bluuebunny/crossref_metadata_2025 huggingface dataset:
Blockade of CD40–TRAF2,3 or CD40–TRAF6 is sufficient to inhibit pro‐inflammatory responses in non‐haematopoietic cells
Authors: Portillo Jose‐Andres C. , Greene Jennifer A. , Schwartz Isaac , Subauste Maria Cecilia , Subauste Carlos S.
Published: July 2014

Inhibition of the CD40–CD154 pathway controls inflammatory disorders. Unfortunately, administration of anti‐CD154 monoclonal antibodies causes thromboembolism. Blockade of signalling downstream of CD40 may represent an approach to treat CD40‐driven inflammatory disorders. Blocking tumour necrosis factor receptor‐associated factor 6 (TRAF6) signalling downstream of CD40 in MHC II+ cells diminishes inflammation. However, CD40–TRAF6 blockade may cause immunosuppression. We examined the role of CD40–TRAF2,3 and CD40–TRAF6 signalling in the development of pro‐inflammatory responses in human non‐haematopoietic and monocytic cells. Human aortic endothelial cells, aortic smooth muscle cells, renal proximal tubule epithelial cells, renal mesangial cells and monocytic cells were transduced with retroviral vectors that encode wild‐type CD40, CD40 with a mutation that prevents TRAF2,3 recruitment (ΔT2,3), TRAF6 recruitment (ΔT6) or both TRAF2,3 plus TRAF6 recruitment (ΔT2,3,6). Non‐haematopoietic cells that expressed CD40 ΔT2,3 exhibited marked inhibition in CD154‐induced up‐regulation of vascular cell adhesion molecule 1, intercellular adhesion molecule 1 (ICAM‐1), monocyte chemotactic protein 1 (MCP‐1), tissue factor and matrix metalloproteinase 9. Similar results were obtained with cells that expressed CD40 ΔT6. Although both mutations impaired ICAM‐1 up‐regulation in monocytic cells, only expression of CD40 ΔT6 reduced MCP‐1 and tissue factor up‐regulation in these cells. Treatment of endothelial and smooth muscle cells with cell‐permeable peptides that block CD40–TRAF2,3 or CD40–TRAF6 signalling impaired pro‐inflammatory responses. In contrast, while the CD40–TRAF2,3 blocking peptide did not reduce CD154‐induced dendritic cell maturation, the CD40–TRAF6 blocking peptide impaired this response. Hence, preventing CD40–TRAF2,3 or CD40–TRAF6 interaction inhibits pro‐inflammatory responses in human non‐haematopoietic cells. In contrast to inhibition of CD40–TRAF6 signalling, inhibition of CD40–TRAF2,3 signalling did not impair dendritic cell maturation. Blocking CD40–TRAF2,3 signalling may control CD40–CD154‐dependent inflammatory disorders.
Clinical implications of interferon‐γ genetic and epigenetic variants
Authors: Smith Nicola L. D. , Denning David W.
Published: July 2014

Interferon‐γ (IFN‐γ) is an integral and critical molecule of the immune system, with multiple functions, mostly related to the T helper type 1 (Th1) response to infection. It is critical for defence against mycobacterial infection and is of increasing interest in defence against fungi. In this article, we review the genetic and epigenetic variants affecting IFN‐γ expression and investigate its role in disease, with an emphasis on fungal diseases such as invasive and chronic pulmonary aspergillosis. Over 347 IFN‐γ gene variants have been described, in multiple ethnic populations. Many appear to confer a susceptibility to disease, especially tuberculosis (TB) and hepatitis, but also some non‐infectious conditions such as aplastic anaemia, cervical cancer and psoriasis. Several epigenetic modifications are also described, increasing IFN‐γ expression in Th1 lymphocytes and reducing IFN‐γ expression in Th2 lymphocytes. Recombinant IFN‐γ administration is licensed for the prophylaxis of infection (bacterial and fungal) in patients with the phagocyte functional deficiency syndrome chronic granulomatous disease, although the benefits appear limited. Interferon‐γ therapy is given to patients with profound defects in IFN‐γ and interleukin‐12 production and appears to be beneficial for patients with invasive aspergillosis and cryptococcal meningitis, but the studies are not definitive. A high proportion of patients with chronic pulmonary aspergillosis are poor producers of IFN‐γ in response to multiple stimuli and could also benefit from IFN‐γ administration. The investigation and management of patients with possible or demonstrated IFN‐γ deficiency in adulthood is poorly studied and could be greatly enhanced with the integration of genetic data.
The efficacy and safety of Grafix® for the treatment of chronic diabetic foot ulcers: results of a multi‐centre, controlled, randomised, blinded, clinical trial
Authors: Lavery Lawrence A , Fulmer James , Shebetka Karry Ann , Regulski Matthew , Vayser Dean , Fried David , Kashefsky Howard , Owings Tammy M , Nadarajah Janaki ,   The Grafix Diabetic Foot Ulcer Study Group
Published: July 2014

