Nishi Nihon Hifuka
Online ISSN : 1880-4047
Print ISSN : 0386-9784
Volume 80 , Issue 3
Showing 1-17 articles out of 17 articles from the selected issue
Index
Color Atlas
Mini Review
Clinical Case Reports
  • Yuka ARISUMI, Kotaro ITO, Shinichi IMAFUKU
    2018 Volume 80 Issue 3 Pages 196-199
    Published: June 10, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    A 7-month-old infant received a BCG vaccination in the upper left arm. After 3 months, scaly erythema appeared at the vaccinated site. He visited our hospital because of the spread of eruption. A biopsy from his back and had revealed parakeratosis with a small abscess and psoriasiform hyperplasia of epidermis. Therefore, treatment with topical corticosteroids was initiated under the diagnosis of psoriasis vulgaris. Eruption was refractory to the therapy at first but improved after 3 months. We concluded this case had psoriasis induced by BCG inoculation. Psoriasis in children has some epidemiological characteristics, such as predominance in girls, high frequency of parental psoriasis, and high genetic prevalence of HLA-Cw6, however, none of these characteristics were found in our case. Treatment options are limited in the practice of infantile psoriasis, and also it is often difficult to avoid mechanical stimuli. We reviewed the characteristics and therapeutic choice of previously reported cases of infantile psoriasis.

    Download PDF (1295K)
  • Kei SAKANO, Kotaro NAGASE, Machiko YOSHIOKA, Takuya INOUE, Yutaka NARI ...
    2018 Volume 80 Issue 3 Pages 200-204
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    A 38-year-old woman presented with a 20-year history of recurrent abdominal pain accompanied with fever lasting for a week. She had experienced diarrhea, followed by knee pain, ankle pain, wrist pain, and painful eruptions in the lower extremities. A dermatological examination revealed tender, reddish nodules on her lower extremities and the dorsa of both wrists. A histopathologic examination confirmed the diagnosis of erythema nodosum. The patient and her father, brother and sister were diagnosed with familial Mediterranean fever (FMF) based on a genetic examination 11 years ago. Erythema nodosum and joint pain reduced after the oral administration of colchicine. Autoinflammatory diseases, including FMF, are important differential diagnoses that should be considered as the underlying cause of urticarial, nodular, or erythematous skin lesions. The early diagnosis and treatment of FMF by colchicine administration are thought to be important in preventing the onset of amyloidosis, which is related to the prognosis of FMF.

    Download PDF (1564K)
  • Kaori KASHINO, Yoichiro TOI, Michiko HAYASHIDANI, Shinichi TAKADA, Mai ...
    2018 Volume 80 Issue 3 Pages 205-208
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS
    We report a Japanese male baby with a rare syndrome of restrictive dermopathy. His parents were non-consanguineous. He was born by Caesarean section after 34 weeks of gestation. The baby presented with tight, translucent skin through which the blood vessels could be seen, as well as skin erosions, a dysmorphic face, and multiple joint contractures. He died 20 minutes after birth. A histologic examination of the skin revealed parakeratosis, a lack of rete ridges in the epidermis, hypoplasia of the appendages, and a depletion of elastic fibers in the dermis. Gene analyses of the patient and his parents were performed and mutations in ZMPSTE24 were detected. Restrictive dermopathy is a lethal neonatal laminopathy. The laminopathies are a heterogeneous group of human disorders caused by a dysfunction of lamin A, which is thought to have roles in maintaining nuclear structures, regulating transcription, controlling differentiation, and organizing chromatin. The endoprotease ZMPSTE24, which is a lamin-binding protein, is required for the post-translational processing of the lamin A precursor (prelamin A) encoded by LMNA. In most restrictive dermopathy patients, a common mutation in the ZMPSTE24 gene leads to prelamin A accumulation in cells.
    Download PDF (1340K)
  • Nahoko IWASAKI-ZEZE, Masayoshi NAKAO, Eriko ITO, Junichi MOTOSHITA, Sa ...
    2018 Volume 80 Issue 3 Pages 209-213
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    We present two female cases of hidradenoma papilliferum on the vulva. The first case was a 40-year-old woman who recognized a subcutaneous nodule measuring 6 mm on the left of her vulva. The nodule was surgically resected and diagnosed as hidradenoma papilliferum histologically. The second case was a 38-year-old woman with a pink-colored pedunculated tumor measuring 1 cm on the left of her vulva, which often bled because of friction from underwear. The tumor was surgically resected and was also diagnosed as hidradenoma papilliferum histologically. It had previously been thought that hidradenoma papilliferum tumors originate from apocrine sweat glands, but recent studies have suggested that they may be derived from the anogenital mammary-like gland (MLG). The MLG is reported to be immunopositive for both estrogen receptor and progesterone receptor, while the majority of the normal eccrine or apocrine glands are not. Our two cases showed both estrogen receptor and progesterone receptor expression in the columnar cells of the tumor, suggesting that these cases of hidradenoma papilliferum may have been derived from the MLG.

