We report a 12-year-old man who developed purpura and bulla in the lower extremities followed by high fever, arthralgia, dry cough, hemoptosis, abdominal pain and diarrhea. The laboratory data revealed proteinuria, hematuria, elevated ESR, eosinophilia, positive reumatoid factor and abnomal shadows in the chest and nasopharingeal X ray examinations. The histological findings revealed leukocytoclastic vasculitis in the skin, pulmonary bleeding in the lung and focal necrotizing glomerulonephritis in the kidney. Prednisolone therapy (PSL; 1 mg/kg/day) failed, but PSL and cyclophosphamide therapy (CPM; 100 mg/day) proved to be markedly effective. After six months of treatment with PSL plus CPM, both the clinical symptons and abnormal laboratory data disappeared.

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A case of adult Still’s disease complicated with fulminant hepatitis is reported. A 21-year-old Japanese woman complaining of recurrent arthralgia and urticaria-like erythema along with high fever was admitted to our hospital. The main laboratory findings included leukocytosis, a slight liver disfunction, elevated serum ferritin, and negative rheumatoid factor. After treatment with prednisolone, her symptoms dramatically improved. Following the discontinuation of prednisolone, a relapse with complications of fulminant hepatitis occurred 2 months later. At the time of relapse the erythema lasted two or three days longer than usual for adult Still’s disease and thereafter developed into postinflammatory pigmentation. Liver disfunction with adult Still’s disease might therefore sometimes be complicated with fluminant hepatitis. Even though the prognosis of adult Still’s disease is generally good, it is necessary to closely observe its clinical course. We also discuss postinflammatory pigmentation and hepatitis in relation to the prognosis of adult Still’s disease.

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We describe the clinical and pathological observations of a 73-year-old female with malignant atrophic papulosis (MAP). She had characteristic eruptions, with either bean-sized erythematous or normal skin colored atrophic papules on her trunk and extremities without any abdominal or neurological symptoms. We performed a histopathologic examination on 3 occasions. Wedge shaped infarctions in the dermal and subcutaneous tissue were observed in all specimens, and only one thrombosis of the vessel in the dermis was found. In the gastrointestinal tract, no lesions of ulceration or perforation were noted. She was treated with prostaglandin I₂, which had vasodilator and antiplatelet aggregatory properties, and she had been followed a good clinical course.

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A 57-year-old female patient suffering from severe burns (grade II and III over 80% of the body and a burn index of 70%) developed leukopenia and thrombocytopenia after 3 days of treatment with famotidine and silver sulfadiazine. The white blood cell (WBC) and platelet counts later recovered after treatment was tapered. The serum silver levels were determined to range from 224ng (at the time of the lowest WBC count) to 161 ng/ml (after recovery). Even after recovery of the WBC and platelet counts, their levels still remained high. These data therefore suggest that adverse effects may be caused by the combined treatment of famotidine and silver sulfadiazine.

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We report a case of a 9-month-old girl with a sacrococcygeal tumor. Microscopical examinations revealed that the tumor was a mature type of sacrococcygeal teratoma which has been well known to increase the rate of malignant changes as time passes. We emphasize that an early diagnosis of this disorder is required in order to obtain a better prognosis.

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We report a case of subungual exostosis as a clinical feature of hereditary multiple exostosis (HME) in a 14-year-old girl. Her father and grand father had many subcutaneous hard bone tumors on their extremities. The tumors had been developing on her extremities since then she was 5 or 6 years old. She visited our hospital complaining of a deformity on her nails of both the left index and middle fingers. A roentgenologic study revealed two bony tumors on the dorsomedian side of the distal phalanges and many other tumors on the metaphysis of the tubular bones. The tumors ocated on the proximal ends of the phalanges and showed the continuance of the contour of the tumor and the cortex of phalanges. These findirgs suggested that this case was distinguishable from traumatic subungual exostosis.