In a randomised, controlled study, we compared the efficacy of Grafix®, a human viable wound matrix (hVWM) (N = 50), to standard wound care (n = 47) to heal diabetic foot ulcers (DFUs). The primary endpoint was the proportion of patients with complete wound closure by 12 weeks. Secondary endpoints included the time to wound closure, adverse events and wound closure in the crossover phase. The proportion of patients who achieved complete wound closure was significantly higher in patients who received Grafix (62%) compared with controls (21%, P = 0·0001). The median time to healing was 42 days in Grafix patients compared with 69·5 days in controls (P = 0·019). There were fewer Grafix patients with adverse events (44% versus 66%, P = 0·031) and fewer Grafix patients with wound‐related infections (18% versus 36·2%, P = 0·044). Among the study subjects that healed, ulcers remained closed in 82·1% of patients (23 of 28 patients) in the Grafix group versus 70% (7 of 10 patients) in the control group (P = 0·419). Treatment with Grafix significantly improved DFU healing compared with standard wound therapy. Importantly, Grafix also reduced DFU‐related complications. The results of this well‐controlled study showed that Grafix is a safe and more effective therapy for treating DFUs than standard wound therapy.
Chronic Tophaceous Gout Presenting as Hyperpigmented Nodules in the Limbs of a Patient with Coexisting Psoriasis
Authors: Cho Soyun , Koh Gwang‐Jin , Choi Jee‐Ho , Sung Kyung‐Jeh , Moon Kee‐Chan , Koh Jai‐Kyoung
Published: July 2014

We describe a 53‐year‐old male renal transplant recipient with hypertension and triglyceridemia, who showed rare manifestations of gout presenting as brownish nodules on the arms and legs as well as chronic tophaceous gouty arthritis of the hands and feet mimicking rheumatoid arthritis, in association with subsequently developed psoriasis of the palms. In elderly Asian men, hypertension and renal insufficiency may be risk factors predisposing to the development of multiple hyperpigmented nodules of tophi in the more proximal extremities.
Detection of Species of the Subgenus Leishmania Parasites Using Polymerase Chain Reaction and Southern Blotting
Authors: Uezato Hiroshi , Takei Kimiko , Maruno Motoyoshi , Khaskhely Noor Mohammad , Nonaka Shigeo , Oshiro Minoru , Kariya Ken‐ichi , Katakura Ken , Mimori Tatsuyuki , Gomez L. Eduardo A. , Furuya Masato , Shamsuzzaman S.M. , Hashiguchi Yoshihisa
Published: July 2014

In this study, an attempt was made to identify different Leishmania species by polymerase chain reaction (PCR). Fourteen Leishmania strains from stock were tested by PCR and Southern blotting. A pair of primers were employed that anneal to the kinetoplast DNA sequence conserved among subgenus Leishmania. Of the 14 Leishmania strains used in this study, six showed strong bands of approximately 170 bp, and all the positive strains belonged to the species of the subgenus Leishmania viz., Leishmania (Leishmania) garnhami, L. (L.) amazonensis, L. (L.) pifanoi, L. (L.) mexicana, L. (L.) chagasi, and L. (L.) major. All the species belonging to the subgenus Viannia used in this study were negative by PCR. These results suggest that the primer pair may be useful for identification of the species belonging to the subgenus Leishmania of the New World as well as to distinguish subgenus Leishmania from subgenus Viannia.
Oral Ivermectin in Scabies Patients: A Comparison with 1% Topical Lindane Lotion
Authors: Madan Vishaal , Jaskiran Kaur , Gupta Usha , Gupta Devendra Kumar
Published: July 2014

Scabies, which constitutes a significant proportion of the outpatient attendance in tropical dermatology clinics, has so far been treated with lindane, crotamiton, sulphur, permethrin, etc. Ivermectin, an orally administered drug, was tried in scabies patients and compared with 1% topical lindane lotion to evaluate its effects and toxicity profile. Two hundred scabies patients were randomly allocated to one of two groups. One group received oral invermectin in a single dose of 200 micrograms/kg body weight. The other received 1% lindane lotion for topical application overnight. Patients were assessed after 48 hours, two weeks and four weeks. After a period of four weeks, 82.6% of the patients in the ivermectin group showed marked improvement; only 44.44% of the patients in the lindance group showed a similar response. A side effects in the form of severe headache were noted in one patient in group A. Oral ivermectin is an easy drug to administer. It is given as a single oral dose, unlike lindane, which has to be applied topically. The compliance is accordingly increased. Moreover, ivermectin induces an early and effective improvement in signs and symptoms. Thus, it may be a better option for scabies than the traditional topical lindane lotion.
A Case of Acral Lentginous Melanoma: The Correlation between CD95L Expression on Melanoma Cells and Apoptosis of Tumor Infiltrating Lymphocytes
Authors: Maeda Akira , Aragane Yoshinori , Kawada Akira , Isogai Rieko , Orita Tomoaki , Tezuka Tadashi
Published: July 2014

There is an increasing amount of evidence that melanoma cells express the ligand for CD95 (CD95L), a potent inducer of apoptosis, which contributes to creating the immune privileged circumstances of tumor sites. However, it still remains to be demonstrated whether the capacity of melanoma cells to express CD95L is acquired during the progression. We addressed this question with a case of acral lentiginous melanoma by employing immunostaining using an antibody directed against CD95L as well as by in situ TUNEL staining. H&E‐staining of tumor specimens revealed that there were two different growth patterns. The central part of the tumor showed a deeper invasion into the dermis (Breslow thickness>4‐mm). The horizontally growing edge of the tumor proliferated more superficially (Breslow thickness<3‐mm). Relatively fewer lymphocytes were observed around the melanoma nests in central areas, which expressed detectable amounts of CD95L. In contrast, more lymphocytes were observed among the melanoma cells in the peripheral lesion, where CD95L was not detected. To evaluate the relevance of the CD95L expression, in situ TUNEL staining was performed. This indicated a significant correlation of lymphocyte apoptosis with CD95L expression on melanoma cells. Together, the data suggest that expression of CD95L is turned on depending on the level of melanoma, and that it may contribute to creating immune privileged circumstances by initiating apoptosis of tumor infiltrating lymphocytes.
Repigmentation after Autologous Miniature Punch Grafting in Segmental Vitiligo in North Indian Patients
Authors: Sarkar Rashmi , Mehta Swami Dass , Kanwar Amrinder J.
Published: July 2014

