bluuebunny/crossref_metadata_2025 huggingface dataset:
The complex nature of informal care in home‐based heart failure management
Authors: Clark Alexander M. , Reid Margaret E. , Morrison Caroline E. , Capewell Simon , Murdoch David L. , McMurray John J.
Published: August 2010

Title. The complex nature of informal care in home‐based heart failure management.

Aim. This paper is a report of a study to examine the complexities of informal caregiving for people with chronic heart failure.

Background. Little is known of the activities involved and underlying informal care. Heart failure is a common and burdensome condition in which carers play an important management role.

Method. Semi‐structured interviews were carried out with 30 informal carers nominated by patients with mild‐to‐moderate heart failure (24 spouses, four children, one sibling and one neighbour). Interviews examined knowledge of heart failure, its effects, reported management practices and concerns, decision making and support. The data were collected in 2001.

Findings. The management of heart failure was a shared and ongoing responsibility between the carer and patient. Carers’ clinical knowledge of the condition and management was often limited, but they developed extensive knowledge of its personal effects on the patient. Invisible care activities included monitoring signs of symptom exacerbation and energy boundaries against perceived current and future demands and priorities. Visible care activities included medication management, dressing, bathing and help‐seeking. Carers responded to patients’ capacities, and adopted philosophies that sought to foster independence while facilitating as normal a life for the patient as was possible and safe.

Conclusion. Interventions for informal carers around effective chronic heart failure management should address both visible and invisible informal caring. Future research is needed to develop interventions with carers to improve quality of care, reduce costs and improve patient quality of life. More research is needed to explore the complexities of lay caregiving and to explore the invisible dimensions of informal care further.
Diagnostic delay in lung cancer: a qualitative study
Authors: Tod Angela M. , Craven Jacqueline , Allmark Peter
Published: August 2010

Title.  Diagnostic delay in lung cancer: a qualitative study

Aim.  This paper is a report of a study to identify factors influencing delay in reporting symptoms of lung cancer.

Background.  Lung cancer accounts for approximately 5% of deaths in the Western world. For up to 80% of patients, their disease is inoperable because it has been diagnosed too late. This suggests that reducing diagnostic delay could reduce mortality.

Methods.  This qualitative study was conducted from July 2005 to May 2006 in community and hospital settings in the United Kingdom. A purposive sample of people diagnosed with lung cancer in the previous 6 months (n = 18), and two 18‐month survivors took part in individual interviews.

Findings.  Participants reported a range of interrelating factors that influenced delay in reporting symptoms of lung cancer. Barriers to symptom reporting included symptom experience, lack of knowledge and fear. Blame and stigma because of smoking were also prevalent influences, as well as cultural factors, non‐standard patterns of healthcare utilization and underlying stoical attitudes. The only factor to emerge as helpful in overcoming delay was the role families played.

Conclusion.  Lack of knowledge and awareness about lung cancer could be addressed by better education of the public. Social marketing is a way of developing education messages which tackle cultural influences on treatment‐seeking delay. Nurses have a potential role in developing and disseminating those messages.
Using evolutionary demography to link life history theory, quantitative genetics and population ecology
Authors: Coulson Tim , Tuljapurkar Shripad , Childs Dylan Z.
Published: August 2010

1. There is a growing number of empirical reports of environmental change simultaneously influencing population dynamics, life history and quantitative characters. We do not have a well‐developed understanding of links between the dynamics of these quantities.

2. Insight into the joint dynamics of populations, quantitative characters and life history can be gained by deriving a model that allows the calculation of fundamental quantities that underpin population ecology, evolutionary biology and life history. The parameterization and analysis of such a model for a specific system can be used to predict how a population will respond to environmental change.

3. Age‐stage‐structured models can be constructed from character‐demography associations that describe age‐specific relationships between the character and: (i) survival; (ii) fertility; (iii) ontogenetic development of the character among survivors; and (iv) the distribution of reproductive allocation.

4. These models can be used to calculate a wide range of useful biological quantities including population growth and structure; terms in the Price equation including selection differentials; estimates of biometric heritabilities; and life history descriptors including generation time. We showcase the method through parameterization of a model using data from a well‐studied population of Soay sheepOvis aries.

5. Perturbation analysis is used to investigate how the quantities listed in summary point 4 change as each parameter in each character‐demography function is altered.

6. A wide range of joint dynamics of life history, quantitative characters and population growth can be generated in response to changes in different character‐demography associations; we argue this explains the diversity of observations on the consequences of environmental change from studies of free‐living populations.

7. The approach we describe has the potential to explain within and between species patterns in quantitative characters, life history and population dynamics.
Density‐dependent impacts of exotic conifer invasion on grassland invertebrate assemblages
Authors: Pawson Stephen M. , McCarthy James K. , Ledgard Nicholas J. , Didham Raphael K.
Published: August 2010

1. Globally, temperate grasslands have been heavily modified by agricultural intensification. The ecological integrity of many remaining semi‐natural grasslands is further threatened by the encroachment of invasive alien woody plant species, such as conifers.

2. Although the ecological impact of conifers is often inferred to be directly related to increasing conifer density at invaded sites, interpretation of these density‐dependent effects on native biodiversity is almost always confounded by strong correlations between density and time since invasion. Here, we isolate the direct effects of exotic conifer density on native invertebrate assemblages from the confounding effects due to time since invasion, by establishing replicated experimental plantings of the invasive Pinus nigra (densities of 400, 800, 1600, 2500 and 3500 trees per ha, established in 1993) in New Zealand.

