodium (CMCNa) and sodium alginate (Alg Na). In addition, the viscosity and the spreading area obtained with PANa were also slightly more affected by temperature than with CMCNa and AlgNa. Regarding the release of SK from the jelly preparations, with PANa no initial burst was observed and SK was continuously released at a constant rate in contrast with CMCNa and AlgNa, thus obviously showing the superiority of PANa in the retention of SK. Although no loss of the potency of SK was found in the jellies containing PANa, CMCNa or AlgNa at 5°C, for 30 days, the remaining potency was greatly decreased to 49-62% at 25°C after 30 days and 33-42% at 37°C after 30 days. These results showed that the adhesion, spreading ability and SK-release were greatly different among the tested thickening agents, and the properties of PANa were found to be clearly superior to those of the other agents. According to these results, SK jelly containing 2% PANa was used for the treatment of intractable decubitus and skin ulcer, and an excellent therapeutic effect was obtained.

A patient with extramammary Paget's disease showed perianal nodules surrounded by erythema. rIFN-α-2a (300 million IU) was intralesionally injected three times a week. After the administration of 17, 700 million IU IFN-α, the skin lesions regressed leaving infiltrating plaques. Histologically, Paget cells were not observed at the site of erythema, but survived in the thickened epidermis. The intradermally infiltrating Paget cells remained. Then, the infiltrating lesions were excised under local anesthesia. Paget cells, however, were observed in the epidermis and in the dermis adjacent to the surgical suture. Paget cells completely disappeared and remission has been lasting for 6 months after electron beam irradiations (total 58 Gy). Intralesional IFN-α injections are effective on erythematous, superficial stage of extramammary Paget's disease, whereas it is very hard to get complete remission of advanced lesions by the intradermal IFN-α injections.

A rare case of papular mucinosis on the back of a 78-year old man is described. The patient developed approximately 20 discrete papules on the upper back. His serum glucose concentration was borderline elevated, but no other systemic symptoms or signs were apparent. A biopsy revealed mucin deposits only in the upper reticular dermis. The papules were distinctly different from lichen myxedematosus in clinical and histochemical appearance. The present case may represent very early-stage lichen myxedematosus complicated by borderline diabetes mellitus.

X-ray photon counting was performed using a readymade silicon-PIN photodiode (Si-PIN-PD) at tube voltages ranging from 42 to 60 kV, and X-ray photons are directly detected using the 100 MHz Si-PIN-PD without a scintillator. Photocurrent from the diode is amplified using charge-sensitive and shaping amplifiers. Using a multichannel analyzer, X-ray spectra at a tube voltage of 60 kV could easily be measured. The photon-counting computed tomography (PCCT) is accomplished by repeated linear scans and rotations of an object, and projection curves of the object are obtained by the linear scan. In the PC-CT, we confirmed the energy-dispersive effect with changes in lower-level voltage of the event pulse using a comparator.

X-ray photons are detected using an Lu 2 (SiO 4 )O [LSO] single-crystal scintillator with a decay time of 40 ns and a multipixel photon counter (MPPC). The photocurrent from the MPPC is amplified by a high-speed current-voltage amplifier with an 80 MHz-gain-band operational amplifier, and the 200-ns-width event pulses are sent to a multichannel analyzer to measure X-ray spectra. The MPPC is driven in the pre-Geiger mode at a bias voltage of 70.7 V and a temperature of 23°C. Photon-counting computed tomography (PC-CT) is accomplished by repeated linear scans and rotations of an object, and projection curves of the object are obtained by linear scanning. The exposure time for obtaining a tomogram was 10 min with scan steps of 0.5 mm and rotation steps of 1.0°. At a tube voltage of 100 kV, the maximum count rate was 350 kcps/pixel. We carried out PC-CT using gadolinium media and confirmed the energydispersive effect with changes in the lower level voltage of event pulses using a comparator.