bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical Course of 44 Cases of Localized Type Vitiligo
Authors: Zaima Hiroyuki , Koga Michiyuki 
Published: July 2014

AbstractVitiligo is often classified into three types, generalized, segmental, and localized, on the basis of their distribution pattern. It is also classified into type A (non‐dermatomal or non‐segmental) and type B (dermatomal or segmental) vitiligo on the basis of both the distribution pattern and physiological function (1). The natural courses of type A and type B vitiligo are characteristic and quite different from each other. Whereas type A vitiligo appears at any age and progresses throughout the patient's life span, type B vitiligo affects the young and stabilizes within a few years (2). Segmental type vitiligo corresponds to type B, and generalized type vitiligo is the late stage of type A. However, no one has observed the course and character of localized type vitiligo, and its nosological position in A/B classification is unclear. We followed 44 cases of localized type vitiligo for periods of 6 months to 8 years. In 3 of the 44 patients, new white patches developed within the same dermatome as their affected areas in the first 12 months and stabilized in a short period. Therefore, these patients were diagnosed as type B vitiligo. In 15 patients, vitiligo developed in other dermatomal areas; the earliest case at 9 months, and others later on. The new white patches continue to develop for a long period, so these patients were diagnosed with type A vitiligo. In 26 of the 44 patients, the vitiligo remained localized within the period of observation. It is concluded that most localized type vitiligo is the early stage of type A, but a small number of cases belong to the early stage of type B.
A Case of Cellular Schwannoma of the Skin Presenting as a Large Ulcerated Tumor on the Ankle
Authors: Kang Sook‐Kyoung , Chang Sung‐Eun , Choi Jee‐Ho , Sung Kyung‐Jeh , Moon Kee‐Chan , Koh Jai‐Kyoung 
Published: July 2014

AbstractCellular schwannoma (CS) is a rare, benign, nerve sheath tumor. It is slowly growing and painless, and it shows a predilection for the paravertebral region of the mediastinum and the retroperitoneum. CS rarely occurs in the skin. It has more cellular and more atypical cytological features than ordinary schwannoma but follows a benign clinical course. The key histological feature is a distinct predominance of cellular Antoni A tissue. Herein we report a case of CS presenting as a 5 × 5 × 3 cm, ulcerated and protruding tumor on the left ankle and describe its indolent clinical course.
Docetaxel Induced Durable Response in Advanced Extramammary Paget's Disease: A Case Report
Authors: Oguchi Shinji , Kaneko Miwako , Uhara Hisashi , Saida Toshiaki 
Published: July 2014

AbstractThe patient was a 54‐year‐old Japanese man with a seven‐month history of an eroded lesion on the scrotum. Skin biopsy confirmed the diagnosis of extramammary Paget's disease, and a CT scan revealed multiple metastases to the lymph nodes along the iliac artery and aorta. The patient underwent combination chemotherapy with mitomycin C, epirubicin, vincristine, cisplatin, and 5‐fluorouracil. After two courses of the regimen, the primary lesions on the scrotum regressed, however, the lymph node metastases did not respond. A regimen of continuous administration of low‐dose cisplatin and 5‐fluorouracil was not effective for the metastases either. Hence, administration of docetaxel at a dose of 60 mg/m2/every four weeks was started. After two courses, the lymph node metastases decreased by 75% compared to the pre‐treatment size, and the effect was judged a partial response. The major toxicities were neutropenia, alopecia, pitting edema, and facial erythema, but these were tolerable. Five courses of this regimen were followed by four courses of weekly administration of 25 mg/m2 docetaxel. The partial response persisted for more than 12 months and the patient was able to enjoy daily life. Docetaxel may be an effective drug for patients with advanced extramammary Paget's disease.
Pseudoxanthoma Elasticum: Report of a Case without any Mutations in 5 Exons of the MRP6 Gene
Authors: Ohtani Toshio , Furukawa Fukumi 
Published: July 2014