3. From the 29 549 invertebrates captured, the relative abundance of major classes and orders was largely unaffected by conifer invasion at densities below 800 trees per ha at age 14 years, but differed substantially in higher‐density conifer stands (canopy cover >50%). At the species level, beetle species composition was highly sensitive to conifer invasion at densities as low as 400 trees per ha at age 14 years. Changes in beetle species composition were correlated with reduced soil moisture, increases in canopy cover, and trap distance from the nearest semi‐natural grassland.

4. Synthesis and applications. The effects of exotic conifer invasion on grassland invertebrate assemblages were strongly dependent on conifer density. In our study, the negative effects of conifer invasion on biodiversity were comparatively low at densities below ≤800 trees per ha at 14 years after invasion. To conserve invertebrate diversity in semi‐natural grasslands we recommend a dual focus of conservation actions targeting: (i) immediate control, or thinning, of high density conifer infestations (>800 trees per ha) to restore habitat to a state suitable for grassland species, and (ii) longer‐term removal of sparse, pre‐coning infestations in order to prevent further spread. We propose the use of canopy cover as a proxy for the confounded relationship between conifer density and time since invasion.
Why some adults with intellectual disability consult their general practitioner more than others
Authors: Turk V. , Kerry S. , Corney R. , Rowlands G. , Khattran S.
Published: August 2010

Background  This research identifies factors affecting why some adults with intellectual disability (AWIDs) consult their general practitioner (GP) more than others. Little is known about these factors, despite AWIDs having higher health needs and reduced longevity. Current barriers to accessing health care need to be understood and overcome to achieve improved health outcomes.

Methods  A secondary analysis of data obtained from a stratified randomised sample of AWIDs participating in a cluster randomised trial of hand held health records. The number of GP consultations was obtained retrospectively for the year preceding initial health interviews from GP records. AWIDs and their carers were given separate health interviews using identical/adapted questions where possible.

Results  Two hundred and one AWIDs and or their carers from 40 practices participated (response rate 64.6%) with GP consultation data extracted for 187 AWIDs. Overall consulting levels were low, 3.2 per annum for women and 2.2 for men. Increased age, gender (women) and type of carer (paid) were all significantly associated with increased consultations. Carers reporting health problems, medications reported by AWIDs, medications recorded in GP records, and pain reported by AWIDs were also significant factors affecting consultations to GP practices after adjustment for age and type of carer.

Conclusions  Overall consultation rates were lower than expected, and affected by age, gender and type of carer. Targeted interventions are needed to improve attendance and promote health.
Distinguishing features of autism in boys with fragile X syndrome
Authors: Brock M. , Hatton D.
Published: August 2010

Background  Males with fragile X syndrome and autism (FXS/autism) represent a distinct subgroup of males with FXS at risk for markedly poorer outcomes. Early identification and intervention can improve outcomes for males with autism spectrum disorder.

Method  To advance the development of a specialised autism screening tool for young males with FXS that could assist in early identification, backward regression was used to identify the combination of parent‐report questionnaire items that best predicted autism symptoms in a sample of 60 males with FXS, ages 4–18 years old.

Results  Both social and repetitive behaviours distinguished males with FXS/autism, with repetitive behaviours playing a more prominent role than previously documented in the literature.

Conclusions  Healthcare workers and early interventionists may be able to interview parents about a few key behaviours to determine if young child with FXS should be formally evaluated for autism. Evidence‐based practices identified for children with autism spectrum disorder can be implemented as early as possible.
The development of adaptive skills in young people with Down syndrome
Authors: Van Duijn G. , Dijkxhoorn Y. , Scholte E. M. , Van Berckelaer‐Onnes I. A.
Published: August 2010

Background  To help children with Down syndrome reach optimum levels of adaptive behaviour, caretakers need to know how and to what extent children with Down syndrome acquire adaptive skills.

Method  The adaptive levels of motor, daily living, communicative and social behavioural skills were determined in a group of 984 Dutch children with Down syndrome, aged between 0 and 12 years, and compared with the adaptive levels of typically developing children using a Dutch version of the Vineland Screener.

Results  Children with Down syndrome acquire their adaptive skills at a slower pace and reach their ceiling scores at about the age of 12 years, at a substantially lower level than a reference group of typically developing children.

Conclusions  Down children seem to acquire skills in a similar sequence and according to a similar trajectory. Development of adaptive skills varies greatly between participants with Down syndrome. For that reason, cohort studies on the development of individuals with Down syndrome over a prolonged period of time are needed.
Effects of gut microbiota on obesity and atherosclerosis via modulation of inflammation and lipid metabolism
Authors: Caesar R. , Fåk F. , Bäckhed F.
Published: August 2010

Abstract.  Caesar R, Fåk F, Bäckhed F (Department of Molecular and Clinical Medicine, Sahlgrenska Center for Cardiovascular and Metabolic Research/Wallenberg Laboratory, University of Gothenburg, Gothenburg, Sweden). Effects of gut microbiota on obesity and atherosclerosis via modulation of inflammation and lipid metabolism. (Review) J Intern Med 2010; 268: 320–328.