Segmental vitiligo is rather refractory to medical treatment. The paucity of literature on the results of autologous miniature punch grafting in segmental vitiligo in dark‐skinned patients led us to conduct such a study in 15 dark‐skinned patients of North Indian origin to observe the repigmentation response to this technique at different sites. The age range was 16–42 years (mean 23.08 years). There were four males and eleven females (M:F ratio being 1:2.75) who had the disease for 3–15 years. After autologous miniature punch grafting, a remarkable repigmentation was obtained in 12 (80%) cases with 90% to 100% improvement. About 40–60% repigmentation was observed within 3–6 months in the majority (66.6%) of cases and near total to total repigmentation was observed within 2 years. The maximum pigment spread was observed over the face (cheeks) and neck, and the minimum pigment spread was observed over the left arm and right upper eyelid. Complications were minimal. The present study demonstrates that autologous miniature punch grafting is a safe and easy technique, that produces good cosmetic results in the unsightly depigmenting lesions of segmental vitiligo, especially on the face and exposed areas, even in dark‐skinned races if patients are selected carefully. This can be of great benefit to patients who are psychologically disturbed by their disease.
The Occurrence of Various Collagen Diseases in One Family: A Sister with ISSc, PBC, APS, and SS and a Brother with Systemic Lupus Erythematosus
Authors: Tanaka Asayo , Igarashi Masaru , Kakinuma Miwa , Oh‐i Tsunao , Koga Michiyuki , Okuda Tomoki
Published: July 2014

We encountered siblings who had collagen diseases and related symptoms. Case 1 was a 53‐year‐old woman who had limited cutaneous systemic sclerosis (ISSc) associated with primary biliary cirrhosis (PBC), antiphospholipid antibody syndrome (APS), and subclinical Sjögren's syndrome (SS). Case 2 was a 48‐year‐old man, her younger brother, with systemic lupus erythematosus (SLE) that developed at 32 years of age. Investigation of their family revealed that their mother had Raynaud's phenomenon, arthritis, and subclinical Sjögren's syndrome, and that another younger brother of Cases 1 and 2 had Raynaud's phenomenon and general fatigue. HLA analysis revealed that the sister and brother had some identical HLA antigens in common, including A2, A33 (19), B67, B44 (12), Cw7, DR2, DR6, DR52, and DQ1. The sister, brother and their mother had common HLA antigens including A2, B67, Cw7, DR2, and DQ1. Although Cases 1 and 2 shared the same HLA system, they presented different phenotypes of collagen disease.
Contact Leukoderma of the Scalp or an Unusual Variant of Vitiligo?
Authors: Verma Shikha , Kumar Bhushan
Published: July 2014

Contact leukoderma due to hair dyes is strongly suspected in patients presenting with depigmented patches sharply localized to the scalp. We describe three patients with a striking pattern of depigmentation, which stopped abruptly at the hair margins. However, no definite correlation with the use of hair dyes could be made in any of them. Our cases represent an unusual pattern of vitiligo of the scalp, which seems to have been previously attributed mainly to hair colorants. Careful examination of the lesions on the scalp may reveal more cases with this pattern.
Dermatological Research in the 21st Century: Our Fantastic Future
Authors: Katz Stephen I.
Published: July 2014

During the second half of the twentieth century, dermatology came of age. Just as clinical dermatology, through new diagnostic and therapeutic modalities, matured to a point where the dermatologist could affect peoples' lives profoundly, so too did dermatological research enhance our understanding of skin diseases enormously. Dermatology should not be viewed as a scientific discipline‐advances come from fundamental scientific areas such as cell and molecular biology, biochemistry, immunology, microbiology, technology, the clinical sciences and others. Thus, dermatology and skin biology live within a universe of science, only a small part of which is dermatology and skin biology, and our patients and our science are dependent on integration and interdigitation with the universe of science for future success. In this lecture I will elaborate on where I think the next 10–20 years will take us in this universe.
Great Cases from the University of Pennsylvania
Authors: James William D.
Published: July 2014

New skin diseases are reported yearly. Some well‐known conditions may develop unusual manifestations. Other cutaneous findings are simply rare. While patients with such diseases represent only a small fraction of our practice they are an important subset of our experience. Many of these diseases are treatable, pinpoint the discovery of internal disease, or simply allow for a diagnosis and prognosis to be given to a concerned patient who had previously escaped classification. Such cases expand our knowledge and provide excitement to our day. Several patients from whom I have learned the most in the last few years are detailed for your enjoyment.
Imported Skin Diseases in Dermatology
Authors: James William D.
Published: July 2014

Millions of afflicted people suffer from conditions which Japanese dermatologists may rarely encounter. Many of our patients travel extensively either as part of work‐related business trips or during vacationing. From three to ten percent of travelers experience skin, hair or nail disorders. With worldwide travel heightening exposure to the causative agents, there is an increasing likelihood that a patient with leishmaniasis, Boutonneuse fever, onchocerciasis, loaiasis, dengue fever, cutaneous larva migrans or other recently acquired skin conditions from a far away land will visit your office for diagnosis and treatment. The clinical characteristics, diagnostic tests and therapeutic options for such imported tropical diseases will be discussed.
Azithromycin Monthly Pulse vs Daily Doxycycline in the Treatment of Acne Vulgaris
Authors: Parsad Davinder , Pandhi Roma , Nagpal R. , Negi K.S.
Published: July 2014