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The four types of leiomyoma of the skin are classified as: multiple piloleiomyoma, solitary piloleiomyoma, solitary genital leiomyoma and solitary angioleiomyoma. There have been 101 reports of multiple piloleiomyoma since 1924 till 1991 in Japan, however the occurrence of familial multiple piloleiomyoma is rare. We report a case of familial multiple piloleiomyoma. The patient was a 41-year-old man who had been suffering from multiple papulo-nodular, firm, cutaneous lesions over both upper extremities since the age of 20 years. The skin lesions gradually became more prominent and numerous until they finally spread over both shoulders, the left lower extremity and chest. The skin lesions were up to 2 cm in diameter, firm, red to brown nodules arranged in groups. Some nodules were tender and gave rise to occasional spontaneous attacks of pain. His younger brother (a 31-year-old-man) had also previously experienced similar skin lesions over the right mandibular and right neck region, while his elder sister had similar skin lesions over the left neck and his paternal uncle had similar skin lesions over his left upper extremity and back. The histopathological findings showed typical leiomyoma in three of the 4 cases. The serum levels of estrogen were normal in all cases, but the tumor cells were positive for immunoperoxidase staining with estrogenic receptor. We considered that the cause of leiomyoma was not related to the serum levels of estrogen, but instead to the level of estrogenic receptor in the erector muscles of body hair.

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We report a case of cutaneous leiomyosarcoma in a 44-year-old Japanese female. The lesion was a brownish plaque of 25×36 mm in size on the left subscapular region and a subcutaneous nodule was palpable underneath. Histopathologically, spindle shaped tumor cells infiltrated from the upper dermis to the subcutaneous fatty tissue in a haphazard manner. Considerable nuclear pleomorphism and atypical mitotic figures were also noted. Electron microscopically, the tumor cells showed a jagged nucleus and few organelles in the cytoplasm. A small amount of thin filaments were noted in the cytoplasm, but a dense body and basement membrane were not conspicuous.

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A 40-year-old woman with von Recklinghausen’s disease developed a firm, subcutaneous mass on the right buttock which was histologically compatible with malignant schwannoma. An immunohistochemical study revealed the positive reaction for S 100 protein, neuron specific enolase and vimentin in the tumor cells. Patients with von Recklinghausen’s disease must be very carefully followed up, so that malignant schwannoma can be diagnosed at an early stage and, then the appropriate surgical treatment can be performed.

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A case of benign symmetric lipomatosis is reported. The patient was a 65-year-old Japanese man with a history of alcohol abuse. Two years earlier he noticed diffuse or nodular soft masses, first on his neck and later on the bilateral forearms as well as in bilateral inguinal areas. Biopsy specimens from his neck and inguinal area showed the proliferation of normal sized fat cells without encapsulation. The laboratory examination showed an increase of enzymes which were considered to have derived from liver.

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We investigated the localization of basic fibroblast growth factor (bFGF) in the neurofibromas (NF) of the patients with von Recklinghausen’s disease (neurofibromatosis) by immunofluorescent staining. We found that bFGF was expressed in the macrophages infiltrating into NF and in the edothelial cells of the vessel walls of NF. Since bFGF has been known to induce oncogene c-fos in NIH 3T3 fibroblasts, the c-fos expression in NF was investigated. The oncogene c-fos products were observed in the nuclei of many NF cells either in the tumor nests or in the interstitial areas. These results indicate that the bFGF expressed in NF may thus induce oncogene c-fos activation and these cellular and molecular events may, as a result, be involved in the pathogenesis of NF in neurofibromatosis.

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Epidermolysis bullosa is classified into three main groups by the level of cleavage within the skin: (1) epidermolysis bullosa simplex; (2) junctional epidermolysis bullosa; and (3) dystrophic epidermolysis bullosa. The last group contains six subgroups, two of which are inherited in a dominant mode and four in a recessive manner. The two subgroups of dominantly inherited dystrophic epidermolysis bullosa are distinguished by the presence (Pasini form) or absence (Cockayne-Touraine form) of albopapular lesions, which appear on the lower back of some patients at adolescence. An early histochemical examination of our case revealed an increased amount of degraded dermatan sulfate in the skin of a patient with the Pasini form of dystrophic epidermolysis bullosa. The present study was designed to examine glycosaminoglycan chains of proteoglycans in the skin of a patient with Pasini form by histochemical and immunohistochemical methods. The monoclonal antibodies used in the present study were: 2B1 (against chondroitin sulfate large proteoglycan), 3B3 (against a stub of ΔDi-6S unit binding to core protein obtained from chondroitin sulfate proteoglycan with chondroitinase ABC), 6B6 (against small proteoglycan with dermatan sulfate and chondroitin sulfates), and CS56 (reacts with chondroitin sulfates but not with dermatan sulfate). A histological examination revealed no dermoepidermal separation. In the dermis, the papillary and subpapillary layers were slightly homogeneous, and also showed a strong affinity for alcian blue or toluidine blue, but these phenomena was abolished either by treatment with chondroitinase ABC or in the presence of MgCl₂ at 0.3M, but was resistant to streptomyces hyaluronidase. When immunostaining with monoclonal antibodies was carried out, the papillary and subpapillary layers were strongly stained with the 2B1 and 6B6 antibodies, as compared to the control sections. These results thus suggest the presence of degraded dermatan sulfate proteoglycan in the upper dermis of albopapular lesions in the Pasini form of dystrophic epidermolysis bullosa.