Recent studies have revealed a close relationship between inflammatory and metabolic pathways, and inflammation is now recognized to have a major role in obesity and metabolic diseases such as insulin resistance and atherosclerosis. The human body is home to a large number of distinct microbial communities, with the densest population in the distal gut (the gut microbiota). Bacteria have long been known to activate inflammatory pathways, and recent data demonstrate that the gut microbiota may affect lipid metabolism and function as an environmental factor that influences the development of obesity and related diseases. Here, we review how the gut microbiota may affect metabolic diseases by activating the innate immune system.
Insulin Secretory Granules Enter a Highly Calcium‐Sensitive State following Palmitate‐Induced Dissociation from Calcium Channels: A Theoretical Study
Authors: Pedersen M. G.
Published: August 2010

Impaired insulin secretion is a major contributor to diabetes. Obesity is a known risk factor for the development of diabetes, and prolonged exposure of pancreatic islets to lipids results in impaired insulin secretion. Insulin is released from pancreatic β‐cells as a result of Ca2+‐induced exocytosis. Recent experiments have shown that chronic palmitate exposure results in the loss of localised Ca2+‐influx and impaired exocytosis of insulin secretory granules in β‐cells. In the present study, the roles of Ca2+‐channel clustering disruption, and dissociation of granules from Ca2+‐channels, in the impaired exocytotic and secretory responses from palmitate‐treated β‐cells, are investigated using mathematical models of Ca2+ dynamics, granule pools, exocytosis and secretion. It is shown that either disruption of Ca2+‐channel clusters or dissociation of granules from Ca2+‐channels with a shift to a highly calcium‐sensitive pool can explain the recent experimental findings of palmitate‐induced defects of exocytosis and insulin secretion. On the basis of imaging results, it is argued that a shift to a highly calcium‐sensitive state after dissociation of granules from Ca2+‐channels is the most likely explanation for the experimental findings from β‐cells exposed chronically to palmitate.
Reversal of an epigenetic switch governing cell chaining in Bacillus subtilis by protein instability
Authors: Chai Yunrong , Kolter Roberto , Losick Richard
Published: August 2010

Bacillus subtilis forms long chains of cells during growth and biofilm formation. Cell separation is mediated by autolysins, whose genes are under the negative control of a heteromeric complex composed of the proteins SinR and SlrR. Formation of the SinR–SlrR complex is governed by a self‐reinforcing, double‐negative feedback loop in which SinR represses the gene for SlrR and SlrR, by forming the SinR–SlrR complex, titrates SinR and prevents it from repressing slrR. The loop is a bistable switch and exists in a SlrRLOW state in which autolysin genes are on, and a SlrRHIGH state in which autolysin genes are repressed by SinR–SlrR. Cells in the SlrRLOW state are driven into the SlrRHIGH state by SinI, an antirepressor that binds to and inhibits SinR. However, the mechanism by which cells in the SlrRHIGH state revert back to the SlrRLOW state is unknown. We report that SlrR is proteolytically unstable and present evidence that self‐cleavage via a LexA‐like autopeptidase and ClpC contribute to its degradation. Cells producing a self‐cleavage‐resistant mutant of SlrR exhibited more persistent chaining during growth and yielded biofilms with enhanced structural complexity. We propose that degradation of SlrR allows cells to switch from the SlrRHIGH to the SlrRLOW state.
The global nutritional regulator CodY is an essential protein in the human pathogen Streptococcus pneumoniae
Authors: Caymaris Stéphanie , Bootsma Hester J. , Martin Bernard , Hermans Peter W. M. , Prudhomme Marc , Claverys Jean‐Pierre
Published: August 2010

CodY is a global regulator highly conserved in low‐G+C Gram‐positive bacteria. It plays a key role in the adaptation of Bacillus subtilis to nutritional limitation through repression of a large gene set during exponential growth and relief of repression upon starvation. In several pathogenic bacteria, CodY regulates major virulence genes. Our interest in Streptococcus pneumoniae CodY originates from our observations that the oligopeptide permease Ami was involved in repression of competence for genetic transformation. We hypothesized that peptide uptake through Ami feeds amino acid pools, which are sensed by CodY to repress competence. As our initial attempts at inactivating codY failed, we launched an in‐depth analysis into the question of the essentiality of codY. We report that codY cannot be inactivated unless a complementing ectopic copy is present. We obtained genetic evidence that a recently published D39 codY knock‐out contains additional mutations allowing survival of codY mutant cells. Whole‐genome sequencing revealed mutations in fatC, which encodes a ferric iron permease, and amiC. This combination of mutations was confirmed to allow tolerance of codY inactivation. The amiC mutation is in itself sufficient to account for the strong derepression of competence development observed in D39 codY cells.
Requirement of a specific group of sphingolipid‐metabolizing enzyme for growth of yeast Saccharomyces cerevisiae under impaired metabolism of glycerophospholipids
Authors: Tani Motohiro , Kuge Osamu
Published: August 2010