Acne vulgaris is a common skin disease seen primarily in adolescent and young adults. As the treatment involves long term therapy with antibiotics, an agent with a long half life can be very useful in increasing the compliance. To evaluate the role of a monthly dose of azithromycin and compare it with daily doxycycline, we conducted this randomized comparative study. Sixty patients with moderate to severe acne were randomly assigned to two treatment groups, A & B. Patients in group A received 100 mg doxycycline daily in addition to topical 0.05% tretinoin cream, whereas patients in group B were given 500 mg azithromycin once a day for four days per month along with 0.05% topical tretinoin for a total of 12 weeks. Of the 60 patients, 22 in group A and 28 in group B were evaluated. The monthly dose of azithromycin was found to be as effective as daily doxycycline on a pure protocol basis and statistically significantly better than doxycycline by intention to treat analysis.
Leukocytoclastic Vasculitis with IgA Deposits in Angioimmunoblastic T Cell Lymphoma
Authors: Sugaya Makoto , Nakamura Koichiro , Asahina Akihiko , Tamaki Kunihiko
Published: July 2014

Angioimmunoblastic T cell lymphoma (AILD) is a type of peripheral T cell lymphoma associated with fever and generalized lymphadenopathy. Cutaneous manifestations are seen in approximately 40% of the patients. We report herein a Japanese male patient with AILD associated with generalized purpura. The histology of the purpura included leukocytoclastic vasculitis with IgA deposits, which is rare in this disease. Using in situ hybridization and PCR methods, we showed that the involved lymph node was positive for Epstein‐Barr virus and that the purpura was negative.
An Association of Twenty‐Nail Dystrophy with Vitiligo
Authors: Khandpur Sujay , Reddy Belum Siva Nagi
Published: July 2014

A rare association of twenty‐nail dystrophy with segmental vitiligo is described in two patients. Vitiligo preceded the nail dystrophy. In both cases, all twenty nails were uniformly affected with the nail plates showing longitudinal striations and loss of luster. Longitudinal nail biopsy revealed a histological picture suggestive of eczematous changes and lichen planus respectively. Intramatrix injections of triamcinolone acetonide into the proximal and lateral nail folds were administered with considerable improvement.
Bullous Lichen Planus Arising in the Skin Graft Donor Site of a Psoriatic Patient
Authors: İnalöz H. Serhat , Patel G. , Holt P.J.A.
Published: July 2014

The coexistence of psoriasis vulgaris and bullous disorders, particularly bullous pemphigoid, has been described previously. We present an unusual case of bullous lichen planus arising in the skin graft donor site of a psoriatic patient. To our knowledge, such an association has not been reported to date.
Pityriasis Rotunda Mimicking Tinea Cruris/Corporis and Erythrasma in an Indian Patient
Authors: Gupta Somesh
Published: July 2014

Pityriasis rotunda is a rare disease characterized by perfectly round to oval, sharply defined, scaly, hypo/hyperpigmented patches of variable number and size located mainly over the trunk and proximal extremities. More than 95% of the reported cases in medical literature are from three countries/ethnic populations, namely Japan, South Africa (Bantu), and Italy (Sardinian islanders). To the best of my knowledge, no patient with the characteristic clinico‐pathologic features has been reported from the Indian subcontinent. I report a 44‐year‐old man with eighteen pityriasis rotunda patches, persistent for nearly 20 years. The lesions in the groin and axillae closely resembled erythrasma and tinea, and he had received treatment for these conditions several times in the past. Histopathology of the skin biopsy showed thinning of the epidermis with a thinned‐out granular layer and a sparse lymphomononuclear infiltrate in the dermis. A review of literature suggests that there are two subsets of the disease. The type I subset is comprised of pityriasis rotunda associated with systemic illness and is seen in Black or Oriental patients with no family history of the disease. The lesions tend to subside on treatment of the underlying illness. The type II subset patients are Caucasians as well as Blacks and Orientals with no underlying systemic illness. Familial occurrence is possible; lesions tend to be persistent and unresponsive to therapy.
Laugier‐Hunziker Syndrome
Authors: Kanwar Amrinder J. , Kaur Sukhjot , Kaur Charandeep , Thami Gurvinder P.
Published: July 2014

Laugier‐Hunziker syndrome is a benign pigmentary disorder which manifests as macular hyperpigmentation of the lips and buccal mucosa. Some patients have longitudinal pigmented bands of nails. The syndrome has no systemic associations. Two patients of this rare syndrome are reported. Disorders producing similar pigmentary changes which must be differentiated are discussed.
Linear and Whorled Nevoid Hypermelanosis
Authors: Mendiratta Vibhu , Sharma R.C. , Arya Latika , Sardana Kabir
Published: July 2014

Linear and whorled nevoid hypermelanosis (LWNHM) is a reticulate pigmentary disorder with a sporadic occurrence, representing genetic mosaicism. It is characterised by hyperpigmented macules in a reticulate pattern along Blaschko's lines, sparing the mucous membranes and stabilising after one to two years. It may be associated with various neurological abnormalities. The disorder may resemble incontinentia pigmenti, epidermal nevus, or zebra‐like hyperpigmentation clinically. We report LWNHM in a 15‐year‐old girl with progressively increasing streaks of reticulate hyperpigmented macules arranged in a whorled pattern over the trunk and extremities, which appeared soon after birth. There was no history of any preceding eruption or any associated systemic abnormality. Histopathological examination revealed basal cell hyperpigmentation without any pigmentary incontinence. CT scan of the brain was normal.
Distribution Density of Intraepidermal Nerve Fibers in Normal Human Skin
Authors: Kawakami Tetsuji , Ishihara Masahiko , Mihara Motoyuki
Published: July 2014