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[in Japanese]

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In Otaru City General Hospital, 84 cases of hymenoptera sting have been diagnosed and treated over the past 4 years since 1989 until 1992. In 1989 only 3 cases were treated, however, 29 and 30 cases were treated in 1990 and in 1991, respectively. The stings were most frequently observed in August. In all, 53 male and 31 female patients were treated. The most frequently treated age groups were those in their first decade and those in their thirties and forties. The most frequent sting sites included exposed areas such as the upper arms, head, face, ears, and neck. Hypersensitive reactions including anaphylactic shock, wheals, nausea, and vomitting were observed in 8 cases (9.5%). Two cases of anaphylactic shock were induced by stings of Polistes snelleni Saussure. A delayed large local reaction was observed in 7 cases (8.3%), while one developped lymphangitis after being stung by a Vespula.

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A statistical survey was performed on four hundred and sixteen Japanese patients with herpes zoster, who visited the dermatological clinic of Asahikawa Medical College since 1978 until 1992. These 416 cases (201 males and 215 females) accounted for 1.66% of the total number of new out-patients. The incidence of herpes zoster did not change from 1978 to 1992. A seasonal variation was observed with two peaks in March to May, and in August to October. Age distribution showed two peaks with the first appearing in patients in their twenties and the second in patients in their fifties and sixties. The most common site involved was the chest (27.1%) followed by the head (23.5%) and the waist and legs (22.3%). In patients over the age of 50, the face and neck areas were the most frequently affected. Two hundred and two patients (48.1%) had underlying disorders such as malignant tumors, hypertension, diabetes mellitus and collagen diseases. Generalized herpes zoster was seen in 25 cases (6.3%) with a predilection at the over 50 age group. A primary involvement of the head and underlying disorders such as malignant tumors and collagen diseases were other features of generalized herpes zoster. Post-herpetic neuralgia and face and neck lesions persisted longer in patients over the age of 50. Hunt syndrome occurred in five cases (1.2%) while herpes zoster ophthalmicus was observed in ten cases (2.4%).

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All cases of systemic scleroderma in Kumamoto Prefecture were examined epidemiologically and prognostically for two years starting on July 1, 1989. A total of 146 patients, 130 females and 16 males were examined. During the two-year observation period, 4 patients died (the mortality rate was 3.92%). The condition of scleroderma was evaluated in 65 patients during the observation period, and the patients were classified into either progressive, stationary or improving groups. The presence or absence of shortened digits, as well as the presence or absence of the disease overlapping with RA (rheumatoid arthritis) were statistically significantly associated with the prognosis of the three groups of patients, as well as with the worsening or improvement of proximal scleroderma, sclerodactylia, flexion contracture, Raynaud’s phenomenon and diffuse hyperpigmentation.

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Twenty-two cases of kerion celsi at Gifu University Hospital from January, 1979 to April, 1993 were statistically examined. The male and female ratio was 13:9, and 19 out of 22 cases were under 15 years old. The onset of lesions was common (11 out of 22 cases) in winter. The parietal region was the most predilected site (17 out of 22). Eleven cases had been previously treated with topical steroids. Dogs in 5 cases and cat in one case were suspected as the most likely source of infection. Contamination within the family occured in 7 cases. Ten strains of T. rubrum, 9 of M. canis, 1 of M. gypseum and 1 of T. violaceum were isolated from these patients. In addition, there were some differences regarding the species in our results compared to previous reports from other districts in Japan.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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Forty cases (31 of nevus Ohta, 6 of blue nevus and 3 of tatoos) were treated with a Q-switched ruby laser. The results were “cured” or “excellent” in 75%. A total of 6 cases received the treatment 6 or more times and all of them were either “cured” or showed “excellent” results. No scar formation or depigmentation was observed. This Q-switched ruby laser therapy may therefore be the best treatment of both nevus of Ota and blue nevus.

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[in Japanese]

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[in Japanese]

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