Sphingolipids play critical roles in many physiologically important events in yeast Saccharomyces cerevisiae. In this study, we screened for yeast mutants showing high sensitivity to Aureobasidin A, an inhibitor of inositol phosphorylceramide synthase, and found that a lack of SAC1 encoding phosphoinositides phosphatase causes high sensitivity to the inhibitor. Double mutation analysis involving the SAC1 and non‐essential sphingolipid‐metabolizing enzyme genes revealed that csg1Δ, csg2Δ, ipt1Δ or scs7Δ causes synthetic lethality with deletion of SAC1. As previously reported, SAC1‐repressed cells exhibited a reduced cellular phosphatidylserine (PS) level, and overexpression of PSS1 encoding PS synthase complemented the growth defects of scs7Δ, csg1Δ and ipt1Δ cells under SAC1‐repressive conditions. Furthermore, repression of PSS1 expression resulted in synthetic growth defect with the deletion of CSG1, IPT1 or SCS7. The growth defects of scs7Δ, csg1Δ and ipt1Δ cells under SAC1‐ or PSS1‐repressive conditions were also complemented by overexpression of Arf‐GAP AGE1, which encodes a protein related to membrane trafficking. Under SAC1‐repressive conditions, scs7Δ, csg1Δ and ipt1Δ cells showed defects in vacuolar morphology, which were complemented by overexpression of each of PSS1 and AGE1. These results suggested that a specific group of sphingolipid‐metabolizing enzyme is required for yeast cell growth under impaired metabolism of glycerophospholipids.
Tinea Capitis
Authors: Sehgal V. N. , Saxena A. K. , Kumari Sudershan
Published: August 2010

ABSTRACT: A study of 50 patients with tinea capitis revealed that it affects school children of low socioeconomic status. Clinical diagnosis of grey patch, black dot, seborrheic, kerion, and pustular inflammatory varieties was made. In all, 37 cultures were positive for dermatophytes, wherein most were Trichophyton violaceum. Some grew T. rubrum, T. mentagropytes, and T. verrucosum. A correlation between the clinical variants and fungi was also attempted.
An Unusual Presentation of Histiocytosis X
Authors: Pareek S. S. , Hawass Nour‐El‐Din
Published: August 2010

A 20‐year‐old, single Saudi man was examined in the outpatient clinic in January 1980, complaning of ulcers in the groin, discharge from the right ear, and gum enlargement of 2 years' duration. Also in 1980, ulcerating lesions developed in both axillae. One year later the patient was admitted to the hospital for spontaneous peneumothorax, and in the same year a small nodule in the upper left eyelid and fissuring of the nails were noted. In 1982, the patient complained of perianal ulceration and rapidly enlarging submandibular swelling with multiple sinuses and ranula.
Onychomycosis Treated with Itraconazole or Griseofulvin Alone with and without a Topical Antimycotic or Keratolytic Agent
Authors: Arenas Roberto , Fernández Genoveva , Dornínguez Luciano
Published: August 2010

Abstract: An open, comparative, randomized study was conducted using griseofulvin or itraconazole for the treatment of onychomycosis of the foot. Group I (45 patients) received itraconazole and Group II (45 patients) received griseofulvin. Each group was divided into three subgroups that received different topical treatment: antimycotic cream (isoconazole 1%), keratolytic cream (urea 40%), or placebo cream. The itraconazole group showed complete clearance in combination with isoconazole cream in 73.3% (11 of 15 patients), in combination with keratolytic cream in 78.5% (11 of 14 patients), and in combination with placebo cream in 91.6% (11 of 12 patients). The griseofulvin group showed complete clearance in combination with isoconazol cream in 46.1% (7 of 15 patients), in combination with keratolytic cream in 42.8% (6 of 15 patients), and in combination with placebo cream in 26.6% (4 of 15 patients). The itraconazole group showed better results compared with the griseofulvin group when the chi‐square statistical method was used.
Naftifine Treatment of Resistant Dermatophytosis
Authors: Rosen Ted , Fischer Marie , Orengo Ida , Abuabara Fuad
Published: August 2010

Abstract: Despite an array of topical and systemic antifungal agents, cases of resistant dermatophytosis still exist. Use of naftifine, the first commercially available allylamine derivative, proved successful in the management of four such cases.
MAURICIO GOIHMAN‐YAHR, M.D., PhD., EDITOR
Authors: Byarlay Jean A.
Published: August 2010

The Correspondence Section serves as a forum for opinion exchange about subjects of general interest such as dermatologic training, relations between deratologists and pharmaceutical houses, governmental control of dermatology and medical practice in general, peculiarities of dermatology related to geographic, climatic, or racial factors, the flow of information and publications, as well as other concerns the readership might have. originality in highly desirable and repetitious contributions or minutiae are discouraged. We wish also to foster submission of hypotheses marked accordingly. These should be based on reality and should stimulate thought. Contributions should conform to the format for correspondence as outlined in, “Notice to Contributions.” Manuscripts will undergo standard editorial procedures. Submit all correspondance to Mauricio Goihman‐Yahr, M.D., Ph.D., Section Editor, Jet International M‐154, P.O. Box 020010, Miami, FL 33102.
Kimura's disease
Authors: Lenk Nurdan , Artüz Ferda , Kulaçoǧlu Sezer , Alli Nuran
Published: August 2010

A 40‐year‐old white Caucasian man presented with a 6‐month history of a 1.5 × 1.5 cm nodular lesion on his left arm. No history of trauma preceeded the lesion and the patient was in good general health.