A total of 74 specimens was obtained from the normal human skin of patients from 3 to 90 years old. The specimens were roughly classified into 5 groups: 15 for the face group from the face; 15 for the abdomen group from the abdomen; 13 for the back group from the back; 14 for the arm group from the upper arm and forearm; and 17 for the leg group from the thigh and lower leg. They were all fixed in 4% paraformaldehyde and 14% saturated picric acid. Cryostat sections were examined by the immunoperoxidase method and indirect immunofluorescence (IF). Primary antibodies against neurofilament, neuron‐specific enolase, protein gene product 9.5 (PGP 9.5) and S‐100 protein were used. The most effective method was found to be the combination of IF with PGP 9.5; it visualized the intraepidermal nerve fibers easily and clearly. Of the 74 specimens, 32 (43%) had intraepidermal PGP 9.5‐immunoreactive (or nerve) fibers (IPIF), and 42 (57%) did not have any. With reference to the different skin locations, the maximal rate of specimens having IPIF was 57% in the arm group, and the minimum was 23% in the back group. IPIF positive specimens had approximate surface lengths of 6 mm, in which the existence number of the IPIF was 1 to 75. Their distribution density per 1000 epidermal basal cells was highest at 9.63 in the arm group and lowest at 2.89 in the back group. Their thickness was 2.94 ± 0.83 μm with no significant differences among the five groups. We concluded that intraepidermal nerve fibers may not be distributed evenly in the hairy portions of normal human skin, but they may be present focally. Physiologically, two‐point discrimination of itch may be explained by the distribution mode of intraepidermal nerve fibers.
Grouping Prurigo: Report of Cases
Authors: Sakurai Yoko , Matsui Haruka , Izaki Seiichi , Kitamura Keijiro
Published: July 2014

Grouping prurigo was reported as a new clinical entity by Ofuji et al. in 1977 and 1988. No further cases have been reported since. Herein we report four cases of grouping prurigo and discuss the possible relationship between this condition and other diseases in the prurigo group, including chronic pruritic papular dermatitis in adult men, which was reported by Chang et al. in 1999. Since grouping prurigo and chronic pruritic papular dermatitis in adult men share approximately the same characteristics in both clinical and histopathological findings, we concluded that these two disorders may be the same entity, characterized by grouped pruritic solid papules on the trunk.
A Case of Lichenoid Purpura Possibly Caused by Diltiazem Hydrochloride
Authors: Inui Shigeki , Itami Satoshi , Yoshikawa Kunihiko
Published: July 2014

A 65‐year‐old Japanese man presented with itchy purpuric lichenoid papules of six months' duration on his legs and buttock. A topical glucocorticoid ointment was not effective. The biopsy specimen histologically showed a dense lichenoid infiltration of lymphocytes and histiocytes into the dermoepidermal junction; red blood cells were seen in the infiltration. Based on the clinical and pathological findings, we diagnosed this case as lichenoid purpura of Gougerot‐Blum. The lesions disappeared when the diltiazem hydrochloride that he had been taking was discontinued.
Pedunculated Basal Cell Epithelioma Which Is Not Pinkus Tumor
Authors: Morita Kazumasa , Ikoma Akihiko , Takagaki Kenji
Published: July 2014

A 33‐year‐old female with pedunculated basal cell epithelioma was reported. She had noticed a cutaneous tumor on the scalp for two years before admission. It developed gradually and clinically resembled fibroma or pigmented nevus. Total resection was performed, and its histopathology revealed the solid or cystic type of basal cell epithelioma.
Elevated Leukaemia Inhibitory Factor (LIF) Expression in Lesional Psoriatic Skin: Correlation with Interleukin (IL)‐8 Expression
Authors: Szepietowski Jacek , Walker Craig , Hunter John A.A. , McKenzie Roderick C.
Published: July 2014

Psoriasis is a disease marked by keratinocyte hyperproliferation, neutrophil and lymphocyte infiltration, and aberrant epidermal and dermal expression of cytokines. Previously, it has been shown that LIF appears to be involved in skin inflammation and can induce the expression of IL‐8. We sought to determine whether expression of LIF is abnormal in lesional psoriatic skin and whether this correlates with the expression of IL‐8. Using reverse transcription polymerase chain reaction, we measured the expression of LIF and IL‐8 mRNA in biopsies from normal individuals, non‐lesional psoriatic skin, and lesional psoriatic skin. No difference was seen between the expression of IL‐8 and LIF in normal and in non‐lesional psoriatic skin. However, LIF expression in lesional skin was increased 160% compared with normal biopsies or non‐lesional skin (p<0.001). Immunostaining of frozen sections showed that the expression of LIF protein was principally suprabasal and, in the majority of sections, concentrated mainly in the stratum corneum of the lesional skin, whereas it was mainly in the stratum spinosum of the normal/non‐lesional skin. IL‐8 mRNA expression did not differ between the non‐lesional and normal skin, but expression in the lesional skin was 17.6‐fold greater than in normal skin (p<0.001), and this expression was correlated with the increased LIF expression (r=0.67, p<0.001). Although a significant negative correlation was demonstrated between LIF mRNA expression and the duration of the last outbreak of the disease, no other correlations were found between levels of cytokine expression and a variety of parameters including PASI score. These data suggest a role for keratinocyte LIF in the psoriatic lesion and a link between LIF and IL‐8 expression.
Staphylococcal Scalded Skin Syndrome in a Healthy Adult
Authors: Oyake Shinichi , Oh‐i Tsunao , Koga Michiyuki
Published: July 2014