    Download PDF (1498K)
  • Naoya MURAYAMA, Yuta KOIKE, Noriko TASAKI, Yutaka KUWATSUKA, Saori TOM ...
    2018 Volume 80 Issue 3 Pages 214-218
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    We report a case of 76-year-old female patient with rheumatoid arthritis, who presented diffuse large B cell lymphoma (DLBCL) developed on the lower leg. Previously, she had been administrated immunosuppressive treatment with methotrexate (MTX) (8 mg/week) and prednisolone (7. 5 mg/day) for 5 years. Physical examination showed sporadic erythema and nodules on the both lower legs, respectively. Topical therapies failed to treat those lesions. After that, the lesions formed a tumor on the left lower leg and formed ulcers on the right lower leg. Histological examination of the left lower leg showed large sized atypical lymphocytes with CD20-positive proliferated in the entire dermis, which demonstrated the diagnosis with DLBCL. In addition, Epstein-Barr virus (EBV) infection in the tumor cells were confirmed by EBV-encoded small RNA and EBV-latent membrane protein 1. On one hand, histological examination of the right lower leg showed oligoclonal proliferating T cells in the superficial dermis. After withdrawal of MTX therapy, the tumor rapidly regressed without chemotherapy. We diagnosed MTX-associated lymphoproliferative disorder (MTX-LPD) appeared as DLBCL due to the histopathological findings and the characteristic clinical course. When we examine a patient under treatment with MTX,we have to keep in mind that there is more risk of MTX-LPD which may induce malignant lymphoma.

    Download PDF (1751K)
  • Toshio ICHIKI, Gaku TSUJI, Kounosuke NAGAE, Maiko WADA, Teiichi MASUDA ...
    2018 Volume 80 Issue 3 Pages 219-223
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    An 81-year-old female noticed a subcutaneous nodule on her left buttock almost 1 year before her first visit to us. Because of its rapid growth, the lesion was resected. On histological examination, a proliferation of small round tumor cells was observed in the deep dermis and the subcutaneous tissue. Nuclear atypia and mitotic figures were also detected in the tumor cells. The tumor cells were positive for cytokeratin 20 and chromogranin A. An analysis by PET-CT scanning showed no other systemic involvement, indicating the diagnosis of primary Merkel cell carcinoma arising on the buttock. We performed an additional wide resection. However, a left inguinal lymphadenopathy was noticed 6 months later, which was histologically diagnosed as a lymph node metastasis of Merkel cell carcinoma. A subsequent left inguinal lymph node dissection revealed no residual Merkel cell carcinoma and the patient has been free from recurrence for 6 months after the final operation. Merkel cell carcinoma preferentially develops on sun-exposed areas and its occurrence on the buttock is rare.