Dermatologic examination revealed a solitary, red, painless nodule located on the left arm (Fig. 1). Histopathologic examination of punch biopsy revealed lymphoid follicles, some of which had active germinal centers in the deep dermis and subcutaneous tissue. Mixed infiltration of lymphocytes, plasma cells and many eosinophils were present in the interfollicular region. Infiltration of the germinal centers by eosinophils, causing partial destruction, was observed (Fig. 2A). There was also proliferation of thin‐walled small vessels and fibrosis in some parts of the interfollicular areas (Fig. 2B). Although Kimura's disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) were considered in the differential diagnosis, it was diagnosed as KD based on these histologic features. Further laboratory examinations were performed. Serum IgE was normal and peripheral eosinophilia was absent.

The lesion regressed after biopsy. Complete healing was achieved with intralesional corticosteroid (triamcinolone acetonide) treatment.
Solitary plexiform neurofibroma is not pathognomonic of von Recklinghausen's neurofibromatosis: a report of a case
Authors: Fisher David A. , Chu Paul , McCalmont Timothy
Published: August 2010

A 42‐year‐old Caucasian man presented with a 1.8‐cm nodule in the proximal thigh (Fig. 1) that had been slowly enlarging for approximately 5 years. The patient had no family history of neurofibromatosis. Physical examination revealed none of the other stigmata of this neurocutaneous disorder. D.A.F. performed an elliptical excision as an out‐patient procedure. Grossly, the lesion was homogeneously opalescent and gelatinous. The tissue was fixed in 10% formalin and embedded in paraffin. Routine hematoxylin and eosin stained sections were prepared. The most notable feature on low power magnification was a large, circumscribed, lobular proliferation of spindle cells in a loose fibrillar background that distorted much of the dermis and subcutaneous fat (Fig. 2). On high power magnification, a population of cells with ovoid and S‐shaped nuclei admixed within densely packed eosinophilic collagenous material was seen at the center of the lobule (Fig. 3A). A small amount of mucin was also present. Within this cellular area, a dense population of S‐shaped spindle cells with eosinophilic cytoplasm was arranged in a parallel array forming microfascicles. At the periphery of this cellular component, a similar population of cells was arranged against a loose fibrillar background of abundant mucin (confirmed by colloidal iron stain) and scattered mast cells (Fig. 4). Lining the lobule was a compressed rim of thin, delicate collagen bundles and perineurial cells. Pleomorphic nuclei and mitotic figures were not observed. The surrounding dermis and subcutaneous tissue were unremarkable. Thickened collagen bundles at the center of the lobule could be seen with a frichrome stain. An S‐100 protein immunoperoxidase stain highlighted scattered Schwann cells within the lobule (Fig. 3B), and nerve bundles adjacent to the neurofibroma served as infernal positive controls.
Polymorphic eruption of pregnancy
Authors: Tarocchi Simona , Carli Paolo , Caproni Marzia , Fabbri Paolo
Published: August 2010

A 29‐year‐old woman developed a pruritic papular eruption that appeared symmetrically on the lateral part of her arms and elbows and on the media part of her thighs and knees in the 35th week of gestation (Fig. 1). The papules were erythematous, firm, flat, with a slightly elevated border, and measured 2–5 mm in diameter. A few papules quickly coalesced to form urticarial plaques. There were no lesions on the abdomen. In the 40th week of gestation, she delivered a healthy girl and a week later the eruption cleared up spontaneously.

Two years later, in the 37th week of gestation of her second pregnancy, the same patient developed an itchy papular eruption, clinically comparable with that of the previous pregnancy, which spontaneously disappeared a few days after delivery of a healthy boy.

During both pregnancies routine laboratory investigations, hormonal tests, and tests to detect cholestasis and viral hepatitis (A,B,C) were performed. A biopsy of lesional and perilesional skin was taken for histologic and direct immunofluorescence (DIF) examination. Serum was tested by indirect Immunofluorescence (IIF) staining for IgG, IgM, and IgA, and by a complement fixation test. For histologic examination, biopsy specimens were fixed in 10% buffered formalin, embedded in paraffin, sectioned at 5 μm, and stained with hematoxylin and eosin. Direct immunofluorescence was performed on cryostat sections from biopsy specimens, washed in phosphate‐buffered saline (PBS) and incubated with fluorescein isothiocyanate (FITC) labeled antibodies (anti‐IgG, IgA, IgM, C3, fibrinogen) (DAKO, Glostrup, Denmark). The slides were mounted in buffered glycerol and examined by fluorescent microscopy.

An immunohistochemical study was also performed on cryostat sections using the alkaline phosphatase antialkaline phosphatase (APAAP) technique with monoclonal antibodies (anti‐CD45RO, CD3, CD4, CD8, CD25, CD22, CD1a, HLA‐DR, CD54).