We report a case of staphylococcal scalded skin syndrome (SSSS) in a 65‐year‐old healthy woman. Fever, purulent conjunctivitis, and exfoliation of the skin in the gluteal region were noted. A scarlatiniform rash was observed on the body, and this erythema was followed by generalized desquamation. Staphylococcus aureus was isolated from her eye discharge, posterior nasopharynx, and the erosive surface of the skin. All the investigated strains produced exfoliative toxin B, but none produced toxic shock toxin‐1 (TSST‐1) or enterotoxin. The patient was treated with antibiotics and fluid supplementation, resulting in subsidence. This case is thought to have been caused by an abortive form of SSSS or a scarlatiniform variant, which is very rare in healthy adults.
Marie Unna Hypotrichosis in an Asian Family
Authors: Kim Hong Suk , Kim Soo‐Chan , Lee Won‐Soo
Published: July 2014

We report on two patients from one family with Marie Unna hypotrichosis who showed a diffuse hair defect which typically occurs as an isolated phenomenon. The ultrastructural hair findings consisted of longitudinal grooving of the hair shafts and a flattening in cross‐section. Characteristically, scalp hair had been lost from the scalp margins and a high frontal and nuchal hairline had been present at birth. Eyelashes were absent, and eyebrows, axillary, and pubic hair were very sparse. Other affected cases have been reported in Caucasian families; however, this is the first case in the English medical literature of a family from Asia affected by Marie Unna hypotrichosis.
Cutaneous Neuroendocrine (Merkel Cell) Carcinoma Occurred after Resection of Malignant Melanoma
Authors: Fang Xinzhi , Wang Zhenhua , Li Yiqun
Published: July 2014

An elderly man presented with a brown border nodule of the left upper lip that appeared to be clinically consistent with metastatic melanoma; he had previously undergone two surgical removals of melanoma in the right temporal region. Routine histologic examination was not sufficient for diagnosis, but Merkel cell carcinoma was diagnosed by immunohistochemical studies Neuroendocrine carcinoma of viscera was excluded. Because this case could not be differentiated from amelanotic melanoma and malignant lymphoma based on histologic appearances, immunohistochemical staining was helpful for diagnosis and differentiation.
Alkaptonuria: A Case Report
Authors: Odabas Ali Riza , Karakuzu Ali , Selcuk Yilmaz , Erdem Teoman , Cetinkaya Ramazan
Published: July 2014

Alkaptonuria is a rare, autosomally recessive, metabolic disorder caused by a deficiency in homogentisic acid oxidase. It results in accumulation and deposition of homogentisic acid in cartilage, eyelids, forehead, cheeks, axillae, genital regions, nail beds, buccal mucosa, larynx, tympanic eardrum, and the tendons. We report a 33‐year‐old woman who presented with alkaptonuria and ochronotic pigment deposited in articular cartilage and cartilage of the ear and sclera.
Churg‐Strauss Syndrome Involving Medium‐sized Arteries
Authors: Inui Shigeki , Itami Satoshi , Iwai Chika , Yoshikawa Kunihiko
Published: July 2014

A 69‐year‐old Japanese man suffered from bronchial asthma, atrial fibrillation, general fatigue, high fever, and weight loss of about 5 kg within a month. He also had intermittent claudication, a tingling feeling in his fingers and toes, and an ulcer on his toe. His laboratory data revealed leukocytosis with absolute eosinophilia. The patient was treated with predonisolone 30 mg daily. Although the ulcers healed once, the lesions recurred with tapering predonisolone. The patient visited us because of the ulcer on his toe. Physical examination showed a 2 cm ulcer surrounded by slight erythema on his right fourth toe. Magnetic resonance angiography detected tapering stenosis of the medium‐sized arteries in both legs. A biopsy from his myocardium showed the infiltration of eosinophils into the myocardium. The neuron conduction rate of his lower leg was slower than that of the normal control, demonstrating mononeuritis. From these findings, we diagnosed this patient as Churg‐Strauss syndrome.
The Effects of Famciclovir and Epidural Block in the Treatment of Herpes Zoster
Authors: Ahn Hyung Jin , Lim Hyun Kyo , Lee Young Bok , Hwang Sang Min , Lee Won‐Soo , Ahn Sung Ku , Choi Eung Ho
Published: July 2014

In our previous study, we concluded that an epidural blockade combined with intravenous acyclovir is very effective in treating the acute pain in herpes zoster and postherpetic neuralgia. We evaluated the efficacy of oral famciclovir and epidural blockade on the pain of herpes zoster, compared to acyclovir administered intravenously and epidural blockade. For this purpose, we examined a new group treated with famciclovir and epidural blockade to compare with the group treated with acyclovir and epidural blockade in our previously study. The changes in the intensity of pain, the number of days required for relief of pain, and the total duration of pain were checked. We compared the days required for relief of pain (DRP) and the total duration of pain (TDP) of this group with those of the previous studied group treated with acyclovir and epidural blockade. DRP was significantly less, but TDP was similar. DRP and TDP were significantly lower, if the patients were treated within 7 days of symptom onset. The patients had a shorter DRP regardless of pain type than the previously studied group treated with acycolvir and epidural blockade. For the severe and moderate pain grades, there was a shorter DRP from 100 to 10. TDP was not significantly different for the groups regardless of pain type or grade. We believe that famciclovir and epidural blockade are very effective in treating the pain of herpes zoster, with a view to shortening the period of acute pain, providing similar effects on the prevention of postherpetic neuralgia, and being convenient to administer, compared to intravenous acyclovir and epidural blockade in our previous study.
Complex Ocular Choristomas in Linear Nevus Sebaceus Syndrome: A Report of Two Cases
Authors: Singal Archana , Dhaliwal Upreet , Bhattacharya Sambit Nath , Rohatgi Jolly , Singh Navjeevan
Published: July 2014