    Download PDF (1378K)
  • Sayaka TAKAI, Yuiko YONEKURA, Aya NISHIZAWA, Michiro SUSA, Takahiro S ...
    2018 Volume 80 Issue 3 Pages 224-226
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS
    An 81-year-old man presented with a 1-month history of a rapidly growing nodule on the bend of the right elbow. Physical examination revealed a red, shiny, and hemorrhagic multilocular nodule measuring 3 cm in diameter. Histopathologically, the tumor consisted of two types of lesions, i.e., mucinous and hypercellular. Tumor cells with polygonal cytoplasms and atypical nuclei proliferated with elongated vessels in the mid-dermis and subcutaneous tissues. In both lesions, the tumor cells were positive for vimentin, but negative for S100 and CD34. A diagnosis of high-grade myxofibrosarcoma was made. The tumor was totally resected and neither local recurrence nor distant metastasis has been noted. Based on the cell density and cellular pleomorphism, myxofibrosarcoma is classified into three types ; low,intermediate, and high grade. Metastasis to the lungs and lymph nodes commonly occurs with high-grade tumors.
    Download PDF (1555K)
Clinical and Investigative Reports
  • Seiko NAITO, Osamu YAMASAKI, Tatsuya KAJI, Minoru TAKATA, Kenji ASAGOE ...
    2018 Volume 80 Issue 3 Pages 227-230
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS
    The clinical features and genotype of melanoma of unknown primary (MUP) in Japan are unknown. We performed a clinical investigation and genetic analysis of 6 patients with MUP who had visited our department during the past 15 years. The average age of the patients was 56.6 years and the ratio of males to females was1:1. The disease was diagnosed with subcutaneous tumors in 4 cases and images in 2 cases. The metastatic sites at the first visit were all lymph nodes, followed by metastases into the subcutaneous, lung, brain, bone, and liver tissues. The survival time of the fatal 4 cases was 10.5 months. Driver mutations were detected in 4 out of 6 patients. The BRAFV600E mutation was found in 2 patients. Two other patients had gene mutations of BRAFV600E + CDK4 R24C and EPHB6 G404S + PDGFRA E996K + ERBB4 E452K, respectively. MUP resembles the genotype of 29 cases of primary cutaneous melanoma.
    Download PDF (1342K)
Statistics
  • Osamu OKAMOTO, Rui SUZUKI, Manami KUSATSU, Kaho MATSUDA, Seiichi SATO ...
    2018 Volume 80 Issue 3 Pages 231-238
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    We statistically analyzed the clinical parameters and laboratory data in patients with cellulitis/erysipelas in order to predict the clinical course and trends. The younger patients were predominantly male, while the older patients were predominantly female. During a pretreatment period, the C-reactive protein (CRP) values remained elevated as long as the pretreatment period was increased, whereas the white blood cell (WBC) counts and body temperature tended to decrease. When treatment was initiated earlier after the disease onset, the CRP values tended to decrease after reaching a peak ; in contrast, the WBC counts and temperature decreased early after the initiation of treatment, irrespective of the time of the disease onset. Eight point eight±6.0 days were required for the CRP values to drop below 2 mg/dl, while the WBC count and temperature required 4.9±5.9 days and 5.7±5.5 days respectively, to normalize. The maximum CRP values and WBC counts showed strong and significant positive correlations with the time required for these values to decrease. As the maximum CRP value increased, the treatment period became longer ; furthermore, the procalcitonin level greatly influenced the time required for the CRP value to decrease. Continuous usage of anti-inflammatory agents slightly decreased the maximum temperature ; however, it did not greatly influence other trends. Patients that presented with a highly deviated treatment period, lesional area, maximal CRP value, WBC count, temperature, or time required for their reduction (extreme cases) tended to be older and to have higher procalcitonin values in comparison to the usual cases. Furthermore, sepsis was significantly more frequent in the extreme cases. On the other hand, there were no significant differences between the usual cases and the cases whose laboratory values remained within the normal limits (normally-valued cases) ; these cases accounted for one quarter of the patients in the study population.

    Download PDF (1696K)
Review
Therapy
  • Naoko MOCHIMARU, Hiroshi KAWASAKI, Ayano FUKUSHIMA, Sayoko OBATA, Fumi ...
    2018 Volume 80 Issue 3 Pages 244-249
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    Improving treatment adherence in atopic dermatitis patients has been highlighted as a major issue by the Clinical Practice Japanese Guidelines for the Management of Atopic Dermatitis 2016. Lotion formulations have gained attention for superior sensation on application, and we hypothesized that switching from ointments to lotions would contribute to improved treatment adherence. This study investigated the efficacy, safety, and sensation on application of betamethasone butyrate propionate lotion and the treatment adherence during continuous eight-week monitoring of patients who switched from a steroid ointment of the same class (‘very strong’steroids), for external use on the trunk and limbs. Switching to betamethasone butyrate propionate lotion yielded significant improvements in the severity scoring of atopic dermatitis index,thymus and activation-regulated chemokine levels, visual analog scale scores, lack-of-sleep index, and dermatology quality of life index. Questionnaire responses revealed high ratings for the sensation on application, suggesting that the lotion contributed to improved adherence. The results of this study indicate that switching to a lotion may be an effective option for the treatment of atopic dermatitis on the trunk and limbs.

    Download PDF (1487K)
Dermatologists around the World
  • Michel Gilliet
    2018 Volume 80 Issue 3 Pages 255-256
    Published: June 01, 2018
    Released: November 09, 2018
    JOURNALS RESTRICTED ACCESS

    Dr. Gilliet is Professor of Dermatology and Chairman of the Department of Dermatology at the Lausanne University in Switzerland. Over the past 15 years Dr. Gilliet's laboratory has focused on translational research studying inflammatory skin diseases. In particular, Dr. Gilliet's lab has discovered mechanisms how dendritic cells initiate and drive inflammation in skin diseases including psoriasis and lupus based the complex formation of self-DNA with antimicrobial peptides. These studies provided a paradigm shift in the understanding on how sterile inflammation is regulated at the site of disease.

    Download PDF (1251K)
feedback
Top