The results of routine laboratory and hormonal tests and tests for cholestasis and viral hepatitis (A,B,C) were normal or negative. The histopathologic examination of a papule showed mild spongiosis in the epidermis, slight oedema, and a moderate perivascular infiltrate of lymphocytes and monocytes in the superficial dermis (Fig. 2). Examination by DIF on lesional skin showed a dense granular‐linear deposit of IgM and C3 along the basement membrane zone (BMZ) of the epidermis and hair follicles (Fig. 3). After healing of the dermatosis, repeated DIF near the previously examined location was negative. An IIF study revealed no circulating anti‐BMZ IgG, IgM, or IgA autoantibodies. The complement fixation test was also negative. The immunohistochemical study showed a perivascular infiltrate in the dermis consisting mainly of T lymphocytes (CD4+, DRH+, CD25+).
Congenital hemangiopericytoma of the skin
Authors: Ferreira Carlos M.M. , Maceira Juan M.P. , Coelho Janice M.CO.
Published: August 2010

A 5‐month‐old white boy had a pinhead red spot on his left malleolar region since birth. His mother noted progressive growth and, when he was first examined, the lesion measured 30x25x20 mm in size, had a pyramidal shape, covered by erythematous and smooth skin, with teleangiectasia. The tumor was soft on paipation and movable over the deeper structures (Fig. 1a). X‐ray examination revealed no bone abnormalities at that time.

Just before the programmed incisional biopsy, the patient had a febrile episode due to upper respiratory tract viral infection during which the tumor became ulcerated, bleeding and painful (Fig. 1b). As the bleeding was constant the lesion was surgically excised, at which time the whole gelatinous mass was easily detached from the aponeurotic structures (Fig. 1c). The tumor measured 36x31 x30 mm and had a whitish, smooth cut surface crossed by small blood vessels.

Histopathologic examination revealed a tumor mass extending from the mid dermis to the subcutaneous fat and composed of tightly packed fusiform cells in close opposition with lined vascular channels (Fig. 2a). There were atypical areas and mitotic figures were frequently seen. A reticulin preparation showed a dense network surrounding vessels and pericytes. The vessels branched in a staghorn configuration (Fig. 2b). The immunoperoxidase technique with monoclonal antibody anti‐Factor VIII was positive in the endothelial cells surrounded by tumor cells (Fig. 2c).

Seven months after surgery the patient returned with a painful subcutaneous mass on the upper third of his left leg that was adherent to the tibial bone. X‐ray examination disclosed osteolytic lesions at the tibial base and destruction of the peroneal head (Fig. 3).

The patient was submitted to radiotherapy, amputation of the left lower limb in the middle portion of the thigh and chemotherapy. He is clinically well 3 years after treatment.
Dermatomyositis and acquired ichthyosis as paraneoplastic manifestations of ovarian tumor
Authors: Roselino Ana Maria F. , Souza Cacilda S. , Andrade Jurandyr M. , Tone Luiz G. , Scares Fernando A. , Llorach‐Velludo Maria Angeles S. , Foss Norma T.
Published: August 2010

Case 1 A 37‐year‐old white woman was seen at the Dermatology Clinic presenting muscle weakness of the pelvic and scapular girdles, as well as a violaceous erythema and periorbital infiltration (heliotropium) and erythematous‐violaceous lesions on the dorsum of the finger joints, with periungual telangiectasia. A diagnosis of dermatomyositis was confirmed and treatment with prednisone, 1 mg/kg per day, was started, with improvement.

One year and two months later she presented ascites, bilateral pleural effusion, and a 7‐kg weight loss. Cytology of the ascitic fluid revealed cells compatible with adenocarclnoma. Exploratory laparotomy demonstrated peritoneal carcinomatosis, and an implant biopsy confirmed a moderately differentiated adenocarcinoma of the ovary of the endometriold type. The patient died 1 year and 4 months after the diagnosis of dermatomyositis (Fig. 1 and Table 1).

Case 2 An 8‐year‐old white girl was seen at the Dermatology Clinic presenting generalized ichthyotic skin of a brovi/nish color, perilabial and palmo‐plantar fissures of 5 months duration, and hair fall. For the last 2 months, she had been presenting daily fever (38°C) and lack of appetite, and had lost 7 kg.

Abdominal ultrasound revealed a large pelvic mass (9 × 8 × 7 cm) located in a small basin, with possible right renal invasion, as well as periaortic and pericaval adenomegaly, findings that were later confirmed by computerized tomography. Radiologic examinations of the thorax, skull, spine, and long bones were normal. Exploratory laparotomy confirmed the presence of a dysgerminoma of the right ovary as determined by biopsy (Fig. 2).

Chemotherapy was started (carboplatin, bleomycin, and vinblastine) and an abdominal ultrasound taken 1 5 days later showed a 67.5% reduction of the pelvic mass, as well as reduction of rectoperitoneal adenomegaly.
Isolated cutaneous Mycobacterium avium complex infection in AIDS
Authors: Alfandari Serge , Ajana Faiza , Senneville Eric , Delaporte Emmanuel , Chidiac Christian , Mouton Yves
Published: August 2010