Two cases of linear nevus sebaceus syndrome (LNSS) are described in which ipsilateral facial nevus of Jadassohn was associated with complex ocular choristoma. One patient also had scleral osteomas, a rare occurrence in LNSS.
Lipid Analysis of Peripheral Blood Monocytes in Psoriatic Patients Using Fourier‐Transform Infrared Microspectroscopy
Authors: Takeda Hidemi , Okubo Yukari , Koga Michiyuki , Aizawa Katsuo
Published: July 2014

In psoriasis vulgaris, there are immunological abnormalities of T cells and monocytes. We previously demonstrated that monocytes in the peripheral blood of patients with psoriasis vulgaris are activated and produce an excess of inflammatory cytokines. It has long been suggested that fat metabolism is impaired in patients with this illness. In addition, it has been reported that macrophages activated by engulfing low density lipoprotein (LDL) immune complexes release large quantities of tumor necrosis factor (TNF)‐α and interleukin (IL)‐1β. Hence we hypothesized that the monocytes of psoriatic patients are activated by engulfing lipids and overproduce inflammatory cytokines. Therefore we measured both the serum and monocyte levels of lipids in the peripheral blood of psoriatic patients. At the same time, we calculated the psoriasis area and severity index (PASI) scores and analyzed their correlations with the lipid kinetics. The results showed that the serum cholesterol ester level and the cholesterol ester level in monocytes of psoriatic patients were significantly higher than those in healthy individuals. However, the cholesterol ester level in monocytes of patients with hyperlipidemia was also high, and there was no correlation between cholesterol ester level in monocytes of psoriatics and PASI scores. The cholesterol ester level in the monocytes of psoriatic patients was high, but this does not seem to play an important role in the pathogenesis of psoriasis.
A Case of Childhood Generalized Pustular Psoriasis Treated with Dapsone
Authors: Yu Hee‐Joon , Park Jin‐Woo , Park Jong‐Min , Hwang Dong‐Kyu , Park Yong‐Wook
Published: July 2014

Acute generalized pustular psoriasis (GPP) is a rare, sometimes fatal, exudative form of psoriasis characterized by acute febrile pustular psoriasis. Unlike the adult forms, GPP in childhood tends to undergo a more benign course. It may resolve spontaneously with only supportive treatment, but may sometimes be life threatening and resistant to therapy. We report a case of GPP in 7‐year‐old girl treated successfully with dapsone.
Sexual Habits and Clinico‐Etiological Profile of Sexually Transmitted Diseases in Nepal
Authors: Garg Vijay Kumar , Agarwalla Arun , Agrawal Sudha , Deb Manorama , Khanal Basudha
Published: July 2014

A total of 100 patients giving histories suggestive of sexually transmitted disease (STDs) and attending the dermatology OPD at BPKIHS (B.P. Koirala Institute of Health Sciences) in Nepal over a period of one year from 1st July 1999 to 30th June 2000 were included in this study. Out of 10,400 new dermatology cases, a total of 100 cases (0.96%) of STDs were seen. Of these 73 were males, and the majority (53%) were in the age group of 21–30 years. Most of the patients were from Sunsari district (54%). The most common profession (21%) was businessman followed by housewife (19%). The most common age for first sexual contact was 16 to 19 years (45%). Eleven percent had their first sexual contact at the age of 15 or below 15 years. Premarital sexual exposure was recorded in 17% of the unmarried males. There were only 3 homosexuals and 1 bisexual patient. Multiple contacts were recorded in 55% of the patients. The most common source of contact was a commercial sex worker in 34.15%. Condoms were always used by only 7%. The various types of sexually trasmitted diseases (STDs) were syphilis (31%) followed by condylomata acuminata (16%), herpes genitalis (15%), gonorrhoea (9%), and mixed infections (12%). Despite their varied sexual behavior, none of the patients were HIV positive; however, it is always advisable to screen all STD patients for HIV antibody.
A Case of Kaposi's Sarcoma Associated with Pemphigoid Nodularis
Authors: Gao Xing‐Hua , Lin Junping , Yang Ciying , Ma Lei , Wang Guanqing , Wang Yakun , Chen Hong‐Duo
Published: July 2014

Pemphigoid nodularis is a rare variant of bullous pemphigoid. Bullous pemphigoid complicated by internal malignancy has been well documented. There have been only a few reported cases of bullous pemphigoid associated with Kaposi's sarcoma. We described a case of pemphigoid nodularis who later developed Kaposi's sarcoma that might be related to immunosupressive therapy, a situation that has not been reported previously.
A Possible Mechanism of Mast Cell Proliferation in Mastocytosis
Authors: Kambe Naotomo , Miyachi Yoshiki
Published: July 2014

The abnormality in mastocytosis is the excessive accumulation of mast cells in the affected tissue. The growth and differentiation of human mast cells are quite dependent on stem cell factor (SCF), the ligand for the protein products of c‐kit. Recent studies have demonstrated that all adult patients examined so far carry c‐kit point mutations, leading to SCF‐independent autophosphorylation of the receptor and autonomous cell growth. On the other hand, typical pediatric patients have been found to bear no activating Asp816Val mutation in c‐kit. Although most mastocytosis patients are children, the mechanism by which mast cells proliferate in these pediatric patients remains unclear. Recently, we reported that human mast cells obtained from adult skin could dramatically proliferate when cultured with SCF. From these experimental results, it is speculated that local excessive production of SCF results in the mast cell proliferation in pediatric patients.
Minocycline Effectively Reduces Acid Producted by Propionibacterium granulosum
Authors: Higaki Shuichi , Kitagawa Taro , Morohashi Masaaki , Yamagishi Takayoshi
Published: July 2014