AbstractPseudoxanthoma elasticum (PXE) is an inherited systemic disorder of connective tissue. We describe a patient with PXE who does not have mutations in exons 16, 24, 27, 28, and 30 of the MRP6 gene.
Sweet's Syndrome Evolved from Recurrent Erythema Nodosum in a Patient with Myelodysplastic Syndrome
Authors: Nishie Wataru , Kimura Tetsunori , Kanagawa Michiyo 
Published: July 2014

AbstractA 63‐year‐old man had painful nodules on his lower legs. Microscopic examination showed septal and lobular panniculitis composed of lympho‐histiocytic infiltrates. Based on the clinical and histopathological findings, the diagnosis of erythema nodosum (EN) was made. Nonsteroidal anti‐inflammatory drugs were temporarily effective, but the eruptions had repeated to the present, and 16 months later, myelodysplastic syndrome (MDS) was diagnosed. Then, 6 months later, he developed a high fever and edematous fresh red‐colored nodules on his neck, arm and upper trunk. Histopathologically, a diffuse, dense, dermal infiltrate of neutrophils was seen, and Sweet's syndrome (SS) was diagnosed. SS is known to develop in patients with MDS, and EN is one of the dermatoses that occur in conjunction with hematoproliferative disorders. Furthermore, SS evolving from recurrent EN and the simultaneous occurrence of SS and EN have been reported in some patients. In our case, we suggest that some mediators such as cytokines associated with MDS might have first induced EN, and then, as the MDS developed, they were replaced by others that caused SS.
Increased Microorganisms DNA Levels in Peripheral Blood Monocytes from Psoriatic Patients Using PCR with Universal Ribosomal RNA Primers
Authors: Okubo Yukari , Oki Noriko , Takeda Hidemi , Amaya Misato , Ito Sonoko , Osada Masako , Utsumi Masako , Koga Michiyuki , Kawashima Hisashi 
Published: July 2014

AbstractIt has long been suspected that systemic and focal infections cause or exacerbate psoriatic lesions. We previously showed that peripheral blood monocytes in psoriatic patients are activated and overproduce inflammatory cytokines. In addition, it has been reported that macrophages activated by ingesting microorganisms release tumor necrosis factor (TNF)‐α and interleukin (IL)‐1β. Therefore we hypothesized that the monocytes in psoriatic patients may be activated by ingesting microorganisms and overproduce inflammatory cytokines. We examined the detection of microorganism DNA in monocytes from 15 patients with psoriasis vulgaris and from 12 healthy controls. DNA was extracted from monocytes, and a polymerase chain reaction (PCR) assay was performed for the detection using universal primers from conserved regions of the bacterial 16S ribosomal RNA gene or the fungal 18S rRNA gene. At the same time, we calculated the psoriasis area and severity index (PASI) scores and analyzed their correlations with the microorganisms DNA levels. The results showed that bacterial 16S DNA levels in monocytes were significantly higher in psoriatic patients than in controls. The fungal 18S DNA levels were also higher in psoriatic patients than in controls, but the differences were not significant. Although the microorganisms DNA levels in monocytes of psoriatic patients were high, there was no correlation between the bacterial DNA levels in monocytes of the psoriatics and PASI scores. Our study suggests that monocytes in psoriatic patients engulf more bacteria than there in controls, causing an activation of monocytes and triggering the formation of new lesions in the initial stages of psoriasis.
Kikuchi's Disease (Histiocytic Necrotizing Lymphadenitis) with Cutaneous Involvement
Authors: Imai Ken , Yokozeki Hiroo , Nishioka Kiyoshi 
Published: July 2014