A 38‐year‐old woman, known to be HIV‐antibody‐positive for 2 months, presented with a progressive swelling of the right thigh of 1‐month duration. She had no history of trauma or breaking of the skin. She suffered from oral candidiasis, but had no previous AIDSdefining illness. She had no systemic symptoms such as pulmonary symptoms, weight loss, organ enlargement, or fever. Her laboratory tests were unremarkable, except for a CD4 count of 115/mm3. On examinafion, an ulcerated, fluctuating 3‐cm mass with surrounding erythema was noted with only a small volume of purrulent discharge. A punch biopsy, showing a necrotizing inflammation of the hypodermis without granulomas, and a swab and two blood cultures (Isolator 10, DuPont, Wilmington, DE, USA) were performed. The patient was discharged with a diagnosis of a bacterial abscess and treated with pristinamycin, 1 g t.i.d., and local wound care. One month later, the ulceration persisted, but the inflammation and purulent discharge had disappeared. Because direct examination of a Ziehl‐Neelsen stained specimen had revealed acid‐fast bacilli, later identified as Mycobacterium avium complex, the therapy was changed to clarithromycin, 2 g b.l.d., given for 3 months, complete healing was obtained without incision and drainage. She was given rifabutine, 300 mg once daiiy, 7 months later as her CD4 cell count became <100/mm”, No relapse had occurred after a 18‐month follow‐up.
T‐cell lymphoma with remarkable muscle involvement
Authors: Saeki Hidehisa , Torii Hideshi , Ogata Fuyuki , Furue Masutaka , Nakagawa Hidemi , Ishibashi Yasumasa
Published: August 2010

A 31‐year‐old Japanese man presented to our hospital in December 1990 with swelling of the lower lip and cheek which he had noticed 8 months earlier. Before his first visit to us, systemic corticosteroids had been administered for a week. This treatment resulted in remarkable improvement of the swelling of both sites; however, after discontinuation of these corticosteroids, recurrent episodes of the swelling occurred. Our examination revealed an enlarged, soft, nontender lower lip and a swollen cheek (Fig. 1). Bilateral hilar lymphadenopathy was not found by a chest radiograph. Computed tomography of the abdomen was normal. Laboratory investigations disclosed that lactic dehydrogenase was 195 U/L and that angiotensin converting enzyme (ACE) was 29.4 IU/L. The antibody against HTLV‐I was negative and a biopsy of the lower lip revealed a relatively dense infiltration of the inflammatory cells composed mainly of lymphocytes and histiocytes. Atypia of lymphocytes was not prominent and some muscle fibers showed degenerative changes (Fig. 2). A skin biopsy of the cheek disclosed a dense lymphohistiocytic infiltrate in the dermis. Based on the clinical findings, we tentatively diagnosed this case as cheilitis granulomatosa and reinstituted systemic corticosteroid therapy.

In December 1991 the patient was admitted for evaluation of muscle weakness of the forearms. Electric myography revealed a myogenic pattern. He showed neither a heliotrope eruption nor Gottron's papules. Laboratory investigation showed that the white cell count was 14 600/mm3 (lymphocytes 27%), creatinine phosphokinase (CPK) was 679 U/L, and ACE was 28.0 IU/L. A biopsy of the deltoid muscle disclosed muscle atrophy and a dense atypical lymphocytic infiltrate around the muscle fibers (Fig. 3). Immunohistochemical findings revealed that most of the Infiltrating cells were CD3(+) and CD4(+). Although molecular biologic investigation did not disclose the monoclonality of the gamma T‐cell receptor gene, this time we diagnosed the case from the histologic findings as T‐cell lymphoma. Needle biopsy of the bone marrow suggested the infiltration of lymphoma cells. In January 1992, chemotherapy of MACOP‐BV (MTX, doxorubicin hydrochloride, CPA, VCR, prednisolone, BLM, and etoposide) was started, but we were unable to prevent the progression of muscle weakness, and in April 1992 the patient died of pneumonia. No autopsy was performed.
Pseudo‐melanoma revealing Laugier‐Hunziker syndrome
Authors: Porneuf Marc , Dandurand Michel
Published: August 2010

A 47‐year‐old Arabic woman presented with a few months history of a 20 × 10 mm brown‐black interdigital macule, irregular in shape and outline (Fig. 1). The patient had no previous personal or familial history of dysplastic nevi, melanoma, or gastrointestinal polyposis. She was receiving fluindione, furosemide, digoxin. and lisinopril dihydrate for chronic atrial fibrillation. She had a mitral valvular bioprothesis secondary to rheumatic heart disease.

On physical examination there were no enlarged lymph nodes. Multiple pigmented macules were noted on the lower lip and buccal mucosa (Fig. 2). Genital mucosa showed no pigmentary abnormalities. There were also many pigmented longitudinal streaks involving five finger‐ and two toenails without Hutchinson's sign, two brownish interdigital macules of the fingers (Fig. 3), and dark brown spots around the nails. Clinical examination of her two sisters and three children did not demonstrate mucocutaneous pigmentation.

Investigations showed normal renal, hepatic, and thyroid function. Human immunodeficiency virus serology was negative. Two‐punch biopsy 4×4 mm of the right darkness and left edges of the interdigital lesion confirmed the diagnosis of Laugier‐Hunziker syndrome with basal hypermelanosis without melanocytic proliferation or cytologic atypia (Fig. 5). After 2 years follow‐up, clinical and histologic examination was not modified.
Omenn's syndrome
Authors: Nazzari Guido , Drago Francesco , Crovato Franco
Published: August 2010

A 7‐month‐old boy presented with a 6‐month history of a skin eruption of the scalp and the diaper area (Fig. 1) resembling seborrheic dermatitis. Since the age of 3 months, he had developed high grade fever, diffuse pruritic skin lesions, stomatitis (Fig. 2), chronic diarrhea, diffuse lymphadenomegaly, and failure to thrive. The parents were nonconsanguineous and no similar disease had ever been reported in the family.