We studied the effects of minocycline on Propionibacterium granulosum. P. granulosum lipase activity was detected from acne lesions. Production of propionic and butyric acids by P. granulosum was well suppressed by all tested media with added minocycline; the higher the concentration of minocycline in the medium, the less of these acids was produced. It appeared that the decrease in these acids due to minocycline was greater in P. granulosum than in P. acnes. Although the influence of P. granulosum on acne lesions might be feebler than that of P. acnes, we should not neglect its presence. More research is needed to elucidate the relationship between the two species in acne lesions.
Rising Incidence of Genital Herpes over Two Decades in a Sexually Transmitted Disease Clinic in North India
Authors: Kumar Bhushan , Sahoo Bijaylaxmi , Gupta Somesh , Jain Rajesh
Published: July 2014

Genital herpes, which was considered to be a minor sexually transmitted disease (STD) in the past in developing countries, is rapidly increasing; in contrast, bacterial STDs are declining. This changing trend of various STDs prompted us to analyze our data retrospectively to see whether a similar change is occurring in this part of India as well. The records of sexually transmitted diseases (STDs) clinic attendees from January 1977 to December 2000 were analyzed. The demographic profile of patients with genital herpes was also considered for analysis. STDs were diagnosed clinically and by appropriate laboratory tests wherever applicable. VDRL test was done in all patients, and HIV antibody detection was performed from 1987 onwards. The incidences of chancroid, donovanosis, and gonorrhea were 12.2%, 6.3%, and 16.9%, respectively, from January 1977 to December 1985. The figures for the same decreased to 2.5%, 0.9%, and 2.3%, respectively, from January 1993 to December 2000. The decreasing incidence of the above bacterial STDs is statistically significant (p<0.001). However, there was an approximately two‐fold increase in the incidence of genital herpes in recent years (20.5%) in comparison to the figures from the late 70s (11.4%). Molluscum contagiosum also showed an upward trend (1% in 1977–85 vs. 9.8% in 1993–2000). Condylomata accuminata remained almost unchanged (21.4% in 1977–85 vs. 20% in 1993–2000). To conclude, a significant increase in the number of viral STDs and a decline in the bacterial diseases were observed in recent years in comparison to the figures from the late 70s. This may be due to awareness of HIV, success of control programs, syndromic management of STDs, and adoption of safer sexual practices, which prevent bacterial STDs more efficiently than viral ones.
A Case of Eruptive Collagenoma Localized on the Neck and Shoulders
Authors: Amaya Misato , Okubo Yukari , Koga Michiyuki
Published: July 2014

A 78‐year‐old woman, who had first noticed asymptomatic eruptions on her neck and shoulders eight years earlier, presented with papules and nodules 2 to 20 mm in diameter that had a normal to white hue and were flatly elevated. These lesions were scattered and multiple, some forming confluent plaques. Histopathologically, the epidermis was slightly atrophied, and collagen fibers in the dermis were coarse and proliferated. In addition, the number of elastic fibers was slightly decreased. No complications were evident. Based on these findings, the patient was given a diagnosis of mild eruptive collagenoma, a type of connective tissue nevus according to the classification of Uitto. This case is unique in that onset was at an advanced age and that distribution was localized on the neck and shoulders.
Pyoderma Gangrenosum Associated with Paroxysmal Nocturnal Hemoglobulinuria and Monoclonal Gammopathy
Authors: Matsubara Kimiko , Isoda Ken‐ichi , Maeda Yoshitami , Mizutani Hitoshi
Published: July 2014

Pyoderma gangrenosum developed in a man with a five‐year history of paroxysmal nocturnal hemoglobinuria and monoclonal gammopathy. He had multiple walnut sized ulcers on his back and extremities, plasma IgM‐k type M‐protein and low erythrocytic CD55 expression. This is an extremely rare association. However, clonal expansion of plasma cells and chimeric expression of hematopoietic cell glycosylphosphatidylinositol (GPI)‐anchored proteins may represent somatic mutations of hematopoietic stem cells in PG as well as PNH. PNH is based on abnormalities in the GPI‐anchor formation on various hematopoietic and non‐hematopoietic cells. Since the GPI‐anchored proteins have pleiotropic functions in complement mediated cell lysis, leukocyte motility, and coagulation systems, the present case may indicate the possible involvement of a GPI‐anchored protein abnormality in the pathogenesis of PG.
Gamma Interferon Directly Inhibits the Growth of Neurofibroma Cells In vitro
Authors: Nakayama Juichiro , Terao Hiroshi
Published: July 2014

Various neurofibroma cell lines isolated from either dermal, plexiform, or diffuse neurofibromas were found to respond to human gamma interferon by decreasing proliferation rates in vitro. The cell number decreased to around 40–50% of controls (without gamma interferon) six days after the treatment. The cell lines showed dramatic inhibition of tritium‐labeled thymidine uptake one day after the treatment with gamma interferon. Either 100 IU/ml or 1000 IU/ml of gamma interferon resulted in the same range of inhibition. It is calculated that venous infusion of one vial of commercial recombinant gamma interferon (200 × 106 IU/ml) reaches more than 100 IU/ml in the peripheral blood, which means that it may be clinically useful. The cell lines also responded to gamma interferon by initiating expression of CD54. Immunological modulation of neurofibroma cell components by gamma interferon in vivo remains to be studied.