AbstractWe report a case of Kikuchi's disease (histiocytic necrotizing lymphadenitis) in a 22‐year‐old Japanese man who presented with cervical and axillary lymphadenopathy and who developed edema of lips with desquamation and erosions and transient indurated erythematous papules on the face, neck and upper extremities. A lymph node biopsy specimen showed the typical necrotizing lymphadenitis of Kikuchi's disease. A skin biopsy specimen showed a dense, lymphohistiocytic, superficial, deep perivascular, and interstitial infiltrate, papillary dermal edema, and abundant nuclear debris with a conspicuous absence of neutrophils, paralleling the nodal histology of Kikuchi's disease. CD68 immunohistochemistry showed many plasmacytoid monocytes. Oral corticosteroid (prednisolone 40 mg) therapy improved the skin lesions and other clinical symptoms. Lip involvement by Kikuchi's disease has not been reported, and should be included as one of the possible cutaneous manifestations of Kikuchi's disease.
A Synovial Sarcoma beneath the Pectoralis Major Muscle
Authors: Yano Toshie , Inoue Chizuko , Yano Kenji 
Published: July 2014

AbstractSynovial sarcoma is a rare malignant neoplasm that develops most frequently in the extremities. A 68‐year‐old man presented with a painful mass on the right side of his chest with a 10‐year history. Gross findings revealed a mass beneath the pectoralis major muscle. The pathologic findings showed the tumor was predominantly composed of spindle cells the arranged in compact interdigitating fascicles and scattered epithelial nests were seen. A diagnosis of synovial sarcoma was made.
Pseudoxanthoma Elasticum
Authors: Ohtani Toshio , Furukawa Fukumi 
Published: July 2014

AbstractPseudoxanthoma elasticum (PXE) is a heritable disease characterized by dermal, ocular, and vascular lesions that result from degeneration of the elastic fibers. Recently, the ATP‐binding cassette subfamily C member 6 (ABCC6) gene has been demonstrated to be responsible for PXE, and 43 mutations have been identified to date. However, it is still unknown how mutations in the ABCC6 gene can lead to manifestations of PXE.
A Case of Recessive Dystrophic Epidermolysis Bullosa with Esophageal Stenosis Treated with Endoscopic Bougienage
Authors: Lee Chan‐Woo , Song Ki‐Hoon , Kim Ki‐Ho 
Published: July 2014

AbstractsRecessive dystrophic epidermolysis bullosa (RDEB) is a rare, hereditary, chronic, noninflammatory mechanobullous disease. Esophageal involvement is invariable in RDEB patients. A 15‐year‐old girl visited with protracted vesiculobullous skin eruptions since birth. On physical examination, bullae, atrophic scars, milia, crusts and erosions were noted primarily on the distal extremities. Loss of entire nails and syndactyly of the right fourth and fifth toes were seen. Diagnosis of RDEB was confirmed by clinical, histopathologic, immunofluorescent and electron microscopic findings. Endoscopic and esophagographic studies revealed a stenosis and several hemorrhagic bullae and erosions. She successfully underwent bougienage under endoscopic and fluoroscopic control
Localized Involutional Lipoatrophy: Report of Six Cases
Authors: Yamamoto Toshiyuki , Yokozeki Hiroo , Nishioka Kiyoshi 
Published: July 2014

AbstractWe herein report six cases of localized involutional lipoatrophy, who presented with a depressive plaque on the lateralis of the upper arm. All patients were female, and three had previous injections for allergic rhinitis at the affected sites before the development of lipoatrophy. One patient received intramuscular injection at the affected site when she had a cold. Histological examination showed fat lobules composed of small lipocytes in the hyaline connective tissue. Immunohistochemical examination revealed a number of CD68‐positive macrophages in the fat lobules. We consider that localized involutional lipoatrophy is more common than has been reported, and is occasionally induced by local injections.
Idiopathic CD4+ T Lymphocytopenia Associated with Disseminated Flat Warts and Alopecia Areata
Authors: Gubinelli Emanuela , Posteraro Patrizia , Girolomoni Giampiero 
Published: July 2014

AbstractIdiopathic CD4+ T lymphocytopenia is a very rare condition characterized by persistent depletion of circulating CD4+ T lymphocytes, without evidence of HIV or HTLV infection, or other identifiable causes of immunodeficiency. The syndrome can present with dermatological diseases, including viral, fungal and bacterial infections, as well as Kaposi's sarcoma, epithelial cell malignancies, lymphoma and inflammatory dermatoses. We report the case of a 47‐year‐old woman with idiopathic CD4+ T lymphocytopenia who presented with a 10‐year history of disseminated and refractory flat warts from which human papillomavirus type 3 DNA was identified. The patient also had alopecia areata.
A Case of Hypersensitivity Syndrome due to Phenytoin
Authors: Nagai Yayoi , Hattori Tomoyasu , Ishikawa Osamu 
Published: July 2014

AbstractWe report a 30‐year‐old female with hypersensitivity syndrome (HS) due to phenytoin. Her disease course was typical with elevation of the antibody titer for human herpes virus 6. Three courses of methylpredonisolone pulse therapy were required to control the disease activity. Since the pathogenic mechanism and treatments for HS have not been established, further clinical analyses are warranted.
New Aspects of Cutaneous Mosaicism
Authors: Happle Rudolf 
Published: July 2014

AbstractThe concept of cutaneous mosaicism has today been proven at the cellular level in at least fifteen different skin disorders. We can distinguish five different patterns of mosaicism, including the phylloid pattern and the lateralization pattern. Etiologically, cutaneous mosaics can be divided into two large categories, epigenetic mosaicism and genomic mosaicism. All forms of epigenetic mosaicism known so far, including the various patterns of X‐inactivation, appear to be caused by the action of retrotransposons. A new concept is functional autosomal mosaicism transmittable through the action of retrotransposons, which has been described in mice and dogs and may explain, for example, the familial occurrence of pigmentary mosaicism along the Blaschko lines in human skin. Among the examples of mosaicism of autosomal lethal mutations, phylloid hypomelanosis is a recently recognized neurocutaneous entity caused by mosaic trisomy 13. Possible examples of a type 2 segmental manifestation now include at least fifteen different autosomally dominant skin disorders. This phenomenon is most frequently found in glomangiomatosis, cutaneous leiomyomatosis, and disseminated superficial actinic porokeratosis. Recently proposed examples of didymosis (twin spotting) include cutis tricolor, paired patches of excessive or absent involvement in Darier disease, and didymosis aplasticosebacea characterized by coexistent aplasia cutis congenita and nevus sebaceus. To the list of possible examples of paradominant inheritance, cutis marmorata telangiectatica congenita and speckled lentiginous nevus syndrome have now been added. Revertant mosaicism giving rise to unaffected skin areas in autosomally recessive cutaneous traits will certainly likewise be recognized more often when clinicians are bearing this concept in mind. Such cases can be taken as examples of “natural gene therapy”.
Initial Recruitment of Interferon‐γ‐Producing CD8+ Effector Cells, Followed by Infiltration of CD4+ Cells in 2,4,6‐Trinitro‐1‐Chlorobenzene (TNCB)‐Induced Murine Contact Hypersensitivity Reactions
Authors: Okazaki Fusako , Kanzaki Hiroko , Fujii Kazuyasu , Arata Jirô , Akiba Hitoshi , Tsujii Kazuhide , Iwatsuki Keiji 
Published: July 2014

AbstractContact hypersensitivity (CHS) is an antigen‐specific, T‐cell‐mediated skin reaction in sensitized individuals. Recent studies have demonstrated that the murine CHS reaction to 2,4‐dinitrofluorobenzene (DNCB) is mediated by CD8+ T cells and down‐regulated by CD4+ T cells. We studied cellular events and functions of infiltrating cells in CHS reactions to 2,4,6‐trinitro‐1‐chlorobenzene (TNCB) in the skin and draining lymph nodes (LN) of BALB/c mice and compared them with the CHS reaction to a house dust mite antigen, Dermatophagoides farinae (Df). Mice were sensitized with TNCB or Df antigens, and CHS reactions elicited with the corresponding antigens were examined immunohistologically. Cytokines produced by individual cells isolated from the CHS reactions were analyzed by flow cytometry, and the expression of cytokine mRNA was assayed by reverse transcription‐polymerase chain reaction (RT‐PCR). Results demonstrated that the intensity of TNCB‐elicited CHS skin reactions reached its peak at 24 hr after elicitation and decreased gradually. Flow cytometric analysis of isolated cells from the TNCB‐elicited CHS reactions demonstrated that the infiltrating cells were composed of approximately 25% CD4+ and CD8+ cells at 12, 24, and 36 hr after challenge, although infiltrating cells became dense at 36 hr by histological observation. The percentage of IFN‐γ‐producing CD8+ cells (Tc1) in the cell fractions reached its peak at 12 hr and decreased gradually. The peak infiltration of IFN‐γ‐producing CD4+ cells (Th1) was observed at 24 hr. IL‐4‐producing cells, however, were always below 5% in the cell fractions. The RT‐PCR method demonstrated that IFN‐γ mRNA was detected in the TNCB‐elicited skin reactions at 12, 18 and 24 hr after elicitation, became weak at 48 hr, and disappeared at 72 hr. No IL‐4 mRNA was detected from 12 to 72 hr. In the draining LN cells, however, the percentages of both IFN‐γ‐producing CD8+ (Tc1) and CD4+ cells (Th1) decreased 12 to 36 hr after TNCB elicitation. CHS reactions of Df antigens were predominantly composed of infiltrations of CD4+ cells in to the skin, associated with the expression of IL‐4 mRNA from 12 to 48 hr after elicitaion. The expression of IFN‐γ mRNA was detected at 48 hr or later. Our findings indicate that the CHS skin reaction to TNCB is induced by early recruitment of IFN‐γ‐producing CD8+ effector cells (Tc1), followed by infiltration of IFN‐γ‐producing CD4+ cells (Th1), whereas IL‐4‐producing T cells (Th2) induce the early CHS response to Df antigens.
Erythema Gyratum Repens Responding to Cetirizine Hydrochloride
Authors: Miyagawa Fumi , Danno Kiichiro , Uehara Masami 
Published: July 2014

AbstractWe report a case of erythema gyratum repens (EGR) in a 59‐year‐old man with inoperable pancreatic cancer and liver metastasis. The patient had a widespread erythema with concentric marginal band spreading in waves over the trunk and extremities. Numerous vesicles were seen on the margin of the erythema. The skin lesions were severely pruritic, and his peripheral blood showed marked eosinophilia. He also had palmoplantar hyperkeratosis. A biopsy specimen of the erythema disclosed spongiosis, microvesicles filled with eosinophils, infiltration of eosinophils into the epidermis, and a perivascular infiltrate in the dermis. The skin lesions and pruritus cleared quickly after the administration of cetirizine hydrochloride.
A Keratin 9 Gene Mutation (Asn160Ser) in a Japanese Patient with Epidermolytic Palmoplantar Keratoderma
Authors: Tsunemi Yuichiro , Hattori Naoko , Saeki Hidehisa , Adachi Makoto , Komine Mayumi , Nakagawa Hidemi , Tamaki Kunihiko 
Published: July 2014

AbstractWe described a 5‐year‐old Japanese girl with epidermolytic palmoplantar keratoderma and examined her for a keratin 9 gene mutation. Physical examination disclosed diffuse yellowish hyperkeratosis with an erythematous border limited strictly to the palms and soles. Histological examination revealed hyperkeratosis with vacuolar degeneration in the spinous and granular layers of the epidermis. Sequence analysis demonstrated an A to G transition at the middle position of codon 160 in the 1A domain of the keratin 9 gene. The amino acid at codon 160 was deduced to have changed from asparagine (Asn) to serine (Ser). This is the first case with an Asn160Ser mutation in a Japanese. The substitution of Ser for Asn at codon 160 of the keratin 9 gene is assumed to be fatal for keratin filament assembly regardless of race or ethnicity.
Psoriasis Vulgaris and Poland's Syndrome in the Same Patient
Authors: Sasmaz Sezai , Coban Y. Kenan , Boran Cetin , Gumusalan Yakup 
Published: July 2014

AbstractMost common anomalies associated with Poland's syndrome are in the chest wall. This type of anomaly causes important psychological distress, especially in adolescent girls. A 19‐year‐old female with Poland's syndrome having psoriatic plaques on her trunk, knees, and elbows is reported in this paper. Such an association of Poland's syndrome with psoriasis has not been described previously.
A Case of Polypoid Dermatofibroma
Authors: Sogabe Yoko , Takahashi Ayumi , Tamura Atsushi , Ryuzaki Keiichiro , Ishikawa Osamu 
Published: July 2014

AbstractDermatofibroma is a common benign cutaneous tumor that usually appears as a slowly growing firm nodule. Polypoid nodular dermatofibroma is a variant type that is rarely encountered. We reported a case of polypoid dermatofibroma with a review of the previously reported cases. Polypoid dermatofibroma tends to arise on the leg, especially below the knee. Its size is often larger than that of common dermatofibroma. It is speculated that both the underlying firm tissue and long‐term development may lead the tumor to form a polypoid appearance.
Co‐Existence of Variants of Porokeratosis: A Case Report and a Review of the Literature
Authors: Kaur Sukhjot , Thami Gurvinder P. , Mohan Harsh , Kanwar Amrinder J. 
Published: July 2014

AbstractRarely, different variants of porokeratosis may coexist in an individual patient or their family members. A patient with the linear form of porokeratosis present since birth subsequently developed the disseminated superficial actinic form at a later age. A review of the literature pertaining to the coexistence of variants of porokeratosis suggests a significant association between the linear and disseminated superficial actinic forms. Genetic linkage between different variants and the basis for their association is discussed.
Intradermal Nodular Fasciitis of the Face
Authors: Kang Sook‐Kyoung , Kim Hyoung‐Hun , Ahn Se‐Jin , Choi Jee‐Ho , Sung Kyung‐Jeh , Moon Kee‐Chan , Koh Jai‐Kyoung 
Published: July 2014

AbstractNodular fasciitis represents reactive myofibroblastic proliferative lesions in the subcutaneous tissue, skeletal muscles, and fascia. Uncommon clinical and pathological variants of nodular fasciitis, such as intravascular, cranial, ossifying, and proliferative fasciitis, have been described. Six cases documenting a dermal location of nodular fasciitis have been reported in the English literature. Some authors have described that the histopathological features of intradermal nodular fasciitis are virtually identical to those of postoperative/posttraumatic spindle cell nodule of the skin. We herein report a 41‐year‐old Korean woman presenting with a 0.5 cm facial papule in which nodular fasciitis occurred in the dermis.
Wolf's Isotopic Response: A Case of Zosteriform Lichen Planus
Authors: Türel Aylin , Öztürkcan Serap , Şahin M. Turhan , Türkdoğan Peyker 
Published: July 2014

AbstractLichen planus is a lichenoid disorder characterized by shiny, flat papules. In addition to the classical appearance, there are several variants. Zonal or zosteriform lesions have been described. A 25‐year‐old male with a complaint of increasing numbers of erythematous swellings on his left groin for twenty days was admitted to our out‐patient clinic. He had a history of herpes zoster in the same localization which had been treated with topical acyclovir two weeks prior to his admission. Dermatological examination revealed multiple, shiny, erythematous, umblicated papules localized to the left inguinal region in a linear pattern. A biopsy was taken from the lesions. According to the clinical and pathological findings the diagnosis was zosteriform lichen planus. Zosteriform lichen planus is a rare variant of lichen planus; its differentiation from zona zoster and other linear dermatoses is difficult. We presented our case because of its rarity as a variant of lichen planus and its appearance in the area of healed herpes zoster as an isotopic response.