Physical examination showed alopecia, generalized lymphadenopathy, and hepatosplenomegaly. The patient's skin was dry with generalized erythematous, scaling pruritic eruptions and small violaceous nodules on the extremities.

Laboratory investigations revealed: slight normochromic anemia, leukocytosis (18,000/mm3) with lymphocytosis (42%), and mild eosinophilia (12%); decreased levels of all classes of serum immunoglobulins were observed with normal IgE levels. Examination of phagocytosis, opsonization, and granulocyte chemotaxis revealed no abnormalities. The number of circulating T cells was within normal limits, whereas that of B cells was low. Recall antigens and T‐ and B‐lymphocyte proliferative response to mitogens and Candida albicans proteins resulted in poor stimulation. Chromosomal analysis of mononuclear ceils from the peripheral blood and skin fibroblasts showed a normal male Kariotype, and HLA typing did not reveal any maternal chimerism.

A skin biopsy specimen showed satellite cell necrosis of Keratinocytes and a dense lymphohistiocytic and eosinophilic perivascular infiltrate in the dermis. A specimen from a lymph node showed a picture consistent with dermatopathic iymphadenopathy, with an increase of cells with histiocytic appearance and a dense infiltrate of eosinophils. On electron microscopic examination, no cells with Birbeck's granulations were found in the skin infiltrate and lymph nodes. A bone marrow biopsy was normal.

One month later the patient developed generalized exfoliative erythroderma with recurrent cytomegalovirus infections and staphylococcal septicemia. Despite antimicrobial and prednisone therapy, the patient died of toxic shock at the age of 9 months. An autopsy showed diffuse lymphohistiocytic infiltration involving the skin and lymph nodes and extreme lymphocytic depletion in the thymus. Meningitis and pneumonia were also present.
The Olmsted syndrome
Authors: Santos Omar Lupi R. , Amorim José Hunaldo , Voloch Karen , Goes Marcia C. , Silva Maria R. , Pereira Antônio Carlos
Published: August 2010

A 35‐year‐old man had a hyperkeratotic disorder of the palmoplantar skin since the age of 6 months. The palmoplantar keratoderma progressed to thick and warty hyperkeratotic plaques, which enlarged and formed verrucous lesions and deep fissures. The acral keratoderma gradually involved the dorsal surface of the hands and feet with flexion contractures of the fingers and toes (Fig. 1). Since the age of 2 years, the patient also showed universal alopecia and small sharply marginated hyperkeratotic plaques around the nose and mouth, in the groin, and in the intergluteal area (Fig. 2). All of these keratotic lesions were strikingly symmetric.

Examination of the oral mucosa revealed a white plaque over the left lateral border of the tongue; crusted white verrucous plaques were found at the labial commissures and underneath the lower lip. There was complete absence of the nails and the roentgenographic examination of the hand showed pronounced osteolysis of the distal phalanx of each of the fingers. The palmoplantar keratoderma has been complicated by the development of constricting bands of keratin around the bases of the fingers, leading to their strangulation and autoamputation (Fig. 1). The mental and physical appearance were normal. A family history of similar verrucous lesions was lacking. Histopathologic examination of the palmar skin showed massive parakeratotic hyperkeratosis and considerable acanthosis. In the thickened horny layer, many cells showed perinuclear edema. The patient was treated with topical retinoic acid cream, 0.1%, at night. This produced an improvement in the plaques around the nose and mouth.
Facial bullous systemic lupus erythematosus
Authors: MD Adriana Lalova , Pramatarov Kyrill , Vassileva Snejina
Published: August 2010

A 37‐year‐old woman presented with a 6‐month history of malaise, fever, arthralgia, and mild butterfly facial erythema. A diagnosis of systemic lupus erythematosus (SLE) was established, based on clinical, laboratory, and histologic findings, and treatment with 6‐methylprednisolone (80 mg daily) was administered. A week later, a blistering eruption appeared in and around the erythematous areas on the face.

On physical examination, infiltrated erythematous plaques were present on the sun‐exposed areas: the arms, dorsa of the hands, and upper chest. In and around the erythematous areas on the face, there were numerous tense vesicles (Fig. 1). A few ulcerations were observed on the lower lip and the hard palate.

Laboratory findings revealed a reduced white blood cell count (WBC) (2500/mm3), with normal values of hemoglobin, red blood cell count (RBC), hematocrit, platelets, creatlnine, urea, total protein, blood glucose, and urinalysis. Antinuclear antibodies (ANA) were detected in titre of 1:320 on rat liver substrate. Anti‐DNA, Ro, La‐antibodies and LE‐cells were positive. Anti‐BMZ‐antibodies were not present on the indirect immunofluorescence (IIF) testing on normal human skin substrate. Histopathologic examination showed a subepidermal blister containing polymorphonuclears and eosinophils. There was a mixed Inflammatory infiltrate in the upper dermis (Fig. 2). Direct immunofluorescence (DIF) of perilesional skin from the face showed homogenous linear deposits of IgG and IgA at the basement membrane zone (BMZ); DiF from non‐sun‐exposed normal skin demonstrated granular deposits of IgA and IgM at the BMZ.

Besides the systemic steroid therapy with 6‐methylprednisolone, a treatment with dapsone at an initial dosage of 50 mg, and later 100 mg, daily was applied. The condition was resistant to the steroid therapy, but after 4 weeks of treatment with dapsone the lesions completely